Chapter 1 - General physical examination

In this chapter, we consider some aspects of the general physical examination that are especially pertinent to neurologic evaluation. In later chapters we will cover other aspects of the neurologic examination, the involvements by specific disease processes, systems abnormalities, and symptom complexes. Not all elements of examination can (or should) be conducted on every patient. Indeed, if all the examinations that we will describe were carried out on all patients, it would be difficult to see more than several patients a day. An efficient diagnostic approach demands careful evaluation and utilization of historical data in a problem-oriented fashion. This demands that the physician focus on those parts of the physical examination that are pertinent to the specific problem or problems elicited by history or by a basic screening exam.

Vital Signs
Of particular importance is evaluation of the respiratory rate and pattern in patients with depressed consciousness. This topic will be elaborated on in the section on the evaluation of coma in Chapter 24. Bilateral involvement of the brain stem from the diencephalon through the medulla may be associated with characteristic and therefore localizing patterns of respiration at each level of involvement. Unilateral dysfunction is usually not reflected by respiratory abnormalities. The abnormal patterns of breathing most likely represent loss of higher control of the primary medullary respiratory center. Suppression of medullary function by metabolic or direct mechanical involvement results in hypoventilation and, ultimately, apnea. Figure 1-1 is a schematic of respiratory patterns associated with bilateral lesions at various levels in the brain stem. Blood pressure is frequently elevated considerably above pre-morbid levels when there is increased intracranial pressure and then drops when intracranial pressure is lowered (although this usually is not of much localizing value). Blood pressure is also frequently elevated during an ischemic stroke, possibly a compensatory response to loss of part of the cerebral blood supply. Typically, the blood pressure will revert to baseline level over hours to days without need of therapeutic intervention. Attempts to lower the pressure, unless at extreme levels, can be counterproductive (resulting in further ischemia). Blood pressure drops to very low levels following loss of medullary function or severe cervical spinal cord damage; however, for therapeutic purposes, severely depressed blood pressure should be assumed to be due to non-neural causes (such as cardiac abnormality or blood loss) until proved otherwise.

Peripheral nervous system dysfunction (i.e., peripheral neuropathy) can produce symptomatic hypotension when the patient assumes the erect position (orthostatic hypotension). This can be related to involvement of the peripheral autonomic nervous system with loss of peripheral vasomotor tone and can be caused by many conditions that produce generalized peripheral neuropathy, such as diabetes, alcoholism, or malnutrition (see chapter 12). Orthostatic hypotension can be detected by recording blood pressure and pulse in the recumbent and upright position, looking for a drop of at least 20 mm Hg of systolic pressure, or a diastolic blood pressure decrease of at least 10 mm Hg within three minutes of standing. Symptoms of light-headedness or faintness would indicate that this was symptomatic orthostasis. If the pulse rate increases as the blood pressure drops, hypovolemia would be more likely than autonomic failure to be causing the orthostasis. Exercise prior to testing (which causes a reactive peripheral vasodilation, lowering peripheral resistance, may exaggerate any orthostasis). Isolated central or peripheral autonomic failure secondary to medications and metabolic or degenerative disease (including Parkinson disease) can also be a cause of orthostatic hypotension or other autonomic dysfunction (decrease in urinary bladder tone, erectile dysfunction, etc). The pulse rate may be slowed (bradycardia) or accelerated (tachycardia) with increased intracranial pressure, and therefore any change must be considered in a person with central nervous system involvement. Arrhythmias, particularly sinus arrhythmias, and nonspecific ST-T wave changes are frequently seen on electrocardiograms of persons who have had subarachnoid hemorrhage or either hemorrhagic or ischemic strokes. If atrial fibrillation is present, systemic embolization of thrombus formed in the non-pulsatile left atrium should be considered the most likely cause of a stroke.

Head
Changes in the shape and size of the cranium may reflect changes in the intracranial contents. In children younger than the age of closure of skull sutures, increased intracranial pressure is reflected in widened suture lines that may be palpable and quite visible in radiographs. If the pressure is prolonged, a mottled decalcification or beaten silver appearance of the skull and demineralization of the dorsum sellae may appear on x-rays. Bulging of the anterior fontanelle in the erect or seated infant is a reliable sign of increased intracranial pressure or contents. Progressively enlarging ventricles,

hydrocephalus, causes enlargement of the skull, which can be observed easily. Early diagnosis of hydrocephalus is possible by measuring the cranial circumference during well-baby check-ups (comparing it with standard charts). Subdural fluid effusions, usually associated with meningitis and subdural or epidural hematomas, also cause excessive skull enlargement in infants and toddlers who have nonfused suture lines. Premature closure of sutures causes characteristic distortions that should be recognized early because associated restriction of brain expansion may result in neuronal damage and mental retardation (Fig. 1-2). In adults, the shape and size of the cranium are less often revealing. The presence of asymmetric bony prominences contralateral to sensory motor deficits or in a person with focal seizures suggests an underlying meningioma, a benign tumor that occasionally causes secondary osteoblastic activity in proximate parts of the skull. In both adults and children who have a history of head trauma or in persons who are stuporous or comatose for unknown reason, the skull should be gingerly palpated for soft-tissue swelling, which suggests head trauma and possibly underlying fracture. Ecchymoses at the base of the occiput (Battle sign) or around the eyes but contained by the orbit (Raccoon eyes) suggest basal skull fractures. When the head is gingerly rotated from side to side, the brain, which is essentially floating in the subarachnoid cerebrospinal fluid and is tethered to the dura and contiguous skull by cranial nerves, blood vessels, and arachnoid membranes, is relatively resistant to the movement. The movement stretches the cranial nerves, the larger blood vessels and the dura. Under normal circumstances, no significant discomfort is caused by this stress. However, when the surface blood vessels or dura, which are sensitive to pain, are already distorted by a mass lesion or swollen with inflammatory edema as with migraine or arteritis, pain may be increased or experienced for the first time on shearing. The patient frequently can point to the area of involvement. Although not part of the routine exam, auscultation of the cranium (with the bell of the stethoscope) over the mastoid region, temporal region, forehead, closed eyes, or (in bald individuals) more extensively may reveal a vascular bruit arising from an arteriovenous malformation over the brain surface. The bruit is caused by the increased, turbulent flow in the arteriovenous short-circuit that makes up the malformation. In whom should auscultation of the head be considered? The person with a history suggesting an arteriovenous malformation is one candidate. This would include patients with headaches that are always on one side of the head (most patients with migraine, for example, have at least occasional headaches on the other side) or patients with focal seizures. Of course, in both these cases, cranial imaging is important. On occasion the

person with arteriovenous malformation hears a bruit, particularly at night when distractions are at a minimum. Careful auscultation of the head may help to localize that. The small child or infant with a congenital arteriovenous malformation in the area of the internal cerebral veins and the vein of Galen has a bruit that is audible over the whole head. S/he may have congestive heart failure from the high flow demands of the shunt, and also an enlarging head, the result of a communicating hydrocephalus. This is caused by the arterialization of pressure in the sagittal sinus, which increases resistance to absorption of cerebrospinal fluid through the arachnoid granulations. Compression of the aqueduct of Sylvius by the swollen vein of Galen may also be the cause of the hydrocephalus. The infant with meningitis and diffuse cerebral vasodilatation and the infant or child with severe anemia may have diffuse cranial bruits caused by high flow through the cerebral or diploic vasculature. These bruits are usually inaudible in the older child or adult whose skull is thicker and thus dampens the sound unless anemia is severe.

Eyes
There are several features of the eyes that should be considered in the neurologic exam. The conjunctiva and sclera can show signs of icterus or of inflammatory, vasculitic processes. A prominent corneal arcus can suggest dyslipidema which, in turn, suggests the potential for atherosclerosis. Funduscopic examination can be very revealing. First of all, it may show whether visual abnormalities are due to refractive problems, including poor visual correction or opacities (of the lens or cornea). It is also the only place in the body where blood vessels can be directly visualized. The health of these blood vessels is a reflection of the health of small blood vessels in the nervous system (including signs of atherosclerosis or of diabetic vascular disease that can effect the brain and peripheral nerves). We will specifically discuss two phenomena or neurologic import, papilledema and subhyaloid hemorrhage. Papilledema results from increased intracranial pressure, which occurs when the contents of the cranium exceed the capacity of the intracranial physiologic mechanisms and anatomy to accommodate. The major accommodating factors are the cerebrospinal fluid space (ventricular and subarachnoid) and its ability to be drained by the venous sinuses, the venous space and its collapsibility, the ability of sutures to spread in infants and toddlers, the ability of brain tissue to be compressed and lose substance, the ability of the foramen magnum (and to a lesser degree other foramina) to transmit pressure to the extracranial spaces, and finally, the possibility of

decreased production of cerebrospinal fluid from the choroid plexi when intracranial pressure rises to high levels. The major causes of increased intracranial pressure are cerebral edema, acute hydrocephalus (blockage of cerebrospinal fluid [CSF] absorption, relative or absolute), mass lesions (e.g., neoplasm, abscess, hemorrhage), and venous occlusion (e.g., sagittal or lateral sinus thrombosis). Papilledema or edema of the optic disk usually indicates increased intracranial pressure. When it is fully developed, recognition is not difficult; swollen, blurred and elevated disk edges, engorged and pulseless veins, and increased vascularity of the disk margins are the obvious signs. This is usually bilateral. At these stages, vision is usually unaffected (outside of possible slight increase in the physiologic blind spot *). With further development, hemorrhage (both superficial and deep) and exudates appear. If the process is chronic, filmy white strands of glia proliferate in and around the disk. It is at this late stage that the patient may complain of episodic obscured vision. This precedes final occlusion of the retinal arterial supply and infarction of the retina with permanent blindness. Early recognition is important in order to diagnose the underlying cause and also in order to prevent vision loss. It is usually possible to detect early, subtle signs of intracranial hypertension. Prior to well-established and easily recognizable papilledema, the normal pulsations in the veins of the optic disc disappear with elevated pressures. These pulsations are best seen in the normal fundus where the veins disappear into the substance of the disk. They reflect the arterial pulse pressure superimposed on a baseline intraocular pressure; the veins partially collapse during systole and expand during diastole. If the pulsations are not spontaneously present (as they are in about 75% of normal individuals), a minimal amount of pressure on the globe brings them out in almost all persons who do not have increased intracranial pressure (i.e., less than 200 mm of CSF). The minimal compression partially collapses the veins and allows them to expand during diastole. If intracranial pressure is 200 mm of CSF or greater, venous pulsations usually are not present and the higher the pressure, the less likely there are to be spontaneous pulsations. ** Papillitis or inflammatory edema of the disk looks very similar to papilledema. Indeed, in most cases, they are identical. Papillitis is most often caused by demyelinating processes in young and middle-aged persons (such as multiple sclerosis) and by optic nerve arterial involvement in older individuals. It is not associated with increased intracranial pressure. As opposed to papilledema, however, it is almost always unilateral. The visual field loss associated with papillitis is usually greatest near the center of vision, because the macular (cone vision) fibers are primarily affected. This leads to early loss of

color, particularly red, vision. A central scotoma (blind area) is typically present and thus visual acuity is severely and uncorrectably limited (see Chap. 3). This is distinct from papilledema which usually only produces slight enlargement of the blind spot until quite late when the arterial supply is compromised by compression. Even then, the vision loss of papilledema is usually distinct because it starts at the periphery, with central vision preserved until late. Subhyaloid hemorrhage is a collection of extravasated blood just beneath the inner limiting membrane of the retina (Fig. 1-3). This is different from most retinal hemorrhages which occur deep to the nerve fiber layer.*** Subhyaloid hemorrhages are frequently observed close to the disk margins with an acute, catastrophic rise in intracranial pressure. This is invariably caused by intracranial arterial hemorrhage (subarachnoid or intracerebral) or head trauma with hemorrhage and brain contusion or laceration. They are often seen in "battered infant" syndrome, for example. They appear almost immediately and frequently on the background of a relatively normal-appearing retina. They are presumably caused by a rapid and excessive rise in the central retinal venous pressure, which leads to rupture of the small venular radicals near the disk. Papilledema may follow within several hours. On a normal background the hemorrhages are diagnostic and should allow the physician to avoid lumbar puncture, which, in the presence of cerebral hemorrhage or cerebral contusion or laceration, could further predispose the patient to brain herniation (see Chap. 24). Acute central retinal vein thrombosis, frequently associated with long-standing diabetes mellitus, can also cause subhyaloid hemorrhages. In these cases, it is almost always unilateral, however, and may be very large or distributed in the segmental distribution of one or several central venous branches. The patient does not appear otherwise ill and complains only of loss of vision in the involved eye. The visual loss may be surprisingly minimal.

Ears
In bacterial meningitis, particularly in children, one of the most frequent portals of entry for bacteria is the chronically or acutely infected middle ear. The presence of otitis media is readily visible on otoscopic examination as an opaque, bulging, erythematous tympanic membrane and should be looked for in all persons who are suspected of having meningitis. Successful care of the meningitis may depend on eradication of the otitis, which may necessitate puncturing the eardrum (myringotomy) for drainage in addition to administering appropriate antibiotics.

The patient who is unconscious and has no history or obvious signs of etiologic significance should be suspected of having head trauma. In addition to palpation of the cranium for evidence of fracture and observation of ecchymosis, the physician should look for a bulging, blue-red tympanic membrane. If present, it indicates hemorrhage into the middle ear and is pathognomonic for severe head trauma. Basilar skull fracture with dissection through the middle ear is considered the cause; however, severe shearing of the ossicles may be enough to cause tears in blood vessels and hemorrhage. The external ear may be implicated in the person with hearing loss, especially if the deficit is of the conduction type (see Chap. 6).

Neck
The spinal cord, meninges and cervical roots are stretched slightly when the head is flexed onto the chest. This ordinarily can be done without any discomfort; however, this is not so when the meningeal sheaths of the roots are inflamed. Pain and reflex stiffening of the nuchal muscles are elicited when meningitis is present (called "nuchal rigidity" or meningismus). Because the spinal cord is pulled by this maneuver and moved upward slightly in the spinal canal, the lower lumbosacral roots are also stretched and pain may be experienced in the low back and legs as well as the neck. Occasionally, spontaneous flexion of the legs and hips occurs on neck flexion (termed Brudzinski sign). This is a reflex attempt to put some slack in the stretched lumbosacral roots (with the legs in the flexed position, the femoral and sciatic nerves and therefore their roots of origin are slackened). It is usually not symptomatically successful; however, it is a strong indication of meningitis and other causes of root irritation. A majority of the population by age 65 to 70 has x-ray evidence of degenerative disease of the cervical spine. This osteoarthritic change, frequently referred to as cervical spondylosis, appears to be a product of the constant trauma of the weight of the oversized human head on the neck when in the erect position. Despite the appearance of severe cervical osteoarthritis on x-rays, disabling symptoms do not occur in the majority of people with cervical spondylosis. Spondylosis does correlate with decreased mobility (mostly in rotation and lateral bending). Flexion is usually not terribly limited and there is usually not much (or any) pain reported by the patient. They also compensate for the loss of mobility, which occurs slowly. The most important symptoms and signs that can be caused by spondylosis result from irritation or destruction of the cervical roots and/or the spinal cord by the hypertrophic degenerative disks and joints. Nerve root involvement gives localized signs

that are positive (e.g., pain and paresthesias) or negative (e.g., loss of sensation, reflexes and power) in character. Damage to the spinal cord by spondylosis can result in motor and sensory symptoms below the lesion due to interruption of the long tracts of the spinal cord by impingement. Lhermitte's symptom (or Lhermitte's sign) includes a sensation of "electric shocks" radiating from the posterior nuchal region into the arms, trunk, and legs separately or in combination by forcibly extending or flexing the neck. This signals cervical spinal cord involvement and is probably caused by rapid distortion of the cervical cord and associated depolarization in irritated sensory nerve fibers. During extension of the neck, the spinal canal is narrowed in its anterior-posterior diameter by anterior buckling of the posterior-lying ligamentum flavum. In persons with cervical osteoarthritis, the ligamentum flavum is hypertrophied and the vertebral canal is narrowed more than it is normally on neck extension; in combination with the canal narrowing caused by posterior intervertebral disk protrusion, this may be enough to cause pressure on the ventral or dorsal surface of the cord. Lhermitte's symptoms presumably are caused by traumatic depolarization of the sensory tracts. This can happen with neck flexion as well, since the spinal cord is stretched (potentially depolarizing irritated nerve fibers). Lhermitte initially described this symptom in patients who had multiple sclerosis involving the cervical spinal cord. In this situation, neck flexion that stretches the spinal cord probably causes symptomatic depolarization in the dorsal column or spinothalamic tracts made irritable by a demyelinating plaque or other lesion (e.g., neoplasm or syrinx). In general, a physician should suspect an extramedullary lesion (i.e., pressure on the outside of the cord) if Lhermitte's symptom is elicited by extension of the neck and an intramedullary lesion if elicited by flexion. A useful maneuver for corroborating your suspicions of root irritation by posterior lateral protrusion of degenerated disk material is for you to extend the patient's neck and then press the head firmly downward, thus narrowing already narrowed spinal foramina. Frequently this elicits the patient's symptoms and thus corroborates suspicions. More marked foraminal narrowing can be elicited by extending the head and then flexing it to left or right. On pressing the head inferiorly (Spurling's maneuver), further foraminal occlusion occurs on the side to which the head is flexed (Fig. 1-4). Direct pressure on the posterior lateral part of the neck with the thumb or index finger may also produce discomfort over the involved roots. Of course, any forced movement of the neck should be done cautiously in patients with possible spinal cord injury or injury to the cervical spinal column. Only enough force should be used to elicit the sign or symptoms and, in the case of acute injury it may be necessary to perform x-rays before any such maneuver.

Extremities
Movements of the lower extremity beyond a certain point will stretch lumbar plexus and nerve roots. Straight leg raising (i.e., passive flexion of the straightened leg on the hip), or the reverse, extension of the leg on the hip, is used to indicate the presence or absence of irritative or destructive lesions involving the lumbosacral plexus and roots. When the leg is flexed on the hip, the posterior-lying sciatic nerve, which originates in the lower lumbar and upper sacral roots (L4-S2), is stretched and therefore also stretches the plexus and roots (Fig. 1-5A). This stretching usually begins after about 30 degrees of flexion. Extension of the leg on the hip (with the patient lying on the side or prone) stretches the anterior-lying femoral nerve, which originates from the middle lumbar roots (L2-4) (Fig. 1-5B). Any mass or inflammatory process impinging on the nerve, plexus, or roots is likely to bind or irritate these structures and cause pain in the peripheral distribution of the nerve. This pain is often in the muscle and bone distribution of the nerves as opposed to the skin or dermatomal distribution. Buttock, posterior thigh, calf, as well as heel discomforts are characteristic of sciatic system involvement, whereas groin and anterior thigh pains are characteristic of femoral system involvement. If there is skin involvement, loss of sensation occurs in appropriate dermatomes or peripheral nerve distribution. However, loss of sensation or weakness will only occur if the lesion is destructive. Paresthesias (pins-and-needles sensations) are more common symptoms of dermatomal involvement than is actual loss of sensation. A symptomatic irritative lesion may not cause any actual loss of nerve function. Low-back pain with or without radiation to the leg or legs is the most common cue for the physician to do the straight leg raising test. A common cause of low-back pain is lumbosacral disk disease and, if pain radiates into the leg, a herniated disk is usually the cause. Ninety-five percent of involved disks are between the L4-5 or L5-S1 vertebral bodies. Therefore, because the L5 and S1 roots exist at these spaces, straight leg raising with the patient supine, causing sciatic stretch, is the maneuver of choice. Four percent of lower spine disk problems occur at L3-4 or L2-3, whereas the remaining 1% incidence is shared by L1-2 and thoracic disks. With the higher lumbar protrusions, femoral stretching is the maneuver of choice. Positive test results reproduce or increase the patient's complaints of leg pain. An increase of back pain, alone, is not considered an indication of nerve root involvement and is considered a negative straight leg raising test. Such a "negative test" may still cause pain, but this is from stretching irritated tendons, joints and muscles in the back. Also, a true positive test must be distinguished

from tightness of the hamstring muscles, which can produce discomfort during straight leg raising. Acute or chronic arthritis of the hip, on occasion, causes referred pain in the knee and, less commonly, in the foot. Straight leg raising may irritate a damaged hip joint and may give a misleading impression of sciatic root irritation. This can be detected by rotating the femur on the hip while the knee and hip are flexed. This flexion of the knee puts slack on the sciatic and femoral nerves (Fig. 1-6). Pain caused or increased by this maneuver (Patrick maneuver) suggests hip disease, and appropriate x-rays can confirm this suspicion. Flexion of the head on the chest (chin on chest) pulls the spinal cord upward and stretches the lumbosacral roots somewhat. Lumbosacral root irritation may therefore be increased, causing reproduction or exacerbation of the patient's low back and leg pain. This maneuver would not change the symptoms of hip disease. Meningitis manifests itself throughout the subarachnoid space, and therefore straight leg raising has positive results because the lumbosacral roots and investments are inflamed. In fact, there may be involuntary flexion of the knees during attempted straight leg raise (Kernig sign). This can easily be inferred from our earlier discussion of Brudzinski sign.

Spine
Pathologic processes (degenerative, neoplastic, or inflammatory) in or near the spinal column frequently give rise to local muscle spasm and pain. If the process is unilateral, muscle spasm, presumably the result of direct or indirect irritation of the dorsal sensory and/or motor branches to the paraspinal muscles, causes characteristic distortion of the spine in addition to palpable firmness and tenderness. When the paraspinal muscles contract unilaterally, they bow the spine laterally; the concave side of the bow appears on the side of increased muscle tension (Fig. 1-7B). This lateral bowing is called scoliosis and can be observed most easily when the patient is erect. Observation is further facilitated by making a mark with a pen on the palpable top of the dorsal spinous processes of the vertebrae. The only exception to the rule of contralateral bowing occurs at the lumbosacral junction where the paraspinal muscles are broadly attached to the sacrum and the ilium. The bowing occurs to the side of the spasm in this instance (Fig. 17A). Percussion of the spine to elicit point tenderness is routinely carried out with the hypothenar portion of the fist. Because a large area is covered with each blow to the

spine and there is diffusion to several vertebral segments, it is more reasonable to use a percussion hammer and tap each spinous process. Often this accurately localizes the segments involved.

Abdomen, Pelvis and Rectum

Every general medical evaluation should include examinations of the abdomen, pelvis and rectum. However, on neurologic evaluation these examinations are not called for unless the patient cues them by certain complaints. The most common cue is the complaint of low-back pain with or without radiation into the legs. Even though disk and spine or paraspinous disorders are the usual cause of this complaint, several common pelvic neoplastic disorders give rise to very similar complaints and may be associated with positive straight leg raising. Carcinoma of the cervix is the most common form of female pelvic neoplasm. It spreads by local extension and therefore (by invading the pelvic [lumbosacral] plexus) may first become symptomatic as low-back and/or leg pain. Carcinoma of the prostate is the most common male pelvic tumor and also spreads by local extension. Low-back and/or leg pain may be the symptom that brings a patient for medical attention. Rectal carcinoma occasionally gives rise to low-back pain because of spread to and enlargement of local lymph nodes. Rectal and pelvic examinations are mandatory when low back pain is a complaint, especially in middle-aged and older adults in the age range when the incidence of neoplasia increases. The lumbar and sacral spine are also common sites of metastasis of these cancers and this must be considered in the patient with a history of cancer or in older individuals with new lower back pain. Low back pain or, for that matter, bone pain involving all levels of the spinal axis in women mandates breast examination to rule out carcinoma of the breast which frequently metastasizes to the spine and other long bones. Acute, new back pain should give rise to consideration of possible abdominal aortic aneurysm (often palpable or visible on lumbar x-rays and measurable by ultrasound). Renal disease should be considered in patients with flank pain and, particularly, if the pain is colicky. When urinary or fecal incontinence is present or a complaint, rectal examination is indicated to evaluate both reflex and voluntary anal sphincter function.

*The retina is very sensitive to mechanical pressure. You may demonstrate this by pressing very lightly on the lateral side of one of your eyes. The depolarization block caused by minimal compression of the retina creates a blind spot (scotoma) in the contralateral field (i.e., next to your nose). In like manner, early and poorly visible

swelling of the disk margin depolarizes and blocks the proximate retina and enlarges the physiologic blind spot. The blind spot represents the retina-deficient optic disk and is routinely plotted and of fairly uniform size when formal visual fields are studied with a tangent screen or perimeter (see Chap. 3). **The mechanism for loss of venous pulsations is presumed to be an increase in venous backpressure subsequent to intracranial hypertension. It is presumed that papilledema is a function of the ratio of intracranial (and, therefore, intravenous) pressure to intraocular pressure; elevation of the former or depression of the latter is adequate to abolish pulsations and elicit edema of the disk. For practical purposes, papilledema is almost always the result of increased intracranial pressure. Increased intraocular pressure (glaucoma) should delay the appearance of papilledema, and this is so; it can be the source of some diagnostic confusion. ***In persons with long-standing diabetes mellitus or systemic hypertension, the blotlike hemorrhages may be present but are usually associated with other abnormalities of the retina, including hemorrhages of the nerve fiber layer (flame-shaped or striated), narrowing and atherosclerotic distortion of the arteries, exudates, capillary aneurysms, and vascular proliferation (neovascularization).

Chapter 2 - Hemispheric function

The cerebral hemispheres, particularly in large and redundant cerebral cortical mantle, are the anatomical substrates of the uniqueness of Man. The cortex of the cerebral hemispheres embodies the higher integrative or intellectual capacities of man and its expanse in area and neuronal numbers far outstrips the same parameters of our nearest phylogenetic cousin, the chimpanzee. The complexity of neuronal ramification and interconnection is fantastic! It is estimated that there are nine billion neurons in each cerebral hemisphere and each neuron has between five and ten thousand interconnections with other neurons neighboring and distant. The mathematical dimensions are staggering, little short of infinite. The latest generation of man-made brains, in all their circuit complexity cannot compare. The higher integrative functions can be divided into those functions having diffuse representation in the cortex and those with more focal representation. Some functions

are in both hemispheres and some are unilaterally represented. Obviously, all of this affects the symptoms produced by damage to the brain.

Diffuse Bilateral Functions

According to Harold Wolff, diffuse bilateral functions include: "(a) the capacity to express appropriate feelings, appetites and drives; (b) the capacity to employ effectively the mechanism of goal achievement (learning, memory, logic, etc.); (c) the capacity to maintain appropriate thresholds and tolerance for frustration and failure, and to recover promptly from their effects; (d) the capacity to maintain effective and well-modulated defense reactions (i.e., repression, denying, pretending, rationalization, blaming, withdrawal, fantasy, depersonalization, obsessive compulsive behavior and bodily reaction patterns involving alimentation, respiration, metabolism, etc.)." These are the kind of functions that are lost only after diffuse and bilateral injuries to the brain, such as occur in the dementing disorders. Dementia is defined as a progressive loss of intellectual and higher emotional capacities. Many of the early changes in rational and emotional behavior are nonspecific for cerebral dysfunction; that is, they can be the reflection of severe psychological stress and disorder not of clear-cut organic origin but of the psychiatric sphere (which have been termed "pseudodementia"). Condensing the above further, one can see that man's major intellectual achievement has been the rational control by inhibitory modulation of the basic drives of self and species preservation common to all animals: feeding, fighting, fleeing and procreation. The complexity and variability of these rational and emotional drives and behavior becomes greater and greater as one ascends the phylogenetic scale, culminating with man. The manifestations of functional loss, reversible or irreversible, therefore become more complex and subtle as the functional anatomy of the nervous system ascends through phylogeny. As neuronal diffuse dysfunction becomes more advanced, obvious and unmistakable changes occur. There are deficits in emotional response, often with a tendency toward apathy and flatness of affect, alternating with wide and inappropriate swings of emotional behavior. The latter reflects a loss of rational inhibitory control of the basic emotional drives mediated by the limbic system. Defects in learning and memory, particularly the former, abstract thinking, general information and capabilities, and in judgment are easily detectible in patients with diffuse bilateral involvement. It may be difficult to distinguish patients with certain psychiatric disorders from those with early diffuse cortical degeneration. It may be impossible to test cognitive

functions in patients who are psychotically depressed or catatonic due to schizophrenia. Fortunately, organic cerebral hemispheric deterioration frequently uncovers primitive reflexes that can be elicited without cooperation from the patient. Most of these complex reflexes occur in infants and are suppressed during normal cortical development. These reflexes reappear with dementia (that is, they become disinhibited). There is a long list of regressive reflexes has evolved. The most commonly sought and elicited are the feeding or mouthing phenomena including involuntary snouting, sucking, rooting, and biting in response to tactile or visual stimuli, forced grasping with the hands and the feet, and extension of the great toe on plantar stimulation (Babinski response). Patients with dementia often show motor perseveration, inappropriately repeating the same movement due to loss of ability to inhibit ongoing activity. This may be observed with repetition of words or ideas (this can occasionally be seen in normal individuals when fatigued or under emotional strain). Another manifestation of this perseveration can be seen when testing resting muscle tone. The patient appears to have an inability to relax, termed paratonia, when the examiner is attempting to passively move a body part. This can be quite frustrating to the examiner, since it appears that the patient is willfully resisting passive movement. This perseveration of tone can meld into a perseveration of movement as the patient overcomes initial inertia and gets into rhythm with the examiners testing movements. The examiner becomes aware of this when releasing the patient's arm or leg and instead of dropping relaxed to the bed, it continues the supposed passive movements. These abnormalities of tone, labeled paratonia (or gegenhalten), can be seen in infants and young children so may also represent a regression of function. It can be seen in normal individuals, but is much more common in patients with dementia, and therefore has been included with other things as a "soft sign" of dementia. Historically, diffuse hemispheric disease (i.e., dementia) has been considered to be a progressive and hopeless condition of old age. Twenty years ago, current knowledge and therapeutics accepted just that attitude with few fortunate exceptions. Today the number of treatable and potentially reversible disorders of the cerebral hemispheres is growing although the majority of cases of dementia are not reversible. Additionally, some of the previously untreatable dementias are yielding partially to new therapies. New diseases are not appearing; old entities are being recognized as treatable with the expansion of etiologically and therapeutically oriented research in dementia. Obviously, the treatable causes of dementia must take diagnostic priority though they may be statistically unlikely.

Specific Hemispheric Regions and Structures

Some brain functions are localized to specific hemispheric regions. For example, there are areas of primary motor and sensory function that are highly localized. On the motor side, skilled movements (particularly of the distal upper limbs) of the contralateral side of the body are initiated by the primary motor cortex in the precentral gyrus. There are areas of highly localized sensory function, including the somatosensory cortex in the postcentral gyrus and the visual cortex of the occipital lobe. damage to there areas can affect the ability to feel things or see things on the contralateral side of the body. The ability to hear and smell are bilaterally represented, and therefore are generally unaffected by unilateral brain injury. There are several more complex functions that lateralize. Included among these functions are language, handedness and visuospatial orientation and, as already alluded to above, learning, emotionality, and behavioral inhibitory control.

Left hemisphere
In 97% of the population language is represented in the left hemisphere, with little if any contribution from the right hemisphere. Only three in one hundred people will have significant right hemispheric representation of speech functions; of those three, two will have significant bilateral representation of speech, with only one individual having right hemisphere dominance. It is known that early brain injury (the earlier the better, but generally before the age of about 4), is associated with transference of language function to the spared hemisphere. With increasing age and gradual lateralization and anatomical fixation of speech functions to the left hemisphere, less and less flexibility remains. The areas involved in the central organization of language, which is man's most advanced capability, are appropriately the most advanced and latest developed neocortical zones. It is not too surprising that this highest function would localize in the most advanced regions and further still that this function would tend to utilize the greatest expanse of advanced cortex, which happens to be, in most, localized on the left. The above is interesting but grossly speculative. "Why unilaterality?" might be the next question. No one has proposed a fully satisfactory answer to this teleological question. It is possible that this is for efficiency, such that language function does not have to occupy similarly large areas on both sides of the brain (leaving more cortex for other functions). However, this is speculative. Man appears to be, with rare exception, the only animal with significant lateralization of such an important function (some birds apparently have lateralization of their singing capabilities).

Handedness correlates fairly closely with language dominance. Ninety percent of the population is right-handed; of 1,000 right-handed people only one will be right hemisphere dominant for speech; overwhelmingly, to be right-handed is to be left brained for language. Ten percent of the population is left-handed; 7 of 10 left-handed individuals are left-brain dominant for speech, essentially breaking down the nice speech-handedness correlations seen in right-handed individuals. The remaining 3 lefthanders will be those with either bilateral representation of speech (2) or with right hemisphere dominance (1). Functional Magnetic Resonance Imaging (fMRI) has added the capability to study regional metabolic activities in the brain, which is adding to and corroborating past findings determined by traditional methods (see Chap. 23). We have learned most that we know about speech functions and localization from disease processes involving the brain. Some minor contribution has come from stimulation studies and observations of the effects of drugs. Table 2-1 summarizes the effects of destructive lesions of the classic anatomical speech areas of Broca and Wernicke. Dysfunctions of language are called dysphasias, complete loss of some component of language function is called aphasia. Testing of the patient with a suspected language disorder requires several steps. These include: observation of the characteristics of spontaneous production; response to variably complex commands; the ability to repeat complex phrases; the ability to name objects and parts of objects; and the ability to read and write. Testing of these functions will usually quite accurately localize the area of involvement. The finding of other areas of cortical damage can also help localize the process, since some functions are located close to the language areas of the cortex. For example, a patient with verbal language dysfunction, homonymous hemianopsia and little motor deficit, will more than likely have a receptive (Wernicke) dysphasia. A patient with a verbal language dysfunction, marked hemimotor and hemisensory deficit and no visual abnormality will probably have an expressive (Broca) dysphasia. For practical purposes it is worth noting that the majority of patients with dysphasia will have a combination of both expressive and receptive dysfunctions (called global dysphasia). This is because the majority of patients who are dysphasic are so because of cerebral infarction and the infarction, usually patchy, involves the middle cerebral artery territory, which encompasses both language areas as well as the pathway connecting them, the arcuate fasciculus (see Fig. 2-1). Damage to the arcuate fasciculus can disconnect the area of the brain that comprehends language (Wernicke area) from the area that is generating language (Broca area). This would abolish the ability to repeat a

complex phrase, since the comprehension of the phrase could not be transmitted to the area generating the words. An even more unusual "disconnection syndrome" occurs when the areas around the primary language areas are damaged, leaving the primary language areas intact (Fig. 22). This "disconnects" the language areas from the rest of the cortex, which is contributing to the thought processes that are then being expressed through the primary language areas. Such individuals would be able to repeat, but would have problem spontaneously generating meaningful language. Damage to the entire corpus callosum can cause a very striking disconnection syndrome (sometimes termed "split brain"), although it may not be observed unless the proper functions are tested. One of the most striking features of the fully expressed "split brain" is the inability to verbally tell you what an article is, if it is placed in the left hand (assuming left hemisphere dominance and that the patient is prevented from looking at it). Additionally, this individual will be unable to understand written language if the writing is presented only to the left visual field. This material reaches only the right hemisphere and cannot be transferred to the left or verbal hemisphere, for interpretation. A rather striking and frequently-quoted example is that of the woman with corpus callosum transection who snickered when a risqu picture was presented to her left field. When asked why she laughed her left hemisphere answered, "It's a funny test." When the picture was flashed into the right visual field, and therefore seen by the left hemisphere, the patient quipped "You didn't tell me I was going to have to see this kind of a picture." During the first presentation of the picture, the right hemisphere saw the picture and laughed. The left hemisphere rationalized that the laugh must have been because the test was funny. From the above it is obvious that the right and left cerebral hemispheres, to some degree, are able to function as two separate individuals if disconnected. In addition to having visual transfer problems, transection of the corpus callosum will prevent transfer of auditory verbal commands from the left hemisphere to the right. Commands to do chores with the left hand will therefore be carried out imperfectly or not at all. These examples and the observation of the patient with a split brain pulling the pant leg up with the right hand and down with the left (as if the right and left hemispheres were in competition) reinforce the assumption of a partial schizo cerebration which comes to light only when the major connection, the corpus callosum, is destroyed. It is noteworthy that there may be other connections between the left and right hemisphere, especially if damage to the corpus callosum occurs early in life (such as agenesis).

Right hemisphere
The right hemisphere must be considered functionally inferior to the left since it lacks significant speech representation. Therefore it has been termed the "non-dominant" hemisphere. However, certain functions do tend to localize to the right hemisphere. For example, the ability to recognize loss of function, visuospatially oriented perception and behavior, and musicality all appear to be predominantly functions of the right cerebral hemisphere. Also, the ability to generate verbal inflections and to detect tone of voice appears to be localized to the right hemisphere. The patient with severe right hemispheric dysfunction (e.g., subsequent to infarction, trauma, hemorrhage, or tumor) will manifest rather obvious deficits in elementary hemispheric functions: s/he will have a hemiweakness, hemisensory depression, and various abnormalities of cranial nerve function. These deficits are not at all surprising based on cortical localization. However, particularly if the non-dominant parietal lobe is involved, the capacity to acknowledge or recognize loss is severely impaired; for example, the patient may not know that there is anything wrong and therefore will deny the allegation that there is a deficit. When asked to move the left arm they may say that they have done so even though no visible movement has occurred. More bizarrely they may reach for the left arm and grasp the examiner's, which has been slipped in the path, and claim that it is their own. Also they may deny that their arm actually belongs to them; this abnormality probably depends to some degree upon the amount of sensory depression on the left. Some time ago, a patient with severe right hemisphere dysfunction due to a stroke was examined at the VA hospital. When turned onto his right side for the purpose of carrying out a lumbar puncture he vociferously objected to the presence of another person who was lying on top of him; the other person was his own left side! The term applied to the lack of appreciation (or neglect) of deficits is "anosognosia" is the term applied to this deficit. In time, anosognosia fades, compensated by recovery of right cerebral function or some transfer of this function to the left hemisphere. However, there are usually some remnants of neglect unless the pathology completely reverses (e.g., the patient, when asked what is wrong, might answer, "The doctors tell me I am weak on the left," etc.). These patients, as you may surmise, tend to be poor rehabilitation candidates because their neglect decreases their motivation for improvement. The patient with a similar motor disorder in the right limbs from left hemispheric damage, despite the fact that they may have severe language deficits, is quite conscious of the motor loss and quite willing, even insisting, to rehabilitate him- or herself.

Lesions of the right hemisphere, particularly when they involve the confluence of the parietal, occipital and temporal lobes are frequently associated with visuospatial disorientation of a disabling degree. This can be tested at the bedside by having the patient fill in well-known cities such as San Francisco, New York and Washington on a map of the United States or by having the patient copy a two dimensional rendition of a cube. At a practical level, visuospatial disorientation creates problems with following directions, reading maps and when an unfamiliar place is encountered navigation may become grossly disordered. Penfield described a patient, who after right temporal lobectomy was lost as soon as he lost sight of home. he was forced to take a job in the post office across the street from home in order to avoid daily confusion (Fig. 2-3). Musicality is also a predominance of the right hemisphere. Lesions, particularly of the temporal-occipital-parietal confluence on the right, cause variable deficits in tune learning and reproduction. Left-sided destruction can leave the patient without speech but musical ability will frequently remain intact with the patient readily and correctly reproducing tunes if s/he is cued by the examiner.

Frontal lobes
The frontal lobes include the areas of the motor cortex and the premotor cortex posteriorly, and the prefrontal cortex anteriorly. The motor and premotor cortices are involved in the planning and initiating of movements. Damage to medial areas of the premotor cortex (supplementary motor area) can prevent the ability to initiate voluntary actions (abulia) that can be so severe as to prevent any movement (akinesia). Additionally, Broca area is part of the premotor cortex in the dominant hemisphere. The prefrontal cortex has more complex functions. Broadly, we divide this part of the cortex into dorsolateral prefrontal cortex and the orbitomedial prefrontal cortex. The dorsolateral prefrontal cortex is involved in what has been called executive functions. Osborn described these functions as: "The ability to organize thoughts and work, to create plans and successfully execute them, to manage the administrative functions of one's life. Individuals with impaired executive function may appear to live moment-tomoment, fail to monitor their activities or social interactions to make sure plans are carried out (or even made). With diminished ability to create strategies, to handle more than one task at a time, to be effective, reliable, and productive, the simplest job may be too challenging." Damage to this area also can affect "working memory" which is the ability to hold something in the mind while manipulating it (such as repeating a string of numbers backward) and also inhibits the ability to perform several tasks simultaneously.

The orbitomedial prefrontal cortex is involved in control of impulses and behavior. Damage to this area severely affects personality. Patients display poor judgment, inadequate planning, and little motivation. With more advanced disease, they may become inappropriately jocular ("Witzelsucht") and irritable and lose their social graces. It has been proposed that the orbitomedial prefrontal cortex is anatomically situated (in terms of their connections) between the perceptual motor systems of the hemispheres and the limbic system. Lesions in this area might then divorce perception and action from motivation. A classic example of this was described by Harlow after prolonged observation of a patient, Phineas Gage, who had sustained severe damage to the orbitomedial prefrontal cortex. The equilibrium or balance, so to speak, between his intellectual faculties and animal propensities, seems to have been destroyed. He is fitful, irreverent, indulging at times in the grossest profanity (which was not previously his custom) manifesting but little deference for his fellows, impatient of restraint or advice when it conflicts with his desires, at times pertinaciously obstinate, yet capricious and vacillating, devising many plans of future operation, which no sooner arranged than they are abandoned in this regard his mind was radically changed, so decidedly that his friends and acquaintances said that he was no longer Gage. There are a variety of physical findings that are common, but not specific, for prefrontal cortex lesions. Grasp, sucking and snout reflexes are common. Paratonia (gegenhalten) and perseveration of actions or speech are often seen. As described above, paratonia (gegenhalten) refers to an increase in tone; instead of relaxing, the patient either resists or tries to help when the examiner attempts to move the limbs passively. Perseveration refers to the repetition of a response when it is no longer appropriate. A person may raise their hand on command, for example, and then continue to raise their hand when asked to point to the floor or touch the nose. Verbal responses can similarly demonstrate perseveration. Perseveration may occur for a variety of reasons: inability to make the correct response, failure to check the response against the question, or lack of attention to the task. However, it may also be an inability to terminate or change ongoing motor activity or postures. This has been considered more likely to occur with loss of the inhibitory influences of the frontal lobes. A number of tests have proved sensitive to the akinesia of patients with frontal lobe disease, and to their tendency to persist in incorrect behavior even when they know they are wrong. A test of word fluency can easily be given at the bedside. The patient is asked to produce as many words as they can that begin with a given letter (excluding proper nouns) in a one minute period. Normal individuals can produce 14 +/- 5, words using the

letters A, F, or S. Patients with left frontal lobe lesions produce fewer words, and often repeat words or persist in using proper nouns.

Limbic system
The limbic system, in addition to subserving higher emotional functions, appropriately subserves a major component of the memory system, declarative memory (memory for facts or relationships that can be expressed verbally or symbolically). The ability to imprint new material (short term memory) is lost with bilateral destruction of most of the major paired structures of the limbic system including the cingulate gyri, hippocampi (and adjacent medial temporal lobes), fornices, mammillary bodies and anterior and medialis dorsalis nuclei of the thalamus. The patient with these lesions will be able to retain material as long as they are concentrating on it; this attentive or immediate memory probably depends upon the integrity of the major sensory pathways, the reticular activating system of the upper brainstem and the dorsolateral prefrontal neocortex. If, however, attention is distracted, s/he will have difficulty or be unable to recall the presented material. In fact, the patient may ask, "What three words?" when asked to reproduce three unrelated words such as table, red, 23 Broadway after a period of distraction. There are several conditions that can produce isolated bilateral depression or destruction of limbic structures involved in short term memory. For example, it can occur as the result of herpes simplex encephalitis, bilateral posterior cerebral artery occlusive disease or may be the result of unilateral temporal lobectomy if the patient has a previously damaged (e.g., from birth trauma) contralateral temporal lobe. Bilateral temporal lobe involvement may be an early and prominent sign of Alzheimer's disease. The limbic system is also much more susceptible to metabolic insults such as hypoxia and thiamin deficiency, the latter being most often seen in malnourished alcoholics. In the case of alcoholic effects on the brain, which probably result from bilateral damage to the dorsomedial nucleus of the thalamus, the memory deficit may be accompanied by confabulation (a tendency to respond to memory tasks by "making up" plausible answers). Of course, if things cannot be remembered over minutes to hours, they can not be remembered long-term. However, well-learned material is probably represented diffusely and is very resistant to focal destruction. Indeed, well-established memories fail last in patients with diffuse bilateral hemispheric dysfunction. Animal experiments suggest that intermediary or less well-established material is first stored in the temporal lobes and becomes more widespread or redundant in localization with reinforcement.

Some clinical corroboration of this is seen in patients with bilateral temporal lobe lesions who have variable and patchy retrograde amnesia. When testing a patient for problems with learning and memory, it suffices to ask for: 1. reproduction of three unrelated words immediately and then after a period of distraction; 2. a description of recent past events, for example front page news items, the contents of breakfast (if not stereotyped fare) and what they have been doing recently (assuming that the examiner knows the answers to these questions); and; 3. a description of some well learned past material such as the past 4 or 5 presidents, birth dates of patients and family, anniversaries, number and location of children and grandchildren, etc.

References
Benson, DF. Aphasia, Alexia, and Agraphia. New York, Churchill Livingstone,

1979.
Geschwind, N. Selected papers on Language and the Brain. Boston, Reidel, 1974 Vinken PJ and Bruyn, F.W. (eds). Disorders of speech perception and symbolic

behavior, in: Handbook of Clinical Neurology, Vol. 4. New York, John Wiley & Sons, 1969.

Questions
Define the following terms:

agnosia, agnosagnosia, apraxia, receptive aphasia, expressive aphasia, global aphasia, alexia, agraphia, dysinhibition, dysnomia, paraphasic, paratonia, perseveration.
Agnosia is the inability to recognize what something is despite being able to perceive it. This can be visual, auditory or tactile. Agnoagnosia is the inability to recognize a particular deficit (for example that one is paralyzed).

Apraxia is the inability to synthesize a complex motor pattern despite having the strength and coordination to perform it. Receptive aphasia is the inability to understand language (written or verbal). Expressive aphasia is the inability to synthesize language (written or verbal). Global aphasia is the inability to either understand or to synthesize language (written or verbal). Alexia is the inability to read. Agraphia is the inability to write. Dysinhibition is the appearance of responses that are normally suppressed. In the context of higher cognitive functions, it is the return of more primitive reflexes or behavior patterns that are normally suppressed by "higher areas" of the brain (often the frontal lobe). Dysnomia is the inability to name objects. Paraphasic errors include substituting words that have inappropriate meaning, although the words sound somewhat similar or start with the same sounds. This often happens with receptive aphasias. Paratonia is and involuntary, irregular resistance to passive movement (it feels like the patient is assisting in movement when they are not attempting to). Perseveration is repeating motions (or responses) when it is inappropriate to do so.

2-1. Name some cerebral cortical functions that are well localized and unilateral. (unilaterally or bilaterally) and some that are diffuse.
Answer 2-1. Well localized, unilateral cortical functions include: somatic sensation, voluntary motor function (especially of hands), expressive language, receptive language, attention to the contralateral world (neglect), understanding of what is wrong (agnosagnosia), vision.

2-2. Name some cerebral cortical functions that are well localized and represented bilaterally.
Answer 2-2. Well localized and bilateral cortical functions: hearing, short term memory, frontal lobe functions (mood, behavior, emotional control, motivation, executive functions), visuospatial function (parietal lobe)

2-3. Name some cerebral cortical functions that are diffusely represented in the cerebral cortex.
Answer 2-3. Diffuse cerebral cortical functions: long term memory, self and species preservation functions (including many behavioral functions.

2-4. Damage to which cerebral cortex produce aphasia?

Answer 2-4. Aphasia is lateralized to the dominant hemisphere

2-5. What can you say about the ability to write in patients with aphasia?

Answer 2-5. Patients with aphasia will write and read the same way that they speak and comprehend speech, respectively.

2-6. What can you say about the ability of a patient with expressive receptive or global aphasia to repeat complex phrases?
Answer 2-6. Patients with expressive, receptive or global aphasia will be unable to repeat complex phrases.

2-7. What can you say about the ability of a patient with transcortical aphasia to repeat complex phrases?
Answer 2-7. Patients with transcortical aphasia will be able to repeat although they may not be able to name objects that are presented to them or to understand language.

2-8. What problems will a patient with a transcortical aphasia have?

Answer 2-8. Transcortical aphasia: will be able to repeat complex phrases but will either be unable to understand complex statements or commands (transcortical receptive) or be unable to come up with names for objects (transcortical motor).

2-9. What are the characteristics of the patient with an expressive aphasia (Broca's).
Answer 2-9. Epresssive aphasia: Brocca's area, nonfluent, frustrated, dysnomic, can read and understand speech, telegraphic speech.

2-10. What are the characteristics of the patient with a receptive aphasia (Wernicke's).
Answer 2-10. Receptive aphasia: Wernicke's area, fluent, not frustrated, dysnomic, can't read or understand complex speech.

2-11. What is the most common lesion to produce alexia without agraphia (can write but can't read)?
Answer 2-11. Damage to the dominant occipital lobe and splenium of corpus callosum can produce alexia without agraphia (can write but can't read).

2-12. What area is involved in immediate recall (for example of a phone number).
Answer 2-12. Immediate recall is a frontal lobe effect (give back a phone number).

2-13. What area is involved in short term memory?

Answer 2-13. Short term memory is a hippocampal function (minutes).

2-14. Where is long-term memory stored?

Answer 2-14. Long term memory is stored diffusely (only lost if large and diffuse areas are damaged).

2-15. What are "executive functions" and where are they primarily located?
Answer 2-15. Executive functions in dorsolateral perfronal part of frontal lobes - these include sequencing, planning, immediate recall, abstractions.

2-16. Where are the areas involved in most of emotional control and "personality"?
Answer 2-16. The orbital and medial frontal (and anterior cingulate) cortex are involved in emotional control.

2-17. Damage to which hemisphere is more likely to produce depression? Which will more likely produce mania?
Answer 2-17. Left frontal damage often produces depression, right frontal may lead to mania.

2-18. Neglect of one side of the world is most commonly due to damage to what area?
Answer 2-18. The parietal lobe is responsible for attention to contralateral world (damage produces neglect) and knowledge of deficits.

2-19. Agnosagnosia most often results from damage to what area?

Answer 2-19. Agnosagnosia, a lack of recognition of problems, is due to damage to the parietal lobe (especially the non-dominant side).

2-20. What "primitive responses" would be expected to be uncovered by damage to the frontal lobes?
Answer 2-20. disinhibited glabellar, snout, suck, palmomental and grasp reflexes.

2-21. Paratonia is a sign of what?

Answer 2-21. Paratonia results from diffuse cortical dysfunction (some degree may be normal).

2-22. What would you expect to see in the patient with a split corpus callosum?
Answer 2-22. Corpus callosum lesions may prevent information from transferring from one hemisphere to the other. The "left hand does not know what right hand is doing."

2-23. The neocortex provides inhibitory modulation of what four basic drives?
Answer 2-23. Feeding, fighting, fleeing and procreation (the four F's).

2-24. What is the clinical term used to describe diffuse hemispheric disease (one word)?
Answer 2-24. Dementia.

2-25. What are the clinical signs of advanced dementia?

Answer 2-25. Loss of cognitive, intellectual functions in more than one sphere of function.

2-26. What regressive reflexes emerge with loss of cortical inhibition?

Answer 2-26. Dysinhibition of glabellar response, palmomental reflex, grasp reflex, suck reflex, rooting reflex, snout reflex and loss of nuchocephalic reflex.

2-27. What what are the functions of the limbic areas of the brain?
Answer 2-27. Self and species preservation functions, the "four F's" and emotional reactivity.

2-28. Of 100 people, how many will have significant R hemispheric representation of speech functions? Of these, how many will have bilateral speech representation?
Answer 2-28. 1% right dominant and 2% mixed.

2-29. What percentage of R-handed people are L-hemisphere dominant for speech?
Answer 2-29. About 99.9%.

2-30. What percentage of L-handed people are L-hemisphere dominant for speech?
Answer 2-30. About 70%.

2-31. Below what age can speech function be recovered if the dominant hemisphere is damaged?
Answer 2-31. Age four.

2-32. What are dysfunctions of speech called? What is a complete loss of speech called?
Answer 2-32. Dysphonia (if hoarseness due to mechanical problems in larynx), dysarthria (if due to problems with cranial nerves or cerebellum) or aphasia (this is actually a language problem, not just speech problem). Dysphasia is incomplete, aphasia complete loss of language function.

2-33. A patient with verbal language dysfunction, homonymous hemianopsright visual field deficit and little motor deficit most likely has what type of dysphasia?
Answer 2-33. Receptive (Wernicke's) - this is because Wernicke's area is closer to the parietal lobe (and the optic radiations).

2-34. A patient with verbal language dysfunction, marked hemimotor and hemisensory deficit, and no visual abnormality most likely has what type of dysphasia?
Answer 2-34. Expressive (Broca's) - this is because Broca's area is closer to the motor cortex.

2-35. Where is Broca's area located? Where is Wernicke's area located? Name the fasciculus that links the two of them.
Answer 2-35. Broca's - Inferior frontal lobe, just anterior to motor cortex and near Sylvian fissure; Wernicke's - posterior part of superior temporal gyrus; the arcuate (superior longitudinal) fasciculus connects these language areas.

2-36. What gyrus is important in language, especially in word retrieval?

Answer 2-36. Angular gyrus.

2-37. Do most patients with dysphasia have Broca's, Wernicke's, or a combination of both? Why is this so?
Answer 2-37. Combination because they are in same vascular distribution on same side.

2-38. What language abnormalities are manifested with a lesion to Broca's area? Wernicke's area? Angular gyrus? Arcuate (superior longitudinal) fasciculus?
Answer 2-38. Broca's - expressive aphasia; Wernicke's - conductive aphasia; Angular - dysnomic aphasia; Arcuate fasciculus - conductive aphasia.

2-39. What part of the corpus callosum transfers COMPLEX [i.e., verbal] visual info between the two hemispheres?
Answer 2-39. The splenium.

2-40. What are the predominant functions of the R cerebral hemisphere?

Answer 2-40. Visuospatial functions, attention to contralateral world, musicality.

2-41. Which patient will be more motivated to recover from a hemispheric lesion, one with damage on the L or the R?

Answer 2-41. The left (the right hemisphere lesion can result in a lack of appreciation for deficits and therefore problems in compensating).

2-42. Where is the location of a lesion that causes visuospatial disorientation? How does this manifest itself?
Answer 2-42. Right parietal lobe. Patients may get lost and have trouble assembling things or figuring out how to make them work.

2-43. What type of lesion will result in the loss of the ability to imprint new information?
Answer 2-43. Hippocampal lesions - medial temporal lobe (bilaterally).

2-44. Can well-learned material be easily destroyed by a focal lesion? Why or why not?
Answer 2-44. No, there is diffuse representation.

2-45. What three categories of questions need to be asked when testing a patient for problems with learning and memory?
Answer 2-45. Immediate recall; short term recall (several minutes after distraction); recall of remote events.

2-46. What evidence would lead to the conclusions that a demented patient has disease localized primarily in the frontal lobes (i.e., what are the manifestations of lesions to the frontal lobes)?
Answer 2-46. Emotional lability, personality change and/or loss of executive functions.

2-47. What is the effect of lesions localized to the medial aspect of the frontal lobes (parasagittal frontal cortex - supplementary motor area)?
Answer 2-47. Inability to initate movements (abulia).

* The patient is stood or seated with eyes closed in front of the examiner. The examiner rotates the patient's shoulders. The uninhibited response consists of the patient's head remaining straight.

Chapter 3 - Olfaction and Vision

Vision and olfaction are two of the special senses. While patients usually easily recognize loss of vision, loss of olfaction may not be recognized without testing.

I. Olfaction
Unilateral depression or loss of olfaction (anosmia) is most commonly due to obstruction of the nasal passages. When it is due to damage to neural structures, it must affect the

olfactory pathway at or rostral to the olfactory trigone (Fig. 3-1). Therefore, dysfunction must be in the olfactory tract, bulbs, nerve filaments, or olfactory mucosa in the roof of the nasal passage.

Olfactory pathway
The olfactory pathway divides immediately anterior to the anterior perforated substance to travel via (1) the lateral olfactory stria to the primary olfactory cortex (prepiriformpiriform) in the ipsilateral mesial part of the temporal lobe, (2) the anterior limb or stria that dives into the anterior perforated substance to join the anterior commissure, which carries it to the contralateral olfactory cortex, and (3) the medial olfactory stria, which travels medially from the trigone to distribute to limbic cortex in the septal, subcallosal, and parasagittal frontal regions. Damage to the olfactory epithelium, the olfactory filiments, the olfactory bulb or olfactory tract can cause unilateral anosmia. Destruction of olfactory cortex or olfactory pathways posterior to the trigone (where the tracts divide) must be bilateral to depress olfactory function. Potentially irritative lesions (tumor, post-traumatic or ischemic scarring, arteriovenous abnormalities, etc.) in the olfactory cortical regions may be the source of epileptic activity and cause olfactory symptoms; that is, the patient may complain of hallucinations of smell. Typically, these olfactory hallucinations are described as acrid and unpleasant and are not lateralized by the patient.

Testing of olfaction
Olfaction is tested by having a patient with eyes closed, sniff a relatively familiar odor from a small vial, occluding the nares alternately to test each side separately. Substances such as acetic acid and ammonia should not be used for testing because they cause strong trigeminal stimulation and can therefore be sensed by an anosmic person. Coffee grounds are popular because they are recognized by approximately 80% of normal individuals and cause minimal trigeminal stimulation. Three distinct levels of function can be determined: (1) cannot smell, (2) can smell something, and (3) recognizes coffee. In addition, a person may recognize an asymmetry of sensitivity despite an inability to recognize the substance. Lack of recognition is not significant if the patient responds by saying that they smell a substance bilaterally; recognition on one side suggests contralateral hyposmia if the patient claims to smell but not recognize something on the opposite side.

Disorders affecting olfaction

Examples of disease processes that cause or are associated with decreased or otherwise abnormal smell are as follows: 1. Mechanical.
o o o o a. Common cold with occlusion of nasal passages (most common cause of b. Unilateral occlusion by deviated nasal septum. c. Occipital head trauma with shearing effect on olfactory nerve filaments passing d. Frontal head trauma, if it results in a fracture line through the cribriform plate, hyposmia).

through cribriform plate (Fig. 3-2). causes anosmia by tearing the fine olfactory nerve filaments. (In the absence of fracture, frontal head trauma is less likely to cause hyposmia.) o e. Tumors (most commonly meningioma) on the mid-portion of the sphenoid ridge or in the olfactory groove, both capable of pressing on the olfactory tract. If the tumor is on the sphenoid ridge so that, in addition to pushing up on the olfactory tract, it compresses down on the optic nerve, it causes the syndrome of ipsilateral anosmia, optic atrophy, and possibly also contralateral papilledema because of increased intracranial pressure caused by the tumor mass effect (Foster-Kennedy syndrome).

2. Metabolic.
o o o o o o a. Pernicious anemia (vitamin B12 deficiency) is frequently associated with b. Vitamin A deficiency is associated with hyposmia and dysosmia (odors are c. Zinc deficiency has been associated with hyposmia and dysosmia. d. Diabetes mellitus. Presumably, this hyposmia is secondary to demyelination in e. Multiple sclerosis is a rare cause of hyposmia, presumably on the basis of f. Herpes simplex encephalitis, which tends to localize in the temporal lobes and bilateral decreased olfaction. unpleasant), possibly the result of nasal mucosal abnormalities.

the olfactory tracts or loss of the peripheral olfactory neuron. olfactory tract demyelination. cause severe hemorrhagic necrotic destruction, may cause anosmia secondary to bilateral olfactory cortex destruction or alternatively, because the virus may enter the nervous system via the olfactory mucosa, may destroy one or both olfactory nerves and bulbs in the process. The presentation of acute-onset anosmia and a severe memoryencoding deficit (the latter secondary to bilateral mesial temporal lobe destruction) in a

person who is febrile suggests the possibility of herpes simplex encephalitis, a treatable disorder. o g. Hepatic disease, particularly acute hepatitis, is frequently associated with an unpleasantness of odors (dysosmia).

Unilateral destructive or compressing (mass) lesions in the anterior temporal lobe may cause olfactory hallucinations, a focal epileptiform discharge that often spreads to involve other portions of the limbic system and neocortex (see Chap.13).

II. Vision
For a more detailed elaboration of visual system anatomy and function, refer to a neuroanatomy text. Figure 3-3 shows diagrammatic representations of the visual pathway including expected abnormalities of vision caused by lesions of its various parts.

Visual deficits
Unilateral lesions of the retina and optic nerves cause monocular defects. Lesions from the chiasm back give rise to binocular field defects because of crossing of the nasal half of the retinal fibers from each eye. An exception to this rule occurs when there is involvement of the fibers representing the nasal retinal (temporal field) peripheral crescents. The fibers from this portion of the retina have no homonymous counterpart in the opposite peripheral temporal retina (nasal field). The peripheral crescents, therefore, remain monocular in representation from the retina to the visual cortex (Figs. 3-3, 3-4 and 3-5).

Testing of vision
Formal testing of vision divides this function into two basic aspects: (1) central or cone vision, and (2) peripheral or rod vision. Peripheral vision is the greatest part of the visual field, whereas central vision represents a relatively small segment of the projected visual world. Nevertheless, it is mainly central vision that is responsible for visual acuity and color vision. However, cones require a lot of light in order to function effectively. Peripheral vision also subserves a major function of directing central vision by visualoculomotor responses toward the peripheral stimuli. The more peripheral the field, the less capable of form or figure perception it becomes, which is in keeping with the centrifugal thinning out of the population of peripheral field receptors (rods) in the retina. At the far periphery one is capable of perceiving only moving objects, although reflex movements of the eye (directing the eyes toward a moving stimulus) can be

elicited from this region. Rods have a very low threshold for activation by light as compared with cones and are thus more suited for night vision. In daylight, pigment "bleaches" and the rods are insensitive. The cones, which have a high threshold for pigment bleaching by light, are relatively useless in the dark.

Visual acuity
Visual acuity is first tested by having a person read a chart (Snellen chart) containing standard-sized figures (numbers, letters or other forms) as perceived at a standard distance. The notation 20/20 vision means that the patient can recognize objects at 20 ft. that a normal person can recognize at that distance. The designation 20/70 means that the patient, at best, can only recognize at 20 feet what abnormal individual can recognize at 70 feet. This type of testing is not practical at the bedside, so charts have been developed to be presented at 14 in. (Fig. 3-6). These give an extrapolated visual acuity in terms of 20 ft. and are adequate to detect neurologic visual dysfunction, but may miss refraction errors, particularly nearsightedness (myopia). For quick screening, it is useful to know that recognition of small-case newsprint at 14 in. is equivalent to 20/30 vision. Visual acuity can be depressed by changes in the refractive structures of the eye anterior to the retina. In neurologic practice, we are not concerned with refractive problems and, therefore, it is important to have a way to detect these causes of loss of visual acuity. If a person customarily wears glasses, s/he should be tested with them on. If difficulties still exist, then further testing is warranted. Ophthalmoscopic examination should detect corneal opacities or cataracts. It would also detect intraocular problems, such as hemorrhage, which could obscure vision. Refractive errors commonly affect visual acuity. However, conditions such as myopia (near-sightedness) or hyperopia (farsightedness) can be corrected with a series of lenses. Alternatively, at the bedside, most simple refractive errors can be corrected by having the patient look through a cluster of pin-holes (Fig. 3-7). This works by only permitting parallel light rays to pass the pinhole. This markedly increases the dept of focus since parallel rays do not have to be focused. Pinholes would make inexpensive but impractical glasses, however, because peripheral vision, most central vision, and a great amount of light are eliminated. If visual acuity is not substantially corrected by the pinhole and if no problems exist with the refractive media of the eye (on ophthalmoscopic examination), it can be assumed there is a neurological (i.e., cone system) central visual defect. A further way to estimate refractive errors, which is particularly useful in the uncooperative patient, is to determine the diopters (e.g., +3, -3) of ophthalmoscope

adjustment necessary to focus on the macula or optic nerve head. This assumes "0" to be equivalent to normal acuity.

Visual field
After determining that the visual deficit is not a refraction or occlusive problem one can deduce, by default, it is a dysfunction in the neural visual apparatus. In order to localize the lesion, it is necessary to evaluate the visual field integrity. Visual field loss tends not to be an all-or-nothing phenomenon. The patient frequently has a partial deficit, particularly in the central field, and can see larger objects after small objects are no longer perceived. Testing that utilizes small objects is more sensitive. Formal testing of visual field can be done in several ways. There are automated tests, such as "Goldman visual field testing" in which the patient is asked to push a button when a flash of light is detected. "Tangent screen" testing is an older method in which the patient fixates on a central target at a distance of 3 meters while objects are moved into the screen. Of course, each eye has to be tested independently (Fig. 3-8). At the bedside, "confrontation" is the most commonly employed method for evaluating visual field. This can be quite accurate if carefully done. One eye is covered and the person is asked to fix their vision on the examiner's pupil at a distance of approximately 1.5 ft. A small, colored object (for example a 3 mm red object such as a fireplace match) is moved into the visual field in a plane halfway between the patient and the examiner. The patient is asked to indicate when s/he sees the object and when it turns red as well as whether it disappears or loses its color anywhere in the field. This technique allows the examiner to compare the patient's vision with their own (presumably normal). Additionally, it is critical to observe whether the patient is fixating on the central object (i.e., examiner's pupil) during the examination. A colored object is used because it defines the major extent of cone vision. On occasion, a partial loss of central vision manifests itself more in depression of color perception than in actual loss of visual acuity. For example, optic neuritis often diminishes the ability to see red objects ("red desaturation"). Screening to test all peripheral quadrants with both of the patient's eyes open and fixating on the examiner's nose reveals all peripheral defects except the rare cases of nasal hemianopias and hemianopias with temporal crescent sparing (see Figs. 3-3 and 3-4). Monocular testing does not miss any peripheral defects but takes twice as long. If there has been damage to optic nerve fibers for more than a couple of weeks, ophthalmoscopic visualization of the optic disk may reveal evidence of "atrophy" of the temporal portion of the optic nerve head (optic disk). This part of the optic disk

transmits the optic nerve fibers from the macula (representing central vision). Atrophy causes the disk to change from its slightly yellowish appearance to a brighter white owing to gradual replacement of myelinated nerve fibers by glial scarring. Comparison with the opposite disk is useful in borderline cases when changes are minimal and the problem is monocular. There is a normal, oval scotoma (the "physiologic blind spot") in the temporal portion of the visual field (see Fig. 3-8). This is the visual representation of the optic nerve head (the disk), which does not contain rods or cones. When a 3 mm object is passed over this area, a person usually says it disappears. It is often helpful to suggest to the patient that the object will likely disappear in some part of the visual field since most of us are completely unaware of the presence of a blind spot. If the object is moving midway between the examiner and the patient and both are fixating on each other's pupils, their blind spots should be superimposed. The ability of the test to pick up the physiologic blind spot is a good check on the reliability of the mode of testing of the visual field. An early sign of edema or swelling of the optic disk (papilledema) is enlargement of the blind spot. This is because the retina is extremely sensitive to mechanical pressure and minimal, unobservable edema of the optic nerve head causes significant dysfunction in the bordering receptive retina and, therefore, enlargement of the blind spot. Also, glaucoma will tend to push out on the optic disk, producing expansion of the optic cup (at the center of the disk) and also some expansion of the blind spot. More severe glaucoma can destroy the retina thorough pressure. Peripheral visual fields are formally tested using the tangent screen (Fig. 3-8) and more completely using a perimeter (Fig. 3-9), which takes into account that the total visual field is an arc (see Fig. 3-5). A tangent screen is flat and so cannot demonstrate the total extent of the peripheral visual field. A relatively large, white (color is not useful with rod vision testing) object (approximately 10 mm in diameter) is moved along the perimeter from the outside in and the peripheral field is mapped out in degrees. The outside limits represent where the patient, while fixing on a central target, first sees the moving object. A simple and more practical technique is used at the bedside. The patient is asked to fixate on the examiner's pupil as in testing central vision, and a large object, frequently the examiner's index finger, tip first, is moved into the patient's visual field (confrontation) from a position lateral to the patient's head. This is a rapid and easy way to approximate peripheral visual fields (Fig. 3-10).

Double simultaneous stimulation

It is useful at the bedside to use tachistoscopic double simultaneous stimulation (TDSS) of the visual fields. This entails the rapid momentary presentation of two objects simultaneously into opposite visual fields. In practice, a momentary movement of the tip of the index finger in both fields is suitable (see Fig. 3-10). TDSS testing is advantageous because minor partial field deficits, which may not be picked up on unilateral stimulation become apparent; the object in the abnormal field is extinguished. A rapid single excursion of the examiner's index fingers in the peripheral fields is adequate (see Fig. 3-10). Extinction in the visual field may represent either a partial dysfunction in the visual pathways or may be due to inattention phenomenon to one side of the body. This latter difficulty is usually part of a broader syndrome of hemispatial inattention (neglect), usually resulting from contralateral parietal (and occasionally frontal) association cortices.

References
Brodal, A.: Neurological Anatomy in Relation to Clinical Medicine, ed. 2. New

York, Oxford University Press, 1969.

Cogan, D.G.: Neurology of the Ocular Muscles, ed. 2. Springfield, IL, Charles C.

Thomas, Publisher, 1956.

Monrad-Krohn, G.H., Refsum, S.: The Clinical Examination of the Nervous

System, ed. 12. London, H.K. Lewis & Co., 1964.

Spillane, J.D.: The Atlas of Clinical Neurology, ed. 2. New York, Oxford

University Press, 1975.

Walsh, F.B, Hoyt, W.F.: Clinical Neuro-ophthalmology, ed. 3. Baltimore,

Williams & Wilkins Co., 1969.

Questions
Define the following terms:

anosmia, homonomous, hemianopsia, quadrantanopsia, scotoma, papilledema, papillitis, optic neuritis.

Anosmia is a loss of the sense of smell (this can be unilateral or bilateral). Homonomous is a term referring to overlapping areas of the visual field of each eye. Hemianopsia (or hemianopia) is a loss of visual perception of one-half of the visual world.

Quadrantanopsia is a loss of visual perception of one-quarter of the visual world. Scotoma is a patch of vision loss. Papilledema is swelling of the optic nerve head usually produced by congestion of the central retinal veins. This usually happens due to increased intracranial pressure. Papillitis is swelling of the optic nerve head due to inflammation. Optic neuritis is inflammation of the optic nerve.

3-1. How do you test olfaction?

Answer 3-1. Olfactory nerve is tested with aromatic compounds presented to each nostril.

3-2. What is the most common cause of unilateral anosmia?

Answer 3-2. Most common cause of unilateral anosmia is blockage of nasal passage.

3-3. In whom is it particularly important to test olfaction?

Answer 3-3. Olfactory testing is most important in patients with head injury, mental status change and seizure.

3-4. How can you determine if visual acuity problems are due to refractive or to nerve problems?
Answer 3-4. Visual acuity problems that are refractive improve with pinhole testing (retinal or optic nerve problems do not).

3-5. What is the significance of finding a monocular visual loss?

Answer 3-5. Assuming that this is not due to refractive problems in the eyeball (usually ruled out by a funduscopic examination) monocular problems are anterior to the optic chiasm (retina or optic nerves).

3-6. What is the significance of finding a homonomous visual field deficit?

Answer 3-6. Homonymous visual field problems are posterior to the optic chiasm.

3-7. Where would a lesion that produced bitemporal hemianopsia be located?

Answer 3-7. Bitemporal hemianposia is often due to problems at the optic chiasm (such as with pituitary tumors).

3-8. How can lesions of the parietal or temporal lobes produce vision loss? What kind of loss would you expect to find?
Answer 3-8. Optic radiations that pass from the thalamus to the visual cortex in the occipital lobe either pass through the parietal lobe (lower visual field) or temporal lobe (Meyer's loop - upper visual field) - may produce quadrananopsia of either the contralateral lower visual world (parietal lobe) or upper visual world (temporal lobe).

3-9. Where on the visual cortex is the representation of the center of vision?

Answer 3-9. The center of vision is represented near the occipital pole (often supplied by middle cerebral artery).

3-10. What artery supplies the visual cortex?

Answer 3-10. Most of the visual cortex is supplied by posterior cerebral arteries.

3-11. How can you distinguish papilledema from papillitis?

Chapter 4 - Extraocular movement

Eye movements are controlled by muscles innervated by cranial nerves III, IV and VI. In this chapter, the testing of these cranial nerves will be discussed. The most common symptom of damage to these nerves is double vision. The oculomotor nerve has the additional function of control of the pupil and therefore this will be discussed here as well. Eye movements are carefully controlled by other systems. Some of these will be discussed here, while others, such as the vestibular system, will primarily be discussed in other chapters.

Cranial nerves III, IV, VI. Ocular Motility

Oculomotor function can be divided into two categories: (1) extraocular muscle function and (2) intrinsic ocular muscles (controlling the lens and pupil). The extraocular muscles include: the medial, inferior, and superior recti, the inferior oblique, and levator palpebrae muscles, all innervated by the oculomotor nerve (III); the superior oblique muscle, innervated by the trochlear nerve (IV); and the lateral rectus muscle, innervated by the abducens nerve (VI). The intrinsic eye muscles are innervated by the autonomic systems and include the iris sphincter and the ciliary muscle (innervated by the parasympathetic component of cranial nerve III), and the radial pupillodilator muscles (innervated by the ascending cervical sympathetic system with its long course from spinal segments T1 through T3).

Extraocular muscle function

The muscles of the eye are designed to stabilize and move the eyes. All eye muscles have a resting muscle tone that is designed to stabilize eye position. During movements, certain muscles increase their activity while others decrease it. The movements of the eye include: adduction (the pupil directing toward the nose); abduction (the pupil directed laterally); elevation (the pupil directed up); depression (the pupil directed down;

intorsion (the top of the eye moving toward the nose); and extorsion (the superior aspect of the eye moving away from the nose). Horizontal eye movements are rather simple. Increased activity of the lateral rectus will direct the pupil laterally, while increased activity of the medial rectus will direct it medially. However, movements of the eyes above or below the horizontal plane are complicated and require, at the minimum, activation of pairs of muscles. This is because the obit is not directed straight forward in the head and, therefore, there is no one muscle positioned to direct the eye straight up or down without the simultaneous occurence of unwanted movements. Because of this, the protocol for testing eye movements is somewhat more complicated than might be expected. Figure 4-1 illustrates the correct eye positions for testing the extraocular muscles in relative isolation. As can be seen in Figures 4-1 and 4-2, a lateral position of the eyeball is necessary for testing the inferior and superior recti, whereas a medial position is necessary for testing the inferior and superior oblique. This is because, in the position of lateral gaze, the superior and inferior rectus muscles are in line with the axis of the globe, "straightening out the pull" of these muscles and allowing them to move the eye straight up or down. When the eye is directed nasally (medially), the oblique muscles align with the axis of the globe and are, therefore, the prime muscles for vertical gaze when the eye is adducted. Vertical gaze from the neutral position (Fig. 4-1) is accomplished by simultaneous activation of the superior rectus and inferior oblique (for upgaze) and of the inferior rectus and superior oblique (for downgaze). It is not necessary to have the patient look straight up and down in order to test each of the extraocular muscles. However, this may reveal evidence of vertical nystagmus (a sign of brain stem vestibular damage) and to determine the integrity of the midbrain center for vertical gaze (which may be defective despite adequate individual muscle activity). illustrates the expected findings with isolated loss of function of cranial nerves III, IV, and VI. Since there is resting tone in all of the eye muscles, isolated weakness in one muscle results in deviation of the eye due to the unopposed action of all of the remaining muscles. This typically results in double vision when the person tries to look straight ahead (although some patient may ignore the input from one eye). The afflicted person often adjusts their head position in an attempt to ameliorate the double vision caused by the muscle imbalance. The position that their head assumes is one that permits them to use their "good eye" to line up with the affected one. This is often successful in cases of isolated damage to cranial nerve IV or VI, with the head assuming the position shown in Figure 4-3. In this figure, the dashed vector lines show which directions of muscle pull are lost. The solid vector lines indicate the resting tonus of the remaining extraocular

muscles. Note that the head is tilted in CN IV damage. This is the classic position from which the English phrase "cockeyed" is derived. When cranial nerve III is involved, there may be enough ptosis to close the eye (preventing diplopia). However, if the eye is open, there is usually too much imbalance to overcome by head positioning and patients usually have diplopia. The person with an extraocular muscle defect of recent onset usually complains of double vision (diplopia). This results from the inability to fuse the images on the macular regions (central vision) of both eyes. Since the weak muscle is unable to bring the eye to a position in which the object is focused on the macula, the image falls on a more peripheral part of the retina. The person sees the object in the field appropriate to the new retinal position (i.e., always farther toward the periphery in the direction of attempted gaze. Additionally, because the image falls on retinal region with fewer cones, it is less distinct. The patient may compare it to the "ghost images" seen on maladjusted television sets. Sometimes it is very obvious which eye is not moving sufficiently when you perform the "6 positions of gaze". Also, the direction of the diplopia can give clues about weakness. For example, horizontal diplopia (where the images are separated horizontally) is due to problems with the medial and lateral recti, while vertical diplopia is due to problems with one or more of the other muscles. When it is not obvious on observation, one can delineate which extraocular muscle or muscles are defective by determining which eye sees the abnormal image (i.e., the blurry image that is farthest toward the periphery in direction of eye movement). This can be done by placing a transparent red piece of plastic or glass in front of one eye and asking the patient (who is observing a small light source such as a penlight or white object) which image is red, the inside or outside, lower or upper, depending on whether the diplopia is maximum in the vertical or lateral field of gaze. Figure 4-4 demonstrates the findings in one patient with medial rectus dysfunction and in one with lateral rectus dysfunction. The abnormal image in both cases is laterally displaced in the field of gaze and blurred (even though different eyes are involved in each case). Alternatively, if a red glass is not available, you can use the cover test to determine which eye is involved. In this case you will need to ask the patient to identify which image disappears when you cover one eye. Again, the eye that is projecting the image most off to the periphery is the one that is affected. The red glass and cover tests are particularly useful in delineating minimal muscle dysfunction, in which it is frequently difficult to determine which muscles are involved by observation on primary muscle testing.

Central control of eye movement

It is worthwhile at this point to review the anatomy of the central pathways of the oculomotor system. Figures 4-5 and 4-6 schematically outline the major central pathways that are important to conjugate lateral gaze, conjugate vertical gaze and convergence. Additionally, the deficits caused by destructive lesions in various parts of these systems are diagrammed. The central control of eye movement can be distilled into the principle types of functions. These include voluntary, conjugate horizontal gaze (looking side-to-side); voluntary, conjugate vertical gaze (looking up and down); smoothly tracking objects; convergence; and eye movements resulting from head movements. These latter movements, are part of the vestibular reflexes for eye stabilization and will be discussed with the vestibular nerve. The vestibular chapter is also where nystagmus (a to-and-fro movement of the eye) will be discussed. The movements of they eyes produce by the central nervous system are conjugate (i.e., both eyes moving in the same direction in order to keep the eyes focused on a target) except for convergence, which adducts the eyes to focus on near objects. Voluntary horizontal gaze in one direction begins with the contralateral frontal eye fields (located in the premotor cortex of the frontal lobe). This region has upper motor neurons that project to the contralateral paramedian pontine reticular formation (PPRF), which is the organizing center for lateral gaze in the brain stem. The PPRF projects to the ipsilateral abducens nucleus (causing eye abduction on that side). There are fibers extending from the abducens nucleus, which is located in the caudal pons, to the contralateral oculomotor nucleus of the midbrain. The projection pathway is the medial longitudinal fasciculus (MLF). The oculomotor nucleus then activates the medial rectus, adducting the eye in order to follow the abducting eye. This is illustrated schematically in figure 4-9 for voluntary horizontal gaze to the left. Damage to the frontal eye-fields will initially prevent voluntary gaze away from the injured frontal lobe. However, that improves with time. Damage to the PPRF will abolish the ability to look toward the side of the lesion. Damage to the MLF produces the curious finding of internuclear ophthalmoplegia in which the patient will be able to abduct the eye, but the adducting eye will not follow. Additionally, there will be some nystagmus in the abducting eye. Vertical gaze (Fig. 4-10) does not have one center in the cerebral cortex. Diffuse degeneration of the cortex (such as with dementia) can diminish the ability to move the eyes vertically (particularly upward). There is a brain stem center for vertical gaze (in the

midbrain the rostral interstitial nucleus [of Cajal]). Degeneration of this nucleus (such as can occur in rare conditions like progressive supranuclear palsy) can abolish the ability to look up or down. Additionally, there are connections between the two sides that traverse the posterior commissure. Pressure on the dorsum of the midbrain, such as by a pineal tumor, can interrupt these fibers and prevent upgaze (Parinaud syndrome). Smooth tracking eye movements are mediated through a more circuitous pathway that includes the visual association areas (necessary in order to fix interest on a visual target) and the cerebellum. Cerebellar damage often produces jerky, uncoordinated movements of the eyes.

Pupillary function
The iris receives both sympathetic and parasympathetic innervation: (1) the sympathetic nerves innervate the pupillary dilator muscles; and (2) the parasympathetic nerve fibers (from CN III) innervate the pupillary constrictor (sphincter) muscles as well as the ciliary apparatus for lens accommodation. Figures 4-7 and 4-8 show the origins and courses of these two systems. During the normal waking state the sympathetics and parasympathetics are tonically active. They also mediate reflexes depending in part on emotionality and ambient lighting. Darkness increases sympathetic tone and produces pupillodilation. Increased light produces increased parasympathetic tone and therefore pupilloconstriction (this also accompanies accommodation for near vision). During sleep, sympathetic tone is depressed and the pupils are small. Normal waking pupil size with average ambient illumination is 2 to 6 mm. With age, the average size of the pupil decreases. Approximately 25% of individuals have asymmetric pupils (anisocoria), with a difference of usually less than 0.5 mm in diameter. This must be kept in mind when attributing asymmetry to disease, particularly if there are no other signs of neurologic dysfunction. At the bedside, the first step in evaluating pupil dysfunction is observation of the resting size and shape. A small pupil suggests sympathetic dysfunction; a large pupil, parasympathetic dysfunction. Loss of both systems would leave one with a nonreactive, midposition pupil, 4-7 mm in diameter, with the size varying from individual to individual. This is seen most often in persons with lesions that destroy the midbrain (see Chap. 24).

Pupillary reflexes
Next, the integrity of the papillary reflex section is evaluated. Parasympathetic function is tested by having the patient accommodate, first looking at a distant object, which tends to dilate the pupils and then quickly looking at a near object, which should cause the pupils to constrict. Additionally, the pupils constrict when the patient is asked to converge, which is most easily done by having them look at their nose. There are rare conditions damaging the pretectal region that differentially affect the constriction produced by convergence from that produced by accomodation. More common is the loss of the light reflex with preservation of accommodation and convergence pupilloconstriction (this has been termed the Argyll-Robertson pupil). This may be caused by lesions in the peripheral autonomic nervous system or lesions in the pretectal regions of the midbrain. Variable amounts of sympathetic involvement are usually present, leaving the pupil small in the resting state. Although this was commonly associated with tertiary syphilis in the past, the Argyll-Robertson pupil is seen most often associated with the autonomic neuropathy of diabetes mellitus. The light reflex is tested by illuminating first one eye and then the other. Both the direct reaction (constriction in the illuminated eye) and the consensual reaction (constriction in the opposite eye) should be observed. The direct and consensual responses are equal in intensity because of equal bilateral input to the pretectal region and Edinger-Westphal nuclei from each retina (see Fig. 4-7). Pupillodilation, which can be tested by darkening the room or simply shading the eye, occurs due to activation of the sympathetic nervous system, with associated parasympathetic inhibition. A sudden noxious stimulus, such as a pinch (particularly to the neck or upper thorax), causes active bilateral pupillodilation. This is called the ciliospinal reflex and depends predominantly on the integrity of the sensory nerve fibers from the area, the upper thoracic sympathetic motor neurons (T1- T3 lateral horn) and the ascending cervical sympathetic chain (see Fig. 4-8). Interruption of the descending sympathetic pathways in the brain stem frequently has no effect on the reflex. Therefore, if the patient has a constricted pupil presumably secondary to loss of sympathetic tone, absence of the ciliospinal reflex suggests peripheral sympathetic denervation or, if other neurologic signs are present, damage to the upper thoracic spinal cord. Presence of the reflex despite depressed resting sympathetic tone suggests damage to the descending central sympathetic pathways. Horner's syndrome is a constellation of signs caused by lesions in the sympathetic system. Sweating is depressed in the face on the side of the denervation, the upper eyelid

becomes slightly ptotic and the lower lid is slightly elevated due to denervation of Muller's muscles (the smooth muscles that cause a small amount of lid-opening tone during alertness). Vasodilation is transiently seen over the ipsilateral face, and the face may be flushed and warm. These abnormalities, in addition to pupilloconstriction, are seen in conjunction with peripheral cervical sympathetic system damage. The final neuron in the cervicocranial sympathetic pathway arises in the superior cervical ganglion and sends its axons to the head as plexuses surrounding the internal and external carotid arteries. Lesions involving the internal carotid artery plexus (as in the middle-ear region) cause miosis (a small pupil) and ptosis and loss of sweating only in the forehead region - the area of the face supplied by the internal carotid system. Lesions of the superior cervical ganglion cause the same problems, except that loss of sweating occurs over the whole side of the face. Destruction of the external carotid plexus causes sweating loss over the face that spares the forehead, without pupillary or eyelid changes. Lesions of the lower portion of the cervical sympathetic chain (e.g., carcinoma of thyroid) cause a Horner's syndrome with loss of sweating in the face and neck, and if the lesion is at the thoracic outlet (such as tumors of the apex of the lung), loss of sweating extends to the upper extremity. Lesions of the brainstem and cervical spinal cord descending sympathetic pathways cause a Horner's syndrome with depression of sweating over the whole side of the body. Lesions of the spinal cord below T1- T3 cause a loss of sweating below the level of the lesion but no Horner's syndrome. Testing for sweating defects can therefore be very useful in localization of the lesion. A simple, but messy way to test sweating is to warm the patient and watch for asymmetrical loss of sweating using starch and iodine. The parts to be tested are painted with an iodine preparation (e.g., forehead, cheek, neck, hand and foot) and then when they are dry, the areas are dusted with starch. When the patient sweats after being warmed with blankets (covering the tested areas with plastic is useful), the iodine runs into the starch and blackens it. Asymmetries are relatively easy to observe.

Amblyopia
Before concluding this discussion of eye movements it would be appropriate to say a few words about "amblyopia" (literally, "dim eye"). This is a condition in which one eye obviously drifts off target (some have called it a "wandering eye"). However, the patient is unaware of this and does not see double. This is most serious in children and occurs for one of two reasons. First of all, it may occur due to severe muscle weakness or scarring. In this case the child cannot keep the two eyes fixed on the same target. The other cause is poor vision (usually in one eye). The

reason that there is no double vision is that the brain "turns off" input from the bad eye. The reason this is so bad in young children is that, up until late childhood, functionally "turned off" synapses will actually loose their connections with neurons at the level of the visual cortex. These synapses will be replaced by synapses of fibers from the intact eye and the patient will become permanently blind in that eye. "Turning off" an eye for one continuous month for each year of life (i.e., for 5 straight months in a 5-year old) is enough to cause permanent blindness. This does not happen in adolescence or adulthood because synapses have stabilized. Interestingly, the pupillary light reflex is unaffected since the projections from the retina to the pretectum are intact. The treatment is to force the patient to use the eye at least part of the day (while providing as much visual correction as possible for the affected eye). This is often done by patching the "good eye" during school time (in a more controlled environment).

References
Brodal, A.: Neurological Anatomy in Relation to Clinical Medicine, ed. 2. New

York, Oxford University Press, 1969.

Cogan, D.G.: Neurology of the Ocular Muscles, ed. 2. Springfield, IL, Charles C.

Thomas, Publisher, 1956.

Monrad-Krohn, G.H., Refsum, S.: The Clinical Examination of the Nervous

System, ed. 12. London, H.K. Lewis & Co., 1964.

Spillane, J.D.: The Atlas of Clinical Neurology, ed. 2. New York, Oxford

University Press, 1975.

Walsh, F.B, Hoyt, W.F.: Clinical Neuro-ophthalmology, ed. 3. Baltimore,

Williams & Wilkins Co., 1969.

Questions
Define the following terms:

strabismus, abduction, adduction, elevation, depression, convergence, accomodation, diplopia, meiosis, mydriasis, myopia, hyperopia, conjugate, consensual, extraocular, amblyopia, ptosis, anisocorea.
Strabismus is a position of the eyes where they are not directed at the same target (in some parts of the country this is termed a "squint"). Abduction is bringing the pupil away from the nose.

Adduction is bringing the pupil toward the nose. Elevation is moving the pupil above the horizon. Depression is moving the pupil below the horizon. Convergence is directing both eyes toward the nose. Accomodation is a combination of convergence, pupil constriction and change in lens shape to permit focus on a near object. Diplopia is double vision. This can be horizontal, vertical or skew. Meiosis is constriction of the pupil. Mydriasis is dialation of the pupil. Myopia is an inability to see at distance ("nearsighted") with light focusing in front of the retina. Hyperopia is an inability to see close up ("farsighted") with light behind the retina. Conjugate - "together". That is, the eyes moving in parallel to keep images focused on the same part of each retina (preventing diplopia). Consensual means "happening on both sides at the same time". A consensual reflex is one where the response is bilateral when the stimulus is unilateral (such as the papillary light reflex). Extraocular means outside the eye and specifically is used to describe the muscles that attach to the outside of the eyeball and move it. Amblyopia literally means "dim eye". This is a drifting or "lazy" eye that usually happens because one eye has bad vision. The brain often "turns off" control of that eye and the eye drifts. The patient usually does not have diplopia because input from that eye is turned off. In one of the most remarkable illustrations of plasticity, the eye can become permanently blind in children if this is not treated. Ptosis is a drooping of the upper eyelid. Anisocorea is an inequality of pupil size. This is usually not clinically significant unless it reaches one millimeter in difference.

4-1. Which muscles would be active in the right and left eye when looking up and to the right?
Answer 4-1. In the right eye the lateral rectus and the superior rectus would be the prime movers, while in the left eye, the medial rectus and the inferior oblique would be most active.

4-2. Which muscles would be active in the right and left eye when looking down and to the left?
Answer 4-2. In the right eye the medial rectus and the superior oblique would be the prime movers, while in the left eye, the lateral rectus and the inferior rectus would be most active.

4-3. What position will the patient's head assume (in order to prevent diplopia) if their right trochlear nerve is damaged?
Answer 4-3. Head tilted to the left and chin turned slightly to the right ("cockeyed").

4-4. When a patient has double vision, in which position will they have the furthest separation of the images?
Answer 4-4. The images will be furthest apart when the eyes look in the direction that the weak muscle is most active.

4-5. What is the significance of horizontal diplopia (where the images are side-by-side) as opposed to vertical diplopia?
Answer 4-5. Horizontal diplopia results from weakness of the lateral or medial rectus muscles; vertical diplopia is due to weakness of one of the other muscles.

4-6. Which eye (the one that is moving normally or the weak one) will see the image that is furthest displaced from the center of vision?
Answer 4-6. The "bad eye" sees the image that is furthest toward the periphery of vision.

4-7. Where is the cortical center that controls lateral gaze? Where is the lateral gaze center in the brain stem?
Answer 4-7. Lateral gaze centers include the frontal eye fields in the frontal lobes of the cerebral cortex and the paramedian pontine reticular formation.

4-8. Is there a vertical gaze center in the cerebral cortex? Is there a brain stem vertical gaze center?
Answer 4-8. Vertical gaze is a diffuse cerebral cortical phenomenon, while there is a vertical gaze center in the rostral midbrain (the rostral interstitial nucleus).

4-9. What are the potential causes of ptosis?

Answer 4-9. Ptosis may be due to weakness of the levator palpebrae muscle (or CNIII damage) or due to damage to the sympathetics (due to weakness of the small, superior tarsal muscle).

4-10. What are the components of Horner's syndrome?

Answer 4-10. Horner's syndrome (ptosis, meiosis, anhidrosis and possibly flushing) is from damage to sympathetics anywhere along their course.

4-11. What are the functions of sympathetic and parasympathetic nerves to the orbit?
Answer 4-11. Sympathetics dilate the pupil, parasympathetics (CN III) constrict the pupil to light and accommodation; there is balance between sympathetics and parasympathetics.

4-12. Where is the brain stem center for the pupillary light reflex?

Chapter 5: Facial sensations & movements

In this chapter, the functions of the trigeminal (CN V) and facial (CN VII) nerves will be discussed. Symptoms of damage to the trigeminal system are mainly loss of sensation in the face, although the mandibular division of the trigeminal nerve also controls jaw motion. Damage to the facial nerve mainly manifests as weakness of muscles of facial expression, although it may also affect taste sensation in the anterior part of the tongue. It is critical to distinguish damage of the facial nerve from damage to the connections from the cerebral cortex to the brain stem, which selectively weakens muscles of the lower portion of the face, contralateral to the side of damage.

V. Trigeminal Nerve
The three divisions of the fifth nerve (I. Ophthalmic, II. Maxillary, and III. Mandibular) are the source for somatic sensation over the entire face (Figs. 5-1 and 5-2), the eye, the nasal passages and the oral cavity.

Facial sensations
Facial sensation can be tested simply at the bedside by having the patient close their eyes and respond affirmatively to touch with a light wisp of cotton over the three divisions of the trigeminal nerve. The patient should be asked to compare the perception on the two sides. Pain perception as tested by a pin can be similarly checked, although temperature sensation (which is mediated by the same pathways, can replace the use of a pin. Sensory testing is, by nature, subjective (i.e., the examiner depends on the reliability of the subject). It is important to define the pattern and distribution of sensory alteration since that can go a long way to both localizing the lesion and also validating the sensory findings. The patient with hysterical or feigned sensory loss in the face frequently has bizarre perceptive patterns such as a hairline or perfect midline demarcation of hyposensitivity. Neither pattern can be explained on the basis of the central or peripheral distribution of the trigeminal system (see Fig. 5-1). Additionally, the inability to detect the vibrations of a tuning fork placed on one side of the head (when the other side can detect it) is not physiological since the entire head vibrates. The subjective tests of facial sensation can be objectified by examining certain reflex responses such as the corneal reflex (where the eye briskly closes in response to a wisp of cotton touching the cornea). Asymmetries of this are a good sign of sensory impairment at least in the distribution of the ophthalmic division of the trigeminal nerve (see below).

Jaw movements
The temporalis, masseter, and pterygoid muscles (muscles of mastication) are supplied by the motor division of the mandibular branch of cranial nerve V and subserve jaw movement. Supranuclear innervation of these muscles (hemispheric and brainstem pyramidal and extrapyramidal systems) is essentially symmetrically bilateral. A unilateral lesion above the level of the fifth-nerve motor nucleus, therefore, does not cause any obvious weakness of jaw motion. Large bilateral lesions of the hemisphere or brain stem (above the fifth-nerve nucleus) can cause bilateral weakness of voluntary jaw movement. If the bilateral involvement lies above the brain stem, very basic brain stemmediated chewing reflexes may remain and actually become hyperactive. The jaw jerk reflex is a muscle stretch reflex in which both the sensory and motor nerve fibers are contained in the trigeminal nerve. This is elicited by lightly tapping the relaxed open jaw in a downward direction. This would be lost after trigeminal nerve damage and hyperactive with injury above the pons (see Chap. 10). The paired temporalis and masseter muscles function in jaw closure, and the medial pterygoid muscle closes the jaw and moves it from side-to-side (grinding motion). The lateral pterygoid muscles (along with some of the upper neck muscles) open the jaw in concert with a downward and opposing inward motion (Fig. 5-3). When one lateral pterygoid is weak, the jaw deviates toward the weak side on opening, with the inward vector of the opposite pterygoid being unopposed (Fig. 5-4). Observation of temporal region for atrophy and palpation of the symmetry of muscle bulk and tension during tight jaw closure test the innervation of the temporalis and masseter muscles.

Corneal reflex
The corneal reflex is mediated by sensory fibers in the trigeminal nerve and motor fibers in the facial nerve. It consists of a bilateral blink response when the edge of the cornea is touched from the side with a wisp of cotton. The examiner should approach from the extreme corner of the eye in order to avoid a visually evoked blink response. It is a useful and objective test for evaluating simultaneously the ophthalmic division of the fifthnerve (the afferent limb) and the seventh-nerve motor innervation of the orbicularis oculi (the efferent limb). Both the eye that is touched and the opposite eye are observed since they should both close equally and consensually (a consensual reflex is one in which the motor response is bilateral to a unilateral stimulus. The corneal reflex is a sensitive and objective indicator of fifth- and seventh-nerve dysfunction. A good example of dysfunction occurs with eight-nerve tumors (acoustic neuromas), which comprise

approximately 5% of all intracranial tumors in adults (see Fig. 5-2). Patients may present with unilateral hearing loss, and on routine neurologic evaluation the only other indication of involvement may be depression of the ipsilateral direct and contralateral consensual corneal reflex. This results from pressure by the tumor, which lies in the angle between the cerebellum and pons, on the superficially positioned descending tract and nucleus of the trigeminal nerve (which mediates pain and temperature sense from the face). If the depression of the reflex were secondary to seventh-nerve hypofunction, only the direct response would be depressed; the contralateral consensual response would be full because the sensory limb of the reflex, mediated by the trigeminal nerve, would be intact.

Trigeminal neuralgia
An instructive example of trigeminal nerve dysfunction is trigeminal neuralgia (tic douloureux), an irritation of the nerve that probably occurs due to contact with anomalous intracranial blood vessels. This process causes severe paroxysms of pain in one or more divisions of the trigeminal nerve, with the maxillary division being most often affected and the ophthalmic least. In the past, surgeons attempted to cut the various peripheral branches of the trigeminal root. However, this would result in "numbness" and pain would usually return some months later. At one time, complete damage to the root became a popular form of permanent cure. A great difficulty with both of these procedures is that the area of anesthesia can become spontaneously painful (denervation hypersensitivity, a form of neuropathic pain). Also, the eye and face can be damaged because of the loss of sensitivity. These destructive surgical procedures have fallen out of favor. Fortunately, various medications (mostly in the family of anticonvulsants) suppress the excess excitability in the trigeminal neurons and are successful in relieving tic in many persons for long periods of time. Nonetheless, there are patients who do not get adequate response to medications and several other interventions can be successful in relieving these medically refractory patients. Glycerol, injected into the region around the trigeminal ganglion, often produces relief that extends for years after the procedure (it is thought to produce some selective nerve damage). A radiofrequency probe can be placed into the trigeminal ganglion (percutaneously, through the foramen ovale) and selective lesions can be made to the nerve fibers from the painful region of the face. The most elegant surgical treatment (but most invasive) involves approaching the trigeminal nerve from an occipital craniotomy and placing some Teflon between any irritating

arteries and the trigeminal nerve root. This often results in permanent relief of the symptoms.

VII. Facial Nerve

Most of the facial nerve is comprised of motor innervation of the muscles of facial expression. In addition, it subserves several other functions including: taste perception from the anterior two-thirds of the tongue; perception of cutaneous stimuli in the external auditory canal and over part of the pinna and mastoid region; innervation of the stapedius muscle in the middle ear; and innervation of the lacrimal gland and two of the salivary glands (the submaxillary and submandibular). Many of these functions are difficult to test and more difficult to quantify (such as salivation and lacrimation (Fig. 5-5). However, some of these functions can be tested and give clues as to the location of facial nerve damage. Taste in the anterior tongue is tested with application of a thick sugar solution on a Q-tip to the protruded tongue. Care must be taken to prevent this from spreading to the other side and the mouth must be rinsed out thoroughly between trials. The chorda tympani (the branch mediating this sensation) leaves the parent nerve, crossing through the middle ear (where it can also be damaged by severe infections, etc). Loss of function of the stapedius muscle may reflect as "hyperacusis", i.e., perception of sound as excessively loud an irritating on the side of damage. This branch also arises at the level of the middle ear.

Facial expression
The great majority of facial nerve fibers are involved in producing facial expressions. Careful observation of the patient's face during conversation and at rest almost always reveals facial weakness. Additionally, the face may "droop" on the side of damage due to the effects of gravity. The nerve can be further tested by: having the patient close their eyes and lips tightly (the force of closure can be felt by manually trying to open them); having the patient grimace (show their teeth); having the patient look up (elevating the eyebrows and creasing the forehead); and also having the patient fill their cheeks with air with their lips tightly pursed. If one or both sides of the face are weak, s/he will have difficulty holding the air in. Tapping each cheek accentuates the difficulty on the appropriate side. The most common cause of facial weakness is Bell's palsy, an idiopathic condition that may result from viral infection-induced inflammatory swelling of the facial nerve in its canal. Since the canal is very long and tight, swelling can put pressure on the nerve,

resulting in damage either by direct effects or by impairing blood flow in the nerve. In some cases, facial palsy is produced by a very clear viral infection with Herpes Zoster, often associated with ear pain and vesicles on the tympanic membrane. Lyme disease also has a proclivity to produce facial palsy, sometimes bilateral. The hallmark of peripheral facial palsy is that it involves the entire side of the face, including weakness of the forehead muscles as well as those around the eye and mouth. This is because fibers to all of these regions of the face are packed together in the facial canal. Most cases of uncomplicated Bell's palsy recover quite well. In its most severe form, infarction of the nerve may occur with a prolonged and not infrequently incomplete process of regeneration. This is more common when a longer course of the nerve is affected, accompanied by ageusia (loss of taste) and hyperacusis. The facial nerve begins at the facial motor nucleus of the caudal pons. It is not common to damage this nucleus and due to the proximity of many sensory and motor pathways running through the brain stem, there are almost always other signs of neurologic damage (hemiparesis, hemihypesthesia, gaze palsy, etc) when the facial nerve is affected here. It should be obvious that face movement is under voluntary control. However, it is also under control of the limbic system, where strong emotions can be seen in face involuntarily. Accordingly, there is more than one pathway for "supranuclear" control of the face and these pathways can be damaged independently. Corticobulbar (pyramidal) projections from the motor cortex (precentral gyrus) through the genu of the internal capsule are the major substrate for voluntary facial movement (Fig. 5-6). The cerebral cortical projections to the facial motor neurons innervating the upper face are essentially bilateral (i.e., each cortical hemisphere provides innervation to both sides). Therefore, unilateral lesions (such as a stroke affecting one hemisphere or the internal capsule) will not produce weakness of the upper face muscles. On the other hand, facial motor neurons that innervate the muscles of the lower face receive input largely from the contralateral hemisphere (i.e., the right hemisphere activates motor neurons of the left facial nucleus, and vice-versa). Therefore, a lesion involving the right motor cortex (e.g., carotid-middle cerebral arterial system occlusion and hemispheric infarction) causes a weakness of voluntary left lower facial movement that is especially noticeable while the patient is talking, grimacing (usually elicited by asking the patient to bare their teeth or gums), or resting. In the latter instance, the corner of the mouth droops and there may be some widening of the palpebral fissure (eye) (Fig. 5-7). On the other hand, the forehead is normally creased when a person raises their eyebrows or looks toward the ceiling. This distinguishes the "supranuclear" weakness of the face from the weakness of

the whole side of the face, due to damage of the peripheral facial nerve, as seen with Bell's palsy. Interestingly, despite severe weakness around the mouth with "supranuclear facial palsy", the mouth may actually move more than normal with emotional triggers (hypermimia, Fig. 5-7). This illustrates that limbic motor pathways (governing postures and movements in response to strong emotion) are distinct from the more usual motor pathways that we employ for normal voluntary movements. When there is bilateral damage to voluntary motor pathways, the face may be markedly overexpressive and may not actually reflect the patient's consciously perceived emotions. This is termed a "pseudobulbar affect".

Somatic sensation
The facial nerve has only a very small cutaneous distribution to the skin of the external auditory canal and over the tympanic membrane, where it overlaps with the small somatic branches of cranial nerves IX, X, and possibly V. Additionally, nerve VII variably supplies small branches to the ear lobe and the mastoid, which overlaps with the distributions of the trigeminal nerve and cervical nerves 2 and 3. It is not surprising with the considerable overlap of dermatomes that sensory testing seldom reveals hypoesthesia when the facial nerve is damaged. However, patients with Bell's palsy may complain of pain in the external canal and over the mastoid region due to irritation of these nerve fibers. Herpes zoster infection may afflict the geniculate ganglion (the sensory ganglion of the facial nerve) and manifests itself as pain and vesicular eruption over the preceding distribution. Facial weakness or paralysis is common with geniculate zoster due to swelling.

References
Brodal, A.: Neurological Anatomy in Relation to Clinical Medicine, ed. 2. New

York, Oxford University Press, 1969.

Cogan, D.G.: Neurology of the Ocular Muscles, ed. 2. Springfield, IL, Charles C.

Thomas, Publisher, 1956.

Monrad-Krohn, G.H., Refsum, S.: The Clinical Examination of the Nervous

System, ed. 12. London, H.K. Lewis & Co., 1964.

Spillane, J.D.: The Atlas of Clinical Neurology, ed. 2. New York, Oxford

University Press, 1975.

 Walsh, F.B, Hoyt, W.F.: Clinical Neuro-ophthalmology, ed. 3. Baltimore,

Williams & Wilkins Co., 1969.

Questions
Define the following terms:

hyperacusis, agusia.
Hyperacusis is the excessive perception of sound. Agusia is the loss of taste perception.

5-1. Which division of the trigeminal nerve has motor fibers?

Answer 5-1. Only the mandibular division of the trigeminal nerve has motor fibers (to the muscles of mastication).

5-2. What are some good ways to distinguish hysterical sensory loss on the face?
Answer 5-2. The trigeminal nerve does not follow artificial lines on the head (hairline, jawline), and there is no lesion that will abolish the ability of the patient to detect a vibrating tuning fork placed on a bony prominence of the skull. The corneal reflex may be useful as well.

5-3. Where is the trigeminal ganglion located?

Answer 5-3. The trigeminal ganglion is lateral to the sella turcica/pituitary and in wall of cavernous sinus.

5-4. Where does the trigeminal nerve root enter the brain?
Answer 5-4. CN V enters the pons.

5-5. What modalities would test the integrity of the spinal tract of the trigeminal nerve?
Answer 5-5. Pin and temperature sense.

5-6. Where do pain and temperature nerve fibers in the trigeminal nerve run after entering the pons?
Answer 5-6. Pain and temperature fibers run caudally through the lateral brain stem to reach the spinal nucleus of V in the medulla and upper spinal cord; lateral brain stem lesions can block ipsilateral pain from face.

5-7. What is the pathway of the corneal reflex?

Answer 5-7. The sensory llimb of the corneal reflex is the ophthalmic division of the trigeminal nerve and the motor limb is the facial nerve. The response is consensual.

5-8. What are the symptoms of Bell's palsy?

Answer 5-8. Bell's palsy damages the facial nerve in the facial canal and weakens all muscles of facial expression on the side of lesion. Depending on where the damage occurs, there may be hyperacusis or loss of taste on that side of the tongue.

5-9. How can you distinguish weakness of the face that is due to damage to the brain (such as with a stroke) from weakness due to damage of the facial nerve?
Answer 5-9. Damage to corticobulbar fibers (from cortex to the pons) will produce supranuclear weakness of the lower face (sparing of forehead) on the contralateral side, while damage to the nerve should produce weakness of all muscles of facial expression on that side.

5-10. Describe the reflex arc of the jaw-jerk reflex.

Chapter 6 - Auditory & Vestibular Function

In this chapter, the functions and clinical examination of the vestibulocochlear nerve (CN VIII) and its central connections will be discussed. The two distinct functions are in hearing and in control of balance. In the case of the auditory part of CN VIII, the symptoms are deafness or tinnitus (ringing in the ears). In the case of the vestibular part of CN VIII, the symptoms are vertigo or imbalance, although visual disturbance when moving may also be a complaint.

Auditory function

Testing
The vast majority of hearing problems result from peripheral disease, i.e., involvement of the eighth nerve or inner ear. Testing of the peripheral system at the bedside is simple and rewarding. For screening persons who do not complain of hearing loss, asking them to compare the sound of rustling fingers or a ticking watch in the two ears is a useful test of acuity. This, combined with the Weber test (see below), is adequate. To this might be added the use a whispered voice, which represents midrange frequencies that frequently are involved in neural deafness. Two basic instruments can aid in testing the auditory system: a C512 tuning fork (C256 is adequate but not as sensitive; C128 is inadequate except for testing for hyperacusis and cutaneous and bony vibratory perception), and a mechanical watch (watch-ticking is in the 1,500 cps range). The watch is placed next to the patient's ear and gradually moved away. The distance at which the patient ceases to hear the tick is noted and compared with the distance from the opposite side. If the examiner has normal hearing, a useful comparison can be made. High-tone deafness is measured by this test. The C512 (or C256) fork is then used to test for lower tone falloff and, more

important, to determine whether hearing loss is caused by defects in the conduction system (conductive deafness) or by damage to the inner ear-auditory nerve system (sensorineural deafness). This distinction is important since different types of conditions produce these types of deafness and the Weber test and Rinne test permit bedside differentiation of these conditions. More detailed clinical evaluation including special audiometric testing is carried out in otolaryngological laboratories and can be very useful in differentiating cochlear (inner ear) disease from direct eighth-nerve involvement.

Weber test
Both the Weber and Rinne tests are most valuable in the patient with a documented hearing loss (see above). These tests are particularly focused on determining whether the loss is sensorineural or conductive. In the Weber test, the stem of a tuning fork is placed gently against a midline structure of the skull (i.e., the maxillary incisor teeth or vertex of the cranium or forehead) and the patient is asked where s/he hears the sound. Sound is transmitted to both ears through the air but particularly through the vibrations of the bones of the skull. If sound is transmitted to both sides equally, the sound is heard in the midline and it can be presumed that the conduction and neural apparatus is intact. With neural deafness, the sound transmits best to the normal side and the patient lateralizes the sound to that side. With conduction deafness, sound transmits best to the side of the deafness. This is thought to occur because ambient sound is prevented from getting to the cochlea on the blocked side. This causes the nervous system to amplify sounds on that side by sensitizing cochlear transduction. In conductive deafness, the patient hears the tuning fork better on the affected side because sound on the normal side is relatively depressed and extinguished. You can demonstrate this yourself by plugging an ear with your finger, causing conduction deafness, and then humming. The sound will be heard better on the occluded side. By the way, you notice the effects of ambient sound on hearing acuity when you must talk to a friend at the top of your voice in a noisy, crowded room and then continue talking and walk into a silent room where you find yourselves shouting at each other.

Rinne test
In some cases the Rinne test can provide some additional information. This tests both bone and air conduction. The examiner places the butt of a vibrating tuning fork on the mastoid region, and when the patient ceases to hear the vibration, the examiner places the tines close to the external auditory meatus to check air conduction. Vibrations

perceived through air are heard twice as long as those perceived through bone, so the normal individual reports, for example, hearing the bone vibration for 30 seconds and then continues to hear the vibration through air for another 30-60 seconds altogether. If there is conductive deafness, bony conduction is either normal or slightly enhanced, whereas air conduction is decreased. If there is neural deafness, both bone conduction and air conduction are equally suppressed. As with the watch tick, the examiner should compare the ability of both sides to perceive the fork. A comparison of the patient's ability to perceive the fork, as well as the watch tick, with the examiner's ability is also useful (Schwabach test).

Pathology
Conductive deafness results from processes that occlude the sound conduction pathways (the external auditory canal, tympanic membrane, middle ear or the ossicles). These may be easily detected when they result from conditions that are visible with the otoscope (blockage of the external canal or rupture or scarring of the tympanic membrane). Some conditions of the middle ear, such as supperative otitis media (where there is pressure in the middle ear due to infection), or serous otitis media (where there is obstruction of the auditory tube with a vacuum in the middle ear and retraction of the ear drum and accumulation of some serous fluid), may be visible, as well. Other conditions, such as otosclerosis, which results in progressive fusion of the ossicles, may not be detectible by observation and may require more testing. There are many conditions that can damage the delicate hair cells of the organ of Corti or the auditory component of CN VIII. These conditions produce "sensorineural" hearing loss. By far, the most common cause of this is exposure to loud noises, which typically affects high-tone hearing. Other conditions should be considered in acute sensorineural hearing loss, including: infectious (usually viral) or inflammatory attack on the inner ear; ischemia (the labyrinthian artery usually arises from the anterior inferior cerebellar artery); or trauma (especially with fracture of the skull base). A more insidious loss of hearing can occur with Meniere syndrome. This condition occurs due to buildup of pressure in the inner ear due to obstructed resorption of endolymph. This pressure can cause "blowouts" of the membranes, with attacks of sudden vertigo that improves over hours (see the vestibular system below). It also affects hearing, with tinnitus (usually a buzz or hum) and hearing loss (usually of low tones). The hearing loss can be detected even in between attacks of vertigo. While damage to the cochlear nuclei, located at the lateral aspect of the pontomedullary junction (where CN VIII enters the brain) can cause unilateral hearing

loss, damage to other regions of the central nervous system is unlikely to cause recognizable hearing loss. Beyond the cochlear nuclei the auditory system makes multiple decussations in the brainstem up to the level of the medial geniculate, and therefore auditory signals are bilaterally distributed in the brainstem, thalamus and primary auditory cortex (Heschl's gyri of the posterior-superior temporal lobes). Significant loss of hearing therefore, does not occur following unilateral lesions of the auditory system above the cochlear nuclei. Bilateral lesions may affect hearing but are usually so devastating as to preclude clinical testing of hearing (there are laboratory tests of hearing, described in Chap. 23, that may help localize brain stem lesions and do not require patient cooperation). Damage to the central nervous system occasionally impairs localization of sound, even without affecting acuity. For example, localization difficulty can occur with large unilateral cerebral cortical lesions. Deficits in sound localization is most likely to occur when the primary auditory cortex is involved but is less frequently observed with large lesions of the frontal and/or parietal cortex. Such patients are unable, with eyes closed, to localize an auditory stimulus with their eyes closed in the auditory field opposite the damaged hemisphere. This can be tested at the bedside by asking the patient to reach for a sound (such as snapping fingers) with their eyes closed. Double simultaneous stimulation (DSS) can also be useful; the patient may extinguish (ignore) the stimulus opposite the lesioned hemisphere (some of this may be a neglect phenomenon, especially when the frontal and parietal lobes are involved).

Tinnutus
Tinnitus (ringing or buzzing in the ears) is a common complaint. It is usually due to some damage of cochlear hair cells, with spontaneous nerve activity being produced by the damaged cells. High-pitched tinnitus is most commonly due to damage to cells at the base of the cochlea due to excessive sound exposure. However, it can also result from virtually any cause of inner ear or CN VIII damage. Low pitch tinnitus (buzzing or humming) is less common in general, but may result from excessive pressure in the inner ear (conditions such as Meniere syndrome). Pulsatile tinnitus is most often due to turbulence in the carotid blood flow. This can be normal but it can also occur with carotid bruits (turbulence due to arterial narrowing). Tinnitus that is not accompanied by hearing loss can result from medications (notably high doses of aspirin), but often defies diagnosis. There is no cure for tinnitus (unless a curable cause of inner ear damage is identified), although it can occasionally be masked with other sounds.

Vestibular function
The vestibular apparatus of the inner ear is specialized to detect movement of the head and, to a lesser extent, position in space. The vestibular portion of CN VIII conveys these signals to the vestibular complex of the dorsolateral brainstem at the pontomedullary junction and also to the vestibulocerebellum (Fig. 6-1). The chief symptom of damage to the system is vertigo, i.e., the illusion of movement. Stabilization of the eyes is a critical function of the vestibular system. It is largely through this effect on eye movement that we can objectively evaluate the vestibular system. The fundamental reflex that we will discuss is the vestibulo-ocular reflex (VOR), which moves the eyes opposite to head movement in order to stabilize vision. Without a vestibular system, we would be unable to see anything clearly while the head is moving. There is another reflex, the fixation reflex, that we will discuss as well. This reflex attempts to fix the image of an object on the retina. Between these two reflexes our vision is actually quite good even when the head is moving (try reading a sign while nodding your head or shaking your head "no" and you are using these reflexes, predominantly the VOR). We are particularly good at detecting angular acceleration (i.e., spinning, pitching or tumbling). If you think about it, these are the movements that would be most important to compensate for, since they would tend to move your eyes off of a visual target. Smooth, linear motion would not tend to blur vision as much, nor would acceleration in a straight line (linear acceleration). The organs of the inner ears that detect angular acceleration are the cristae within the semicircular canals. Since the canals are at right angles to one another in three major planes, angular acceleration in any direction will move fluid in the particular canals that are in the plane of movement because of flow of endolymph (actually, in normal movements, the endolymph stays behind and the inner ear moves with the skull). At rest, the hair cells are tonically active, releasing transmitter that activates the peripheral end of the vestibular nerve fibers. The tonic activity on one side is balanced by the tonic activity in the other ear and the patient perceives that they are stable. Differential movement of fluid in one direction in the semicircular canal increases this activity in one ear and decreases it in the corresponding canal of the other ear, leading to a perception of movement and reflex movement of the eyes. Most diseases of the inner ear or vestibular nerve are destructive in nature, decreasing input from that ear. Therefore, the tonic firing level of the opposite canal system is no longer opposed and the patient perceives motion. Additionally, there will be VOR-induced eye movements that result from the brain attempting to move the eyes opposite to the direction of perceived motion. This will trigger competing reflexes (as

part of the visual fixation reflex) that will result in eye movement in an attempt to maintain a stable image. This to-and-fro motion is termed nystagmus. Actually, there are two major types of nystagmus, "jerk nystagmus" and "pendular nystagmus". The first type (and they type that is generated by the vestibular system) is" jerk nystagmus". In this type of nystagmus, there is relatively slow eye drift to one side produced by the VOR, with a fast compensatory "jerk" of the eyes to reacquire the visual target. Jerk nystagmus is named according to the direction of the fast movement, since this is the easiest to see. This type of nystagmus can normally be seen when an individual spins around. Here the nystagmus is initiated by the VOR produced by head movement. Spontaneous nystagmus is produced by vestibular damage because of the imbalance of inputs from the ears. Jerk nystagmus can also be elicited by the visual input of objects passing by rapidly (for example, if one stares out the side window of a moving vehicle with posts or trees flashing past). In this case, the nystagmus is elicited by the fixation reflex that is attempting to lock onto and track objects visually. This type of jerk nystagmus has been termed "optokinetic" nystagmus or "railway" nystagmus. The other major type of nystagmus is termed "pendular" nystagmus. This type of oscillation of the eyes does not have a fast and slow direction of movement but rather consists of an even motion from side to side. This is most often due to poor visual acuity (especially when young), and the fixation reflexes that stabilize the eyes are, therefore, poorly formed. This is more of a tremor of the eyes and does not reflect problems with the vestibular system. If the two inner ears are damaged symmetrically, there is little in the way of vertigo or nystagmus. If the horizontal canals are damaged, there is predominantly horizontal nystagmus (see Fig. 6-1), while damage to the anterior and posterior canals will tend to produce rotary nystagmus (clockwise or counterclockwise) due to the addition of a vertical component to the horizontal nystagmus. Damage to the inner ear does not produce vertical nystagmus. Rather, this suggests damage to the brain stem vestibular apparatus. As an illustration of what happens with unilateral vestibular damage, let's consider the effects of sudden loss of the right labyrinthine system, for example, as in vestibular neuronitis (a common affliction presumably viral in origin). In this example (Fig. 6-2), the normal response of the now unopposed opposite (left) horizontal canal system is to tonically drive the eyes conjugately to the right. In an alert individual, there is a reflex attempt to contain the abnormal tonic drive. This checking attempt is called the fast component and in combination with the tonic or slow component, forms the rhythmic to-and-fro movement - nystagmus. The tonic component encompasses the vestibular-

oculomotor brain stem systems, whereas the fast component depends on the integrity of the cerebral hemispheres. The right hemisphere, including cortex, basal ganglia, and diencephalon, is responsible for the fast component to the left and the left hemisphere for the fast component to the right, just as for voluntary and visual tracking horizontal gaze (see Figs. 4-5 and 4-6). With loss of hemispheric function and preservation of basic brain stem functions (e.g., in a coma from sedative overdose), the fast component becomes weak, irregular, and finally disappears, leaving only tonic deviation of the eyes following vestibular-oculomotor activation. With acute unilateral hemispheric depression, such as caused by a middle cerebral artery occlusion, the fast component to the opposite side is depressed. The tonic component is predominant during vestibularoculomotor activation and drives the eyes toward the side of the abnormal hemisphere, which is capable of little, if any, checking. It is important to note that the brain has compensatory mechanisms for damage to the vestibular system. Therefore, with chronic, slowly progressive disease such as an acoustic neuroma (a tumor arising from the neurolemmal sheaths of the eighth nerve at the internal auditory meatus), a person is much less likely to complain of vertigo or to have significant nystagmus. This is true also in persons following recovery from an acute destructive process despite the lack of effective function in the destroyed system. Compensation for even massive damage to an inner ear is quite effective. The lack of symptoms and signs is due to central compensation and in large part, though not entirely, depends on visual fixation. If a patient with chronic disease or compensated acute disease closes their eyes, the examiner may be able to detect the reappearance of the nystagmus by using and electrical test of eye position (the electronystagmogram) or simply by feeling the elevated corneas move through the closed lids. Vertigo usually does not reappear, which suggests that there are means other than visual for suppressing the illusion of movement. An excellent example of the suppression of nystagmus and vertigo is seen in the figure skater who is subjected to marked acceleration and deceleration of the horizontal endolymph-cristae systems during every spin. Using visual fixation (a fix on one object as long as possible while spinning), skaters learn to suppress after-spin nystagmus and vertigo almost entirely. Imagine what figure skating would like if this were not possible!

Examination
On examining a patient with suspected vestibular dysfunction, observation for nystagmus is of primary importance prior to formal testing. A person with, for example, acute right vestibular apparatus destructive disease has horizontal nystagmus with the

tonic component toward the diseased right side (release of the normal left) and the fast component toward the left (see Fig. 6-2). Usually s/he complains of vertigo (illusion of movement of self or environment), saying that the room is spinning in the direction of the fast component, to the left -- an illusion caused by the forced tonic movement of the eyes and retinae. This should be called object vertigo as opposed to subject vertigo, which is the sensation that the subject is spinning and which occurs almost exclusively with the eyes closed. Subject vertigo is the true vestibular illusion, unsuppressed by the retinal image. The patient usually complains that s/he feels s/he is rotating in the direction of the clinically observed fast component of the nystagmus. Testing of the inner ear is not a simple matter. Asking the patient to focus on your nose while using your hands to rapidly move the head to either side ("head thrust") can provide some information. Usually, individuals are able to maintain good focus if the inner ears are intact since the head thrust activates the VOR. Inability to maintain fixation when doing this indicates damage to the inner ear. Another test of vestibular function employs rotation of the patient in a spinning chair (a Barany chair). It is very awkward to do this in the clinic and creates considerable discomfort for the subject, particularly nausea. Additionally, it cannot test each ear individually (since the whole head is spinning and since both ears are moved simultaneously). Because of these limitations, the use of a spinning chair has largely been replaced by caloric testing, which is easier to carry out and tends to be less noxious. In addition, only one horizontal canal is involved and therefore evaluated in routine caloric testing, whereas both are involved in Barany rotation. Barany rotation is more useful for testing the vertical canals; this can also be done with bilateral simultaneous caloric testing with, however, some inconsistency in the results. Vertical canal testing is rarely necessary, so a rotating barber's chair need not be part of a physician's clinical armamentarium.

Caloric testing
Caloric testing is an elegant method for evaluating the integrity of the vestibular apparatus of each ear, independently. Caloric testing is carried out most simply by irrigating the external auditory canal (observed by otoscope to be unobstructed by wax, not infected, and with no tympanic perforation) with water warmer or colder than body temperature, the presumed resting temperature of the labyrinths. The differential warming or cooling of the horizontal semicircular canal where it lies closest to the external auditory canal causes a decrease or increase, respectively, in the specific gravity of the endolymph at that point. If the head is positioned so that the horizontal canal is

vertical (see the position of the canal in Fig. 6-3), significant convection currents are caused in the canal by the induced changes in specific gravity (Figs. 6-4 and 6-5). Vertical upward currents are caused by warming because of the decreased specific gravity and, with the patient supine, the current in the horizontal canal is toward the ampulla and crista (see Fig. 6-5). This direction of flow is excitatory to the crista, causing increased firing over the pathways diagrammed in Figures 6-1, 6-2, and 6-5. This results in vertigo (in which the patient feels that they are spinning toward the ear being irrigated with warm water), and there will be VOR-induced reflex movement of the eyes away from that ear. If the patient is awake and alert, the drift of vision that is produced by the VOR will result in a rapid corrective "jerk" of the eyes to try to keep them focused on a target. Nystagmus is the result (remember, nystagmus is named by the direction of the fast phase). Nystagmus "to the right" means nystagmus with the fast component to the right. In order to maintain clarity, many examiners use the term "right-beating" to clarify that they are referring to the direction of the fast phase. Cold-water irrigation will have opposite effects. With the horizontal canal in the vertical position (i.e., patient supine with their head on a slight pillow), cold-water irrigation increases the specific gravity of the endolymph closest to the external auditory canal. Therefore the fluid sinks and a current is created away from the crista/ampulla (see Fig. 6-5). This decreases the spontaneous firing of the ipsilateral horizontal canal vestibular system and causes an imbalance with the resting tone of the opposite horizontal canal system becoming dominant. The eyes are thus driven tonically toward the irrigated side and the checking or fast component is opposite in direction. This same nystagmus (and concomitant vertigo) is seen in persons with destructive lesions of the vestibular apparatus (see Fig. 62). In performing caloric tests with warm water, 20 cc of approximately 48 degrees C water (higher temperature is painful) is irrigated into the external auditory canal, which should be clear of wax, uninfected, and with no tympanic membrane perforation (Fig. 66). Each auditory canal should be irrigated separately for the same duration (30 seconds is convenient), and the time of onset of nystagmus from the beginning of irrigation, as well as its duration and direction should be recorded. The findings from the two sides should be compared; a difference of approximately 20% is considered significantly abnormal. At least five minutes should elapse between irrigations to allow the stimulated canal to return to body temperature. The patient should be asked whether s/he is experiencing spinning sensations or nausea and whether there is a difference between the two sides. If there is less vertigo on one side, you must consider that there is hypofunction of the inner ear on that side.

You may have already surmised that vestibular-oculomotor testing has considerable diagnostic usefulness in the unconscious patient since it is objective and not dependent on patient cooperation. The vestibular-oculomotor reflex pathway encompasses an expanse of the brain stem (upper medulla through mesencephalon) that contains much of the reticular formation necessary for the maintenance of consciousness. Caloric testing is good at assessing the integrity of the brain stem (see Chap. 24). There are two basic causes of depression of consciousness: diffuse bilateral hemispheric dysfunction; or dysfunction of the brain stem reticular formation (patients can have both). Caloric testing provides a method for rapidly screening to determine which of these causes is producing the depressed consciousness. From our discussion of the mechanisms of the VOR it can be surmised that the patient with an intact reflex has an intact brain stem. Also, since the fast phase of nystagmus is mediated by activity in the cerebral cortex, a vestibulo-ocular reflex with tonic eye deviation but no fast, corrective movement, indicates that the brain stem is intact and that the cause of depressed consciousness is diffuse cortical depression. This is most often related to toxic, metabolic or drug-related effects. This occurs because the brain stem response is more resistant to these effects than is cerebral cortical function (of course, if brain activity is sufficiently depressed by toxic or metabolic upsets, even the brain stem can be ultimately affected). In cases of encephalopathy (i.e., depressed consciousness due to diffuse cerebral cortical suppression), caloric irrigation thus elicits only tonic deviation of the eyes. Warm caloric irrigation causes tonic conjugate deviation of the eyes to the side opposite the irrigation, and cold irrigation elicits deviation of the eyes toward the irrigated ear (Fig. 6-7A). An interesting and important observation is the finding of normal oculocephalic test results in the patient who is apparently in "coma". The normal slow component of nystagmus indicates the integrity of the brain stem and the normal rapid phase indicates that the cerebral cortex is awake, alert and functional. Therefore this "coma" is actually fictitious and the patient is more appropriately labeled as "catatonic". Brain stem damage produces variable effects on the reflex depending on the location of the reflex. For example, a destructive process (e.g., infarction, hemorrhage or tumor) at the midbrain level involves the oculomotor complex with subsequent loss of the medial rectus portion of conjugate horizontal deviation, with preserved lateral rectus deviation during irrigation (Fig. 6-7B). A bilateral lesion of the pons, involving the abducens nuclei and the proximate medial longitudinal fasciculi, destroys the vestibularoculomotor reflexes entirely (Fig. 6-7C). What effect would be seen after complete transection of the basis pontis sparing the tegmentum (see Figs. 4-5 and 6-1)?

"Doll's eyes"
The poorly named "doll's eye" maneuver is a simple mechanical test that is particularly useful in the patient with depressed consciousness. More appropriately called the oculocephalic maneuver, it is composed of a rapid passive rotation of the head laterally, which causes an inertial flow of the horizontal canal endolymph in the opposite direction of the head rotation. As seen in Figure 6-8, the eyes are driven in a direction opposite the head rotation. If the patient is awake, the hemispheric checking component (this has the same substrate as the fast component of the nystagmus) keeps the eyes from deviating from midposition and actually may drive the eyes beyond the midposition toward the direction of turning. If the patient is in a coma due to bilateral hemispheric suppression, such as with toxic or metabolic disease (e.g., sedative overdose or uremia), the checking component (also the fast component of nystagmus) is lost. In this case, the eyes deviate away from the direction of head rotation in an unchecked manner (the reflex response is not inhibited by cerebral cortical input). Of course, if dysconjugate gaze is produced during the maneuver, damage to the brain stem in areas that control brain stem extraocular function must be assumed.

Conditions affecting vestibular function

There are a large number of conditions that can affect the vestibular apparatus. Broadly, these can be divided into peripheral causes and central causes. These two types of causes can often be distinguished on clinical grounds (see below). Peripheral causes include conditions damaging the inner ear or the vestibulocochlear nerve while central causes affect the brain stem, vestibulocerebellum or, in rare cases, the cortex. The most common cause of peripheral vertigo has been termed acute labyrinthitis or vestibular neuronitis. While there may be subtle distinctions between these conditions, the presumed etiology is inflammation. In this condition the vertigo comes on quickly and patients often have severe nausea and can't walk. They are at their worst in a matter of hours and then there is slow improvement over days to weeks. There is usually no hearing loss. If it comes on very rapidly (and particularly if there is hearing loss), you should consider that the condition might result from infarction due to occlusion of the labyrinthian artery. Meniere syndrome is not uncommon. It is believed to result from obstructed drainage of endolymph, resulting in increased pressure due to continued production. The pressure damages the delicate hair cells (both vestibular and auditory) with loss of sensitivity. The clinical course is punctuated by paroxysms of sudden vertigo (often with worsened tinnitus), lasting hours with spontaneous

resolution. This is believed to occur due to sudden puncture of the membranes, with resolution of symptoms dependent on sealing the puncture and reestablishment of the normal equilibrium between the fluid compartments of the inner ear. These attacks of vertigo (which can occasionally be triggered by loud noises) can be violent enough to throw the patient to the ground, though, in between attacks, there may be little residual other than some low-tone hearing loss. Perilymph fistula is another cause of peripheral vertigo that is due to leakage of fluid. This condition is often precipitated by barotrauma (abrupt pressure changes) and individual attacks can occasionally be precipitated by pressure changes (including Valsalva maneuver, coughing, sneezing, airplanes, scuba diving, etc). Fluid usually leaks around the round window into the middle ear (and can occasionally be seen there). Acoustic neuroma (actually a neurolemmoma) is a common tumor that grows on the vestibular nerve. Ironically, despite the fact that it damages vestibular nerve fibers, it is a rare cause of vertigo. This is because it progresses slowly, with ample time for compensation of deficits. Positional vertigo will be discussed below. Central causes of vertigo include damage to the brain stem or vestibulocerebellum. Stroke, usually involving the posterior inferior cerebellar artery (which supplies the lateral brain stem and part of the cerebellum) often produces severe vertigo (along with diminished pain and temperature sensations in the face). Isolated infarction or hemorrhage in the cerebellum can produce vertigo. These are particularly important to recognize because they can produce swelling and mass effect that can occasionally be fatal due to brain stem damage. Both of infarction and hemorrhage often produce occipital headache (particularly common with hemorrhage). It is important to consider this before attributing vertigo to vestibular neuronitis, which shouldn't produce headache. Neoplasms of the cerebellum and brain stem usually don't produce much vertigo (for the same reason of slow growth with compensation that we invoked with acoustic neuroma). Inflammatory disease (such as MS or rare conditions such as neurosarcoid) can produce vertigo although this is usually not severe. Paroxysmal vertigo can result from the aura of migraine or seizure. This is presumed to result from activation of the part of the sensory cortex that perceives motion. If vertigo is the only symptom, it is difficult to diagnose seizure or migraine until or unless more characteristic features arise. We will consider positional vertigo below.

Peripheral versus central vertigo

As can be seen in preceding section, there are quite different conditions producing central and peripheral vertigo. Fortunately, it is usually possible to distinguish central from peripheral vertigo on clinical grounds (Table 6-1). First of all, acute central vestibular involvement (as contrasted with acute peripheral disease) is associated with less severe vertiginous symptoms and less nausea. Additionally, central disease often produces more severe nystagmus than peripheral conditions. As distinct from central conditions, where the nystagmus is out of proportion to the vertiginous sensations, with peripheral conditions it is usually possible to predict how vertiginous the patient is by examining the nystagmus. Furthermore, central vertigo often is quite bizarre, changing directions depending on the way that the patient is looking. That does not occur with peripheral vertigo, which is unidirectional and most evident when the patient endevors to look in the direction of the fast phase of the nystagmus. Vertical (upward or downward) nystagmus does not occur with any normal lesion of the peripheral vestibular apparatus. Therefore, vertical nystagmus must be presumed to be central. If not present on forward gaze, vertical nystagmus is best observed by having the patient look directly up or down (Fig. 6-9). Horizontal and rotary nystagmus can occur with either peripheral or central disease and are therefore not of value in differentiation.

Positional vertigo
Positional nystagmus and vertigo are relatively common disorders associated which have several potential causes (both peripheral and central). The patient complains of vertigo only when the head is in certain positions, commonly looking up. The vertigo may persist if the head is kept in the same position (this is particularly true with central disease) or it may rapidly fade (typical of the more common peripheral disease). The most common single cause of positional vertigo is so-called "benign paroxysmal positional vertigo" (BPPV). The characteristic complaint is of vertigo, which is severe and relatively brief, after turning in bed. This can also be triggered by looking up, lying back, getting up quickly or bending to tie the shoes (either when bending forward or, more commonly, when bringing the head back up). This condition results from loose otoliths in the inner ear. When these are in the semicircular canals, position-induced movement of the stones can produce severe vertigo that resolves in under a minute (often leaving the patient quite shaky and nauseated). This can occur after head trauma but is increasingly common with age where the otoliths are less securely anchored to the macula. Testing for positional nystagmus and vertigo is done by rapidly dropping the patient backward as in Figure 6-10 (the Hall Pike or Barany maneuver). The patient's head is held right side

down, left side down, and in the midline on each of three trials. Vertigo and attendant rotatory nystagmus are seen usually beginning after a few seconds and terminating in less than a minute. The condition usually is self-limiting (in months, with dissolution of the stones). However, the "canalith repositioning maneuver" can often move these stones to a less sensitive part of the inner ear, terminating the attacks. Some examples of etiologic significance are head trauma, frequently of only minor severity, vertebrobasilar distribution ischemia, and acoustic neurolemmoma, the latter involving both the nerve directly and the brain stem by compression. The most commonly affected individual is the elderly patient with no predisposing factors and no threatening pathology. Presumably the dysfunction, which is called benign positional vertigo, is caused by aging and minor asymmetrical degenerative changes in the macula-otolith apparatus. Cervical problems can produce positional vertigo by either of two mechanisms: by impairing blood flow through the vertebral artery system; or by activating sensory nerves from cervical muscles. With aging, cervical osteoarthritis becomes common. Occasionally, the bony overgrowth impinges on the transverse foramina through which the vertebral arteries course. Turning the head may increase the foraminal narrowing and compress the vertebral arteries to such a degree that brain stem ischemia occurs. Vertigo on head turning may be the presenting symptom, but usually other evidences of brain stem involvement clarify the picture. The vertigo and other symptoms and signs should be reproducible by turning the head; it is usually not necessary to go through the whole Barany maneuver (see Fig. 6-10). In the case of vascular insufficiency, the vertigo and nystagmus usually take significantly longer to develop than with other causes of positional vertigo, up to 20-30 seconds. However, the nystagmus is variable in type, persists for longer periods, and is associated with only mild vertigo. It has been shown that sensory nerve fibers coming from the cervical musculature have connections with the vestibular nuclei. These connections probably mediate headneck-trunk axis orientation information. Disorders of the neck that are associated with abnormal muscle tightness or spasm can produce vertigo with head movement even without impairing the circulation.

References
Brodal, A.: Neurological Anatomy in Relation to Clinical Medicine, ed. 2. New

York, Oxford University Press, 1969.

Cogan, D.G.: Neurology of the Ocular Muscles, ed. 2. Springfield, IL, Charles C.

Thomas, Publisher, 1956.

 Monrad-Krohn, G.H., Refsum, S.: The Clinical Examination of the Nervous

System, ed. 12. London, H.K. Lewis & Co., 1964.

Spillane, J.D.: The Atlas of Clinical Neurology, ed. 2. New York, Oxford

University Press, 1975.

Walsh, F.B, Hoyt, W.F.: Clinical Neuro-ophthalmology, ed. 3. Baltimore,

Williams & Wilkins Co., 1969.

Questions
Define the following terms:

conductive hearing loss, sensorineural hearing loss, tinnitus, vertigo, nystagmus.

Conductive hearing loss is the loss of the ability to transmit sound waves to the inner ear. Obstruction of the external ear, problems with the tympanic membrane or problems with the middle ear (or the ossicular chain) are the cause. Sensorineural hearing loss is hearing loss due to damage to the hair cells of the organ of Corti or to the auditory part of the vestibulocochlear nerve. Tinnitus is ringing or buzzing in the ears. Vertigo is the illusion of movement. Nystagmus is a to-and-fro movement of the eyes. It can be pendular (even swings in both directions, often due to congenital vision problems) or can be jerk. In jerk nystagmus, there is a fast and a slow component, typically due to vestibular problems (the direction is named by the fast component).

6-1. What brain lesions will cause loss of hearing in one ear?
Answer 6-1. It is nearly impossible to cause loss of hearing in one ear by damage to the brain after the point at which the vestibulocochlear nerve enters the brain. This is because the distribution of hearing is bilateral at all levels of the central nervous system auditory pathway. Localization of sound may be slightly affected by auditory cortex lesions. In fact it is nearly impossible for central nervous system lesions to cause clinically detectable hearing loss and if these is hearing loss you must look at the conductive system, the inner ear and the vestibulocochlear nerve.

6-2. What kind of hearing loss will be produced by damage to the inner ear?
Answer 6-2. Inner ear and CN VIII lesions will produce senorineural deafness.

6-3. What do you call problems in which the sound wave can not reach the inner ear?
Answer 6-3. Blockage of the outer ear or damage to the tympanic membrane or middle ear are called conductive deafness.

6-4. What would it mean if Weber's test lateralized to the left?

Answer 6-4. Weber's test lateralized toward the side of conductive deafness and away from sensorineural.

6-5. What is the most common symptom of damage to the vestibular system?
Answer 6-5. Vertigo is common with vestibular damage.

6-6. What does it mean when someone says that nystagmus was in a particular direction?
Answer 6-6. Nystagmus is named according to the direction of fast movement.

6-7. How can you distinguish vertigo from inner ear damage from that causes by damage to the central nervous system?
Answer 6-7. With peripheral causes of vertigo (the illusion of movement), nystagmus is proportional to the amount of vertigo and nystagmus is always in the same direction regardless of the direction that the patient looks. Also, peripheral nystagmus is almost never in a vertical (up or down) direction. With central vertigo (cerebellum, vestibular nuclei and brain stem) nystagmus is usually greater than vertigo and may shift direction depending on gaze direction.

6-8. What is the only way to examine the integrity of the vestibular system on one side?
Answer 6-8. Caloric testing is the only way to check each inner ear vestibular function independently.

6-9. What would you anticipate finding during cold-water caloric testing in the intact patient who is awake?
Answer 6-9. When the patient is awake, the eyes will drift slowly toward the side of cold water with rapid correction to opposite side (the opposite for warm water). There is tonic balance in vestibular input from each ear; cold water caloric testing decreases tonic input from the inner ear (warm water increases it).

6-10. What would you expect to find in the comatose patient whose brain stem was still working if ice-water was infused into the right ear?
Answer 6-10. The eyes would drift toward the side of the ice-water infusion and remain there for several minutes. There would be no nystagmus (which is a response generated by the conscious cerebral cortex when the visual image slips across the retina).

6-11. Damage to the inner ear produces response that look like (cold/warm) water caloric testing (choose one)?

Chapter 7 - Lower cranial nerve function

The lower cranial nerves are involved in pharynx and larynx function as well as in movements of the neck and tongue. Damage usually manifests as problems with speech and swallowing. These nerves arise from the medulla and, in the case of the accessory

nerve (CN XI), the spinal cord. These nerves are commonly affected by conditions damaging the medulla but bilateral damage to corticobulbar connections can create motor problems that effect tongue and pharynx movement and speech.

IX, X. Glossopharyngeal and Vagus Nerves

These two nerves are considered together because exit from the brain stem side by side, and have similar and frequently side-by-side and overlapping functional and anatomical distributions in the periphery. Also, these nerves connect with many of the same brain stem nuclei (dorsal motor nucleus of the vagus, nucleus ambiguus, nucleus solitarius, spinal nucleus of the trigeminal) and are often damaged together.

Pharynx and palate

The pharynx is innervated by nerves IX and X, with motor and sensory contributions from both. In general, the vagus nerve is motor to the palate elevators and constrictors of the pharynx (as occurs in swallowing and gagging). The glossopharyngeal contains more sensory fibers, including from the posterior part of the tongue and pharynx down to the level of the larynx (where the vagus nerve begins to take over). The entire palate, including the soft palate, has a sensory distribution from the maxillary division of the trigeminal nerve. Contraction of the paired and fused muscles of both sides of the soft palate causes superolateral movement vectors (Fig. 7-1). The sum vector is an upward, midline movement of the palate to seal the nasopharynx when swallowing and making certain sounds (such as "guh"). When examining palate elevation, look at the point of attachment of the uvula to see if it remains in the midline. Also, if there is deviation, inspect the palate to make sure this is not simply due to scaring of the soft palate due to prior throat surgery. If the vagus nerve of one side is damage (e.g., by a tumor at the jugular foramen), the palate elevates asymmetrically, being pulled up toward the strong side (i.e., away from the weak side of the palate, Fig. 7-2). If both sides of the palate are weak, as can occur in certain muscle diseases or if the vagus is damaged bilaterally (such as from invasion by a retropharyngeal carcinoma at the base of the skull), the palate does not elevate normally during phonation and a hypernasal quality is imparted to the voice (especially noted when making a "G" sound). Air usually emanates from the nose when the patient tries to puff up the cheeks and liquid tends to regurgitate into the nose when swallowing. Rarely, a similar finding of bilateral weakness can be seen in patients with bilateral supranuclear lesions (such as by

bilateral cortical damage or bilateral damage to the corticobulbar tracts. In this case, the patient will often show signs of "pseudobulbar" affect (see Chapt. 5).

Gag reflex
The gag reflex involves a brisk and brief elevation of the soft palate and bilateral contraction of pharyngeal muscles evoked by touching the posterior pharyngeal wall. It is tested on the left and the right sides and the reflex response should be consensual (i.e., the elevation of the soft palate should be symmetrical regardless of the side touched). As with all reflexes, the gag reflex has a sensory and a motor limb. The sensory limb is mediated predominantly by CN IX. The motor limb by CN X. Touching the soft palate can lead to a similar reflex response. However, in this case, the sensory limb of the reflex is the trigeminal nerve. In very sensitive individuals, much more of the neuraxis may be involved; a simple gag may enlarge to retching and vomiting in some. The gag response varies greatly from individual to individual but is relatively constant in any one person. In some individuals, this reflex is under such strong voluntary control that probing causes very little or no response. This could make differentiation of normal suppression of the gag from symmetric pathologic depression of motor and/or sensory function difficult. However, actual damage can usually be determined by asking the patient count to 10 immediately after rapidly swallowing 4 oz. of water. If there were bilateral sensory and/or motor deficit, one would anticipate that fluid would penetrate into the unprotected larynx, producing a "wet voice" often with choking and coughing. The water-swallowing test is also a useful screen in detecting which patients with neurologic deficit are likely to have trouble eating (neurologic disease is more likely to affect swallowing of thin liquids, like water, than it is to affect the wallowing of pudding consistencies, which are easiest). In glossopharyngeal nerve (sensory) involvement, there will be no response when touching the affected side. With vagal nerve damage, the soft palate will elevate and pull toward the intact side regardless of the side of the pharynx that is touched. If both CN IX and X are damaged on one side (not uncommon), stimulation of the normal side elicits only a unilateral response, with deviation of the soft palate to that side; no consensual response is seen. Touching the damaged side produces no response at all.

Larynx
The vagus nerve is both the sensory and motor innervation of the larynx. Sensory and motor nerve fibers reach the larynx by different courses, with the superior laryngeal nerve being sensory and the recurrent laryngeal nerve being motor. The recurrent

laryngeal nerves take a long, circuitous route before reaching the larynx, with the left nerve passing all the way around the aortic arch. Mediastinal lesions (e.g., carcinoma of the esophagus, cancerous lymph nodes or aortic aneurysms) may be first evidenced by hoarseness due to paralysis of the left vocal cord. The same can be true on either side for malignancies in the neck, such as thyroid cancer, since both the left and right recurrent laryngeal nerves pass posterior to that gland to reach the larynx. Loss of function of one or both recurrent laryngeal nerves causes "hoarseness". Persistent, painless hoarseness should alert the examiner to the possibility of unilateral or bilateral vocal cord weakness or paralysis. This warrants examination of laryngeal appearance and function. This can either be done by fiberoptic laryngoscopy or by indirect laryngoscopy with a simple curved dental mirror and a light source (a bedside lamp shining over the physician's shoulder or a flashlight held by an assistant) (Fig. 7-3). The mirror must be warmed to prevent fogging. The tongue is held protruded with cotton gauze or is depressed with a tongue blade, and the mirror is then placed face down just below the soft palate, not touching the pharyngeal walls to avoid gagging. It is sometimes useful to spray the nasopharynx with a small amount of a weak topical anesthetic, such as 1% Xylocaine. The mirror allows a view of the superior aspect of the larynx covered by the epiglottis. The patient is asked to say "aah." The epiglottis then uncovers the vocal cords, which should be in a relatively open position. The patient then attempts to say "eee," a high pitched sound, the cords should closely appose unless they are paralyzed on one or both sides (see Fig. 7-3). Laryngoscopy (direct or indirect) is not part of a routine bedside examination, however. It should be done only when phonation changes are persistent.

Central vs. peripheral involvement

To differentiate between involvement of the peripheral portion of a cranial nerve and the brain stem portions, it is important to consider whether there is associated involvement of other cranial nerves or evidence of damage to cerebellar functions or the tracts that course through the brain stem (corticospinal or the lemniscal or spinothalamic sensory paths). It is unusual for brain stem lesions to involve one or two cranial nerves in isolation, without also affecting the contiguous long-tract and cerebellar system structures. Motor neuron disease (a degenerative condition involving upper and lower motor neurons) is an exception to this rule as is poliomyelitis (a rare condition today). Supranuclear motor pathways to the palate, pharyngeal, and laryngeal musculature are bilateral. Therefore, unilateral lesions, even large strokes, rarely produce any persistent problem with lower cranial nerve function (there may be some transient

swallowing trouble). Bilateral acute or subacute loss of hemispheric connections to the medullary nuclei causes difficulty with swallowing, phonating and, initially, a depressed gag reflex. In time, the gag reflex may become uncontrollably hyperactive (as do many other skeletal and autonomic reflexes when they are no longer under supranuclear control).

Taste
Both the glossopharyngeal and vagus nerves (CN IX and X) have taste and somatic sensory functions that are not routinely examined. However, the taste function in the glossopharyngeal nerve (CN IX) can be examined if there is suspicion of damage to the nerve (vagus nerve taste function can not be tested). A saturated solution of salt, a substance normally tasted best by the posterior and lateral taste buds (sweet is tasted best by the anterior and midline tastebuds), is used in the testing with the same technique described for the facial nerve (CN VII, see Chapt. 5).

Somatic sensation
The glossopharyngeal and vagus nerves (along with the facial nerve) supply tiny sensory branches to the external auditory canal. This extensive overlap (which also includes some contributions from the trigeminal nerve and the 2nd cervical nerve) precludes detecting loss of sensation caused by lesions of any one of these nerves. However, pain in the ear may be a prominent early symptom of irritation of any one of these cranial nerves. If the vagus or glossopharyngeal nerve is involved, the pain often extends into the pharyngeal region, helping to differentiate from the pain of seventh-nerve irritation (which would be confined to the ear and mastoid region). If facial weakness is present, this would be a clue to facial nerve irritation, while depression of the gag reflex would suggest vagus or glossopharyngeal nerve involvement. Trigeminal involvement is differentiated by pain in the face and deficits in sensation in the trigeminal distribution; involvement of the upper cervical nerves is indicated by hypoesthesia or pain in the scalp and upper back of the neck.

Carotid sinus (baroreceptor) reflex

The baroreceptor reflex is mediated by sensory fibers in the glossopharyngeal nerve and motor fibers in the vagus nerve. The normal reflex detects increased blood pressure in the carotid sinus, triggering a slowing of the heart and lowering of blood pressure. Because the receptor works as a mechanical transducer, any kind of distortion of the carotid sinus can cause slowing of the pulse and hypotension. Firm massaging of the

carotid bifurcation while monitoring pulse and blood pressure is the bedside technique for testing the reflex. However, this is hazardous due to the potential for excessive slowing of the heart and for disrupting any athersclerotic plaque that might be in the carotid sinus region (potentially producing embolic stroke).

XI. Spinal Accessory Nerve

Technically, the accessory nerve (CN XI) has two components: (1) a central branch arising from medullary nuclei, and (2) a spinal accessory branch arising in the first five to six cervical spinal segments from the lateral portion of the ventral horn. The central branch joins the vagus immediately after leaving the brain stem and is involved in innervation of the laryngeal musculature. We typically consider this component with the vagus nerve and have discussed examination of the larynx and pharynx (above). The spinal accessory branch has an unusual course. It arises from motor neurons in the upper 6 cervical segments. These neurons send their nerve roots to exit the spinal cord laterally (not with the ventral motor nerve root). The nerve roots that comprise the spinal accessory nerve ascend the vertebral canal adjacent to the lateral side of the spinal cord and they enter the skull by passing upward through the foramen magnum. This nerve then turns laterally to pass through the jugular foramen along with cranial nerves IX and X. The spinal accessory nerve provides the motor innervation of the sternocleidomastoid (SCM) muscle before passing through the posterior triangle of the neck to reach the trapezius muscle (which it also innervates). Cervical nerves provide sensory innervation of these muscles. When examining the SCM muscle, the bulk and outline of the muscle should be observed. Atrophy is common in damage to the nerve and fasciculations may be seen especially if the motor neurons are diseased. The SCM muscle rotates the head away from the side of contraction. Testing entails having the subject turn their head against the examiner's hand, which is pressed against the patient's chin (Fig. 7-4). The bulk of the muscle is then easily seen and palpated, and its strength can be determined. Having the patient attempt to bring their chin toward their chest can test the left and right SCM as they work together in this action. Paralysis of this muscle will produce weakness, although not complete loss of ability to rotate the head away from the lesion. This is because there are other muscles that are able to partially compensate. For this same reason, the resting head position is usually not affected by isolated SCM paralysis. Rarely, the patient will hold their head turned slightly toward the side of lesion. The two sternomastoids contracting together will flex the head toward the chest. Bilateral

weakness may prevent the patient from lifting their head off a pillow and the head may be inclined posteriorly for lack of flexor tone. Bilateral weakness suggests muscle or neuromuscular disease. Spasmodic torticollis is a condition that often affects the tone of the SCM muscle, although it can affect several other cervical muscles as well. In this condition, there is an excessive activity of unknown etiology in one (rarely both) of the sternomastoids. This results in an obvious deviation of head postion. The subject's head is spasmodically turned away from the involved muscle, which usually shows hypertrophy. One rather striking observation is that the patient can often terminate the spasm by simply touching the opposite side of the chin or cheek. The head drifts back into its dystonic position once the touch is removed. The spinal accessory nerve innervates the trapezius muscles, which elevate the shoulders and rotate the scapula upward during abduction of the arm. Denervation is evidenced by atrophy and often fasciculations. The shoulder droops on the side of the weak muscle and there is downward displacement of the scapula posteriorly. Shrugging the shoulders against resistance is the standard way of testing the upper trapezius (Fig. 7-4). Both the SCM and the trapezius muscles are under voluntary control, requiring some input from the corticospinal system. The projections from the cerebral cortex to the motor neurons innervating the SCM are bilateral. Therefore, even large unilateral lesions do not produce weaknes of the SCM or any deficits in head turning. However, in the case of corticospinal innervation of trapezius motor neurons there is usually a contralateral predominance. This contributes to mild to moderate contralateral weakness of shoulder elevation following large, unilateral injuries of corticospinal systems. This is rarely very severe, however

XII. Hypoglossal Nerve

The hypoglossal nerve (CN XII) has an entirely motor function, innervating the muscles of the tongue. It originates from the columns of motor neurons located near the midline in the dorsal aspect of the medulla. The nerve exits the ventral side of the medulla as a row of small nerve rootlets adjacent to the pyramid. After a short course through the subarachnoid space, the rootlets come together as a single nerve that passes through the hypoglossal foramen in the base of the skull. Ultimately it reaches the tongue and innervates the intrinsic and extrinsic tongue muscles. The tongue is under voluntary control. Accordingly, corticobulbar pathways activate hypoglossal motor neurons. As

with most cranial nerves, these corticobulbar projections are bilateral, although there is a slight contralateral predominance. Therefore, large lesions to the corticobulbar system, such as large strokes, can produce slight weakness of the contralateral tongue. Weakness of the tongue manifests itself as a slurring of speech. The patient complains that their tongue feels "thick", "heavy", or "clumsy." Lingual sounds (i.e., l's, t's, d's, n's, r's, etc.) are slurred and this is obvious in conversation even before direct examination. Examination of the tongue first involves observation for atrophy and fasciculations. With supranuclear lesions, weakness, frequently mild, is not accompanied by loss of muscle mass or fasciculations. Lesions of the nerve (e.g., hypoglossal neurolemmoma, nasopharyngeal tumor along the base of the skull, basal skull fracture) or of the nucleus in the brain stem (e.g., medullary stroke, motor neuron disease or bulbar poliomyelitis) the tongue displays weakness, atrophy and, possibly, fasciculations on the side of the involvement (Fig. 7-5). Atrophy and fasciculations in combination suggest disease or damage to the motor neurons of the brain stem, but can be seen with peripheral nerve damage, as well. Fasciculations are fine, random, multifocal twitches of muscle. They are evaluated by observing the tongue while it is at rest in the floor of the mouth. They are best seen along the lateral aspect of the tongue. Protrusion frequently causes a fine tremor in the normal tongue, which can obscure or mimic fasciculations. Simply having the patient protrude their tongue in the midline tests strength of the tongue. The normal vectors of protrusion are illustrated in Figure 7-5. When one side of the tongue is weak, it protrudes toward the weakened side (Fig. 7-5). A repetitive or complex lingual sound (e.g., "la la la la" or "Methodist artillery") often shows impediment when any part of the vocal apparatus is affected (e.g., Broca's region, motor cortex, basal ganglia, cerebellum, brain stem, nucleus, or nerve). The most common process causing major involvement of the hypoglossal nerve is motor neuron disease (amyotrophic lateral sclerosis). This is a degenerative disease that has a predilection for early and severe involvement of the hypoglossal motor neurons. The involvement is almost always bilaterally symmetrical. Unilateral damage of the hypoglossal nerve can be produced by tumors or trauma involving the base of the skull, whereas stroke damaging corticobulbar projections is the usual cause of unilateral supranuclear dysfunction.

References

 Brodal, A.: Neurological Anatomy in Relation to Clinical Medicine, ed. 2. New

York, Oxford University Press, 1969.

Cogan, D.G.: Neurology of the Ocular Muscles, ed. 2. Springfield, IL, Charles C.

Thomas, Publisher, 1956.

Monrad-Krohn, G.H., Refsum, S.: The Clinical Examination of the Nervous

System, ed. 12. London, H.K. Lewis & Co., 1964.

Spillane, J.D.: The Atlas of Clinical Neurology, ed. 2. New York, Oxford

University Press, 1975.

Walsh, F.B, Hoyt, W.F.: Clinical Neuro-ophthalmology, ed. 3. Baltimore,

Williams & Wilkins Co., 1969.

Questions
Define the following terms:

dysarthria, dysphonia, dysphagia.

Dysarthria is inability to speak clearly due to problems with control of the motor apparatus of speech (generation and understanding of language should be normal and reading/writing are unaffected). Dysphonia is problems with speech due to disorders of the vocal apparatus in the larynx (laryngitis is an example). Dysphagia is difficulty with swallowing.

7-1. What are the main functions of the glossopharyngeal nerve?

Answer 7-1. The glossopharyngeal nerve provides sensation to the carotid barroreceptor and chemoreceptor, the pharynx and the middle ear.

7-2. What would be the effect on soft palate movement of unilateral damage to the vagus nerve?
Answer 7-2. The vagus nerve activates the elevator of the soft palate. The palate will elevate with deviation of base of uvula away from the side of lesion (toward the intact side).

7-3. What would be the effect of unilateral damage to the vagus nerve on larynx function?
Answer 7-3. The vagus (recurrent laryngeal) innervates larynx muscle. Damage would produce painless hoarseness with weakness or paralysis of vocal cord on that side.

7-4. Describe the course of the spinal accessory nerve.

Answer 7-4. The spinal accessory nerve comes from cervical spinal cord, enters the head through formen magnum and exits through jugular foramen.

7-5. What does the spinal accessory nerve innervate?

Answer 7-5. CN XI innervates the SCM and trapezius muscles.

7-6. What does the hypoglossal nerve innervate?

Answer 7-6. The hypoglossal nerve innervates the tongue.

7-7. What would be the findings in unilateral damage to the hypoglossal nerve?
Answer 7-7. The tongue deviates toward the side of weakness when protruded; it will be weak when attempting to push against cheek on strong side of tongue and, with time, there would be atrophy of the tongue on that side.

7-8. What would be the effect of a large stroke in the motor cortex on tongue movement?
Answer 7-8. Corticobulbar damage (like a stroke) will slightly weaken the contralateral side of the tongue.

7-9. What is the reflex pathway of the gag reflex?

Answer 7-9. Gag reflex - afferent is glossopharyngeal, efferent is vagus. It is a consensual reflex.

7-10. What is the reflex pathway of the cough reflex?

Answer 7-10. The cough reflex - afferent is vagus, efferent is complex including respiration centers and vagus.

7-11. What is the reflex pathway of the barroreceptor reflex?

Chapter 8 - Reflex evaluation

Reflexes are the most objective part of the neurologic examination and they are very helpful in helping to determine the level of damage to the nervous system. We will first discuss the various reflexes used in clinical practice and will conclude the chapter with a discussion of the significance of the findings. In some situations reflexes may be the major part of the examination (e.g., the comatose patient). They have the value of requiring minimal cooperation on the part of the patient and of producing a response that can be objectively evaluated by the examiner. A list of all possible reflexes would be almost endless and a tangle of eponymic jargon for those with an historical bent. It is necessary to know the most commonly elicited reflexes and this knowledge is not terribly difficult to acquire. However, the interpretation of the reflex response requires some discussion. Table 8-1 is a list of many reflexes, some of them in common clinical use (and some less common). As a group, these reflexes can aid in evaluation of most of the segmental levels of the nervous system from the cerebral hemisphere through the spinal cord. In this chapter we will discuss the evaluation of commonly tested reflexes of the spinal cord. We have previously considered reflexes involving the cranial nerves such as

the pupillary light reflex, the jaw-jerk reflex, the baroreceptor reflex and gag. We have also discussed reflex eye movements and many of the autonomic reflexes (such as the oculocardiac and the pupillary light reflex). Here we will consider muscle stretch reflexes and superficial reflexes that are used to evaluate sensorimotor function of the body. All reflexes, when reduced to their simplest level, are sensorimotor arcs. At the minimum, reflexes require some type of sensory (afferent) signal, and some motor response. While the simplest of reflexes involve direct synapse between the sensory fiber and the motor neuron (monosynaptic), many reflexes have several neurons interposed (polysynaptic reflexes). It is important to note that, even with the simplest of reflexes, there are multiple inhibitory and facilitatory influences affect that can affect the excitability of the motor neuron and thus amplify or suppress the response. These influences can arise from various levels of the nervous system. There are intrasegmental and intersegmental connections in the spinal cord, as well as descending influences from the brain stem, cerebellum, basal ganglia and cerebral cortices. All of these can influence the excitability of motor neurons, thereby altering reflex response. Lesions that damage the sensory or motor limb of a reflex arc will diminish that reflex. This can occur at any level of the sensory or motor pathway (in the case of the muscle stretch reflex, for example, this can include: the peripheral nerve and receptors; the dorsal root or dorsal root ganglion; the spinal cord gray matter; the ventral root; the peripheral nerve; the neuromuscular junction; or the muscle). Most of the pathways that descend the spinal cord have a tonic inhibitory effect on spinal reflexes. For this reason, the net result of lesions that damage the descending tracts is facilitation of reflexes that are mediated at only the level of the spinal cord (a classic example being the muscle stretch reflex). With few exceptions, this means that these spinally-mediated reflexes become hyperactive. After acute lesions, spinal reflexes often pass through an initial stage of hypoactivity. This stage has been called "spinal shock" or diaschesis and is more severe and long lasting in proportion to the degree of damage. For example, transection of the spinal cord removes the greatest amount of higher influence and may be associated with weeks of hypoactivity. Small lesions may have little effect on reflexes. When reflexes return after spinal transection, they become extremely hyperactive. Some reflexes, such as the muscle stretch reflex, are semiquantitatively graded. This is also true for some responses as the pupillary light reflex, where the speed of reaction may indicate a "sluggish" response. On the other hand, many reflexes are simply noted as present or absent. This is true of the superficial reflexes (see Table 8-1) and the

"primitive reflexes" that are associated with diffuse bilateral hemispheric dysfunction. In this latter case the reflexes are often designated as "dysinhibited" because these are infantile responses that are suppressed in the normal adult nervous system.

Examination of myotatic ("deep tendon") reflexes

The muscle stretch (myotatic) reflex is a simple reflex, with the receptor neuron having direct connections to the muscle spindle apparatus in the muscle and with the alpha motor neurons in the central nervous system that send axons back to that muscle (Fig. 8-1). Normal muscle stretch reflexes result in contraction only of the muscle whose tendon is stretched and agonist muscles (i.e., muscles that have the same action). There is also inhibition of antagonist muscles. Reflexes are graded at the bedside in a semiquantitative manner. The response levels of deep tendon reflexes are grade 0-4+, with 2+ being normal. The designation "0" signifies no response at all, even after reinforcement. Reinforcement requires a maximal isometric contraction of muscles of a remote part of the body, such as clenching the jaw, pushing the hands or feet together (depending on whether an upper or lower limb reflex is being tested), or locking the fingers of the two hands and pulling (termed the Jendrassik maneuver). This kind of maneuver probably amplifies reflexes by two mechanisms: by distracting the patient from voluntarily suppressing the reflex and by decreasing the amount of descending inhibition. The designation 1+ means a sluggish, depressed or suppressed reflex, while the term trace means that a barely detectible response is elicited. Reflexes that are noticeably more brisk than usual are designated 3+, while 4+ means that the reflex is hyperactive and that there is clonus present. Clonus is a repetitive, usually rhythmic, and variably sustained reflex response elicited by manually stretching the tendon. This clonus may be sustained as long as the tendon is manually stretched or may stop after up to a few beats despite continued stretch of the tendon. In this case it is useful to note how many beats are present. One sign of reflex hyperactivity is contraction of muscles that have different actions while eliciting a muscle stretch reflex (for example, contraction of thigh adductors when testing the patellar reflex or contraction of finger flexor muscles when testing the brachioradialis reflex). This has been termed "pathological spread of reflexes." Practice observing normal reflexes in patients and initially among students is an excellent way to determine the range of normalcy. Almost any grade of reflex (outside of sustained clonus) can be normal. Asymmetry of reflexes is a key for determining

normalcy when extremes of response do not make the designation obvious. The patient's symptoms may facilitate the determination of which side is normal, i.e., the more active or the less active side. If this is a problem, the remainder of the neurologic examination and findings usually clarify the issue. Decreased reflexes should lead to suspicion that the reflex arc has been affected. This could be the sensory nerve fiber but may also be the spinal cord gray matter or the motor fiber. This motor fiber (the anterior horn cell and its motor axon coursing through the ventral root and peripheral nerve) is termed the "lower motor neuron" (LMN). LMN lesions result in decreased reflexes. The descending motor tracts from the cerebral cortex and brain stem are termed the "upper motor neurons" (UMN). Lesions of the UMNs result in increased reflexes at the spinal cord by decreasing tonic inhibition of the spinal segment. Lesions of the cerebellum and basal ganglia in humans are not associated with consistent changes in the muscle stretch reflex. Classically, destruction of the major portion of the cerebellar hemispheres in humans is associated with pendular deeptendon reflexes. The reflexes are poorly checked so that when testing the patellar reflex, for example, the leg may swing to-and-fro (like a pendulum). In normal individuals, the antagonist muscles (in this example, the hamstrings) would be expected to dampen the reflex response almost immediately. However, this is not a common sign of cerebellar disease and many other signs of cerebellar involvement are more reliable and diagnostic (see Chapt. 10). Basal ganglia disease (e.g., parkinsonism) usually is not associated with any predictable reflex change; most often the reflexes are normal.

Superficial reflexes
Superficial reflexes are motor responses to scraping the skin. They are graded simply as present or absent although markedly asymmetrical responses should be considered abnormal as well. These reflexes are quite different than the muscle stretch reflexes in that the sensory signal has to not only reach the spinal cord, but also must ascend the cord to reach the brain. The motor limb than has to descend the spinal cord to reach the motor neurons. As can be seen from the description, this is a polysynaptic reflex. This can be abolished by severe lower motor neuron damage or destruction of the sensory pathways from the skin that is stimulated. However, the utility of superficial reflexes is that they are decreased or abolished by conditions that interrupt the pathways between the brain and spinal cord (such as with spinal cord damage).

Classic examples of superficial reflexes include the abdominal reflex, the cremaster reflex and the normal plantar response. The abdominal reflex includes contraction of abdominal muscles in the quadrant of the abdomen that is stimulated by scraping the skin tangential to or toward the umbilicus. This contraction can often be seen as a brisk motion of the umbilicus toward the quadrant that is stimulated. The cremaster reflex is produced by scratching the skin of the medial thigh, which should produce a brisk and brief elevation of the testis on that side. Both the cremaster reflex and the abdominal reflex can be affected by surgical procedures (in the inguinal region and the abdomen, respectively). The normal planter response occurs when scratching the sole of the foot from the heel along the lateral aspect of the sole and then across the ball of the foot to the base of the great toe. This normally results in flexion of the great toe (a "down-going toe") and, indeed, all of the toes. The evaluation of the plantar response can be complicated by voluntary withdrawal responses to plantar stimulation. The "anal wink" is a contraction of external anal sphincter when the skin near the anal opening is scratched. This is often abolished in spinal cord damage (along with other superficial reflexes).

"Pathological reflexes"
The best known (and most important) of the so-called "pathological reflexes" is the Babinski response (upgoing toe; extensor response). The full expression of this reflex included extension of the great toe and fanning of the other toes. This is actually a superficial reflex that is elicited in the same manner as the plantar response (i.e., scratching along the lateral aspect of the sole of the foot and then across the ball of the foot toward the great toe). This is a primitive withdrawal type response that is normal for the first few months of life and is suppressed by supraspinal activity sometime before 6 months of age. Damage to the descending tracts from the brain (either above the foramen magnum or in the spinal cord) promotes a return of this primitive protective reflex, while at the same time abolishing the normal plantar response. The appearance of this reflex suggests the presence of an upper motor neuron lesion.

Evaluation of reflex changes

We now list the reflex changes associated with dysfunction at various levels of the nervous system.

1. Muscle: Stretch reflexes are depressed in parallel to loss of strength.

2. Neuromuscular junction: Stretch reflexes are depressed in parallel to loss of

strength.

3. Peripheral Nerve: Stretch reflexes are depressed, usually out of proportion to

weakness (which may be minimal). This is because the afferent arc is involved early in neuropathy.

4. Nerve root: Stretch reflexes subserved by the root are depressed in proportion
to the contribution that root makes to the reflex. Superficial reflexes are rarely depressed since there is extensive overlap in the distribution of individual nerve roots of the skin and muscle tested in the superficial reflexes. However, extensive nerve root damage can depress superficial reflexes in proportion to the amount of sensory loss in the dermatomes tested or the motor loss in the involved muscles.

5. Spinal cord and brain stem: Stretch reflexes are hypoactive at the level of the
lesion and hyperactive below the level of the lesion. As noted, during the initial state of spinal shock following acute lesions, the spinal reflexes below the lesion are also hypoactive or absent.
o Superficial reflexes are hypoactive at and below the level of the lesion and normal above. The abdominal superficial reflexes are not reliably present in normal individuals who are excessively obese, who have abdominal scars, or who have had multiple pregnancies, and they are frequently poorly elicited in otherwise normal elderly persons. Therefore, though classically depressed in persons with corticospinal system involvement, one should not place great emphasis on depressed abdominal reflexes if they are the only abnormality found in the examination. The plantar response is exceptional and is abnormal, extensor (Babinski response), when the descending tracts (upper motor neurons) are involved.

6. Cerebellum: Classically the stretch reflexes are hypoactive and pendular as

mentioned above. When this is so, the test is reliable; however, more often than not, the reflexes are not visibly abnormal.

7. Basal ganglia: There are no consistent deep-tendon or superficial reflex

changes. There may be the appearance of some of the "primitive reflexes" (e.g., the glabellar, oculocephalic, grasp, and feeding reflexes see Chap. 2) associated with some diffuse cerebral dysfunction (dementia).

8. Cerebral cortex: Unilateral disease affecting motor cortex will produce an

upper motor neuron pattern of weakness (i.e., hyperactive muscle stretch reflexes and depressed or absent abdominal and cremasteric reflexes) on the contralateral side. Additionally, there may be a Babinski response.

Bilateral disease is associated with the same abnormalities bilaterally, and in

addition, there may be "primitive reflexes" due to release of these responses from cortical inhibition (see Chap. 2). o With bilateral damage to the motor cortex (particularly when corticobulbar system is heavily affected), inhibitory control of the complex emotional expression reflexes becomes defective. These individuals cry or laugh with minimal emotional provocation and the patient usually says that they do not understand why they are crying or laughing. These complex emotional reflexes are subserved by the limbic system and are normally under inhibitory modulation by the neocortex. Bilateral damage may release these responses in a pattern that is termed "pseudobulbar" (see Chpt. 5).

References
DeJong, R.N.: The Neurologic Examination, ed. 4. New York, Paul B. Hoeber,

Inc., 1958.
Monrad-Krohn, G.H., Refsum S.: The Clinical Examination of the Nervous

System, ed. 12, London, H.K. Lewis & Co., 1964.

Wartenberg, R.: The Examination of Reflexes: a Simplification. Chicago, Year

book Medical Publishers, 1945.

Questions
Define the following terms:

hyper-reflexia, pathological spread of reflex, clonus, Babinski sign, Hoffmann's sign, myotatic reflex, upper motor neurons, lower motor neurons, reinforcement.
Hyper-reflexia is excessively brisk reflexes Pathological spread of reflex occurs when a reflex contraction occurs in a muscle whose tendon was not stretched (i.e., finger flexion when testing the brachioradialis reflex or thigh adduction when the patellar reflex is tested). It is a suggestions of hyperactive reflexes. Clonus is repeated contraction of muscles (usually the calf muscles or the wrist flexor muscles) when the muscles are stretched manually (such as by ankle dorsiflexion or wrist extension). Sustained clonus is when this occurs repeatedly as long as the stretch is maintained. Babinski sign is reflex dorsiflexion of the great toes and fanning of the other toes by stroking the lateral side of the sole of the foot. This stroke is often continued across the ball of the foot toward the base of the great toe. This occurs in patients with upper motor neuron damage. The normal plantar response is for the treat toe to flex.

Hoffmann's sign is flexion of the thumb following a maneuver that consists first of passive flexion of the patients middle finger by pressure over the nail bed, followed by sudden release of this pressure. It is a sign of brisk reflexes but is not pathological unless it is accompanied by other signs of upper motor neuron damage or is asymmetrical. Myotatic reflex is the muscle stretch reflex (often termed the deep tendon reflex). Upper motor neurons are the principal descending motor pathways for voluntary movement, including the corticospinal and corticobulbar tracts (and some other associated tracts). Lower motor neurons are the anterior horn motor neurons and their axons that extend through the ventral nerve root and the peripheral nerves to reach the neuromuscular junction. Reinforcement involves the strong contraction of muscles outside of the area in which muscle stretch reflexes are being tested. This will serve to increase the reflexes. Specific examples include clenching the jaw, pressing the feet together or clasping the hands and attempting to pull them apart (the Jendrasik maneuver).

8-1. What is the main effect of descending motor systems on reflexes?

Answer 8-1. Motor cortex and descending motor pathways generally involved in suppressing (inhibiting) reflexes.

8-2. What are the 7 Deep Tendon Reflex exams (DTRs)? What sensory/motor nerves are they testing?
Answer 8-2. Biceps - musculocutaneous nerve and mainly C6; Triceps - radial nerve and mainly C7; Brachioradialis (radial periosteal) - radial nerve and mainly C6; Finger flexor - musculocutaneous nerve and mainly C7-8; Patellar - femoral nerve and mainly L3-L4; Achilles' reflex (ankle jerk) - tibial nerve and mainly S1; Jaw jerk - trigeminal

8-3. What are the superficial reflexes?

Answer 8-3. Superficial reflexes include: abdominal, cremaster, plantar, anal wink.

8-4. What is the effect of damage to corticospinal fibers on myotatic (deep tendon) reflexes? What is the effect on superficial reflexes?
Answer 8-4. DTRs increase with damage to descending motor pathways; superficial reflexes decrease with damage to descending motor pathways.

8-5. What primitive reflexes emerge with diffuse bilateral hemispheric dysfunction?
Answer 8-5. Diffuse bilateral hemispheric dysfunction can dysinhibit grasp, glabellar, suck, rooting, oculocephalic and nuchocephalic reflexes.

8-6. What happens to DTRs with lesions in the cerebellum & basal ganglia?
Answer 8-6. Usually no change, though may be sluggish with cerebellar damage.

8-7. How are DTRs graded?

Answer 8-7. 0-4+. To grade a reflex as "0", you must try reinforcement. 4+ means there is sustained clonus. 1 is sluggish, 2 is "normal" and 3 is "brisk".

8-8. What is the most important consideration in testing reflexes?

Answer 8-8. Symmetry.

8-9. What reflex changes would occur in lesions of muscles?

Answer 8-9. No change unless end stage.

8-10. What reflex changes would occur in lesions of the neuromuscular junction?
Answer 8-10. Normal to decreased depending on severity of weakness.

8-11. What reflex changes would occur in lesions of the peripheral nerves?
Answer 8-11. Decreased in clinically affected areas.

8-12. What reflex changes would occur in lesions of the nerve root?
Answer 8-12. Decreased in clinically affected areas.

8-13. What reflex changes would occur in lesions of the spinal cord and brain stem?
Answer 8-13. Usually reflexes will be increased unless the gray matter (anterior horn cells, lower motor neurons) is damaged right at the reflex level. Acute spinal cord injury can result in spinal cord shock (flaccid, decreased reflex).

8-14. How can damage to sensory nerve fibers affect reflexes?

Answer 8-14. Damage to sensory nerve fibers may also decrease reflexes by damaging the afferent limb of the reflex arc.

8-15. What is the effect of neuropathy on muscle stretch reflexes?

Answer 8-15. Neuropathy often produces decreased reflexes out of proportion to weakness.

8-16. What are some visceral reflexes that can be tested?

Chapter 9 - Sensory system evaluation

Evaluation of sensation is hindered by several difficulties. Sensation belongs to the patient (i.e., is subjective) and the examiner must therefore depend almost entirely on their cooperation and reliability. A demented or psychotic patient is likely to give only the crudest, if any, picture of their perception of sensory stimuli. An intelligent, stable patient may refine asymmetries of stimulus intensity to such a degree that insignificant differences in sensation are reported, only confusing the picture. Suggestion can modify a subject's response to a marked degree (e.g., to ask a patient where a stimulus changes suggests that it must change and may therefore create false lines of demarcation in an all too cooperative patient). Obviously the examiner must not waste time and efficiency on detailed sensory testing of the psychotic or demented patient, and must warn even the

most cooperative patient that minute differences requiring more than a moment to decipher are probably of no significance. Additionally, the examiner must avoid any hint of predisposition or suggestion. Nonetheless, even after all precautions are taken, problems with the sensory exam still arise. Uniformity in testing is almost impossible and there is considerable variability of response in the same patient. Factors that may affect the patient's variability and should be controlled are fatigue and mood. Fatigue is particularly likely to be induced by a long, detailed, unnecessary, and tedious sensory examination during which the examiner is frequently exhorting the patient's undivided attention. A rapid, efficient exam is the most practical means of diminishing fatigue. Mood is less subject to modification. Use of a pressure transducer, such as VonFrey monofiliments, allows more consistent stimulus intensities and therefore more objectivity in the examination; however, this is impractical at bedside and does not eliminate patient variability. Sensory changes that are unassociated with any other abnormalities (i.e., motor, reflex, cranial, hemispheric dysfunctions) must be considered weak evidence of disease unless a pattern of loss in classical sensory pattern is elicited (for example, in a typical pattern of peripheral nerve or nerve root distribution). Therefore, one of the principle goals of the sensory exam is to identify meaningful patterns of sensory loss (see below). Bizarre patterns of abnormality, loss, or irritation usually indicate hysteria or simulation of disease. However, the examiner must beware of their own personal limitations. Peripheral nerve distributions vary considerably from individual to individual, and even the classic distributions are hard to keep in mind unless one deals with neurologic problems frequently. Therefore, it is advisable for the examiner to carry a booklet on peripheral nerve distribution, sensory and motor (such as: Aids to the Examination of the Peripheral Nervous System, published by the Medical Council of the U.K.).

Screening exam
As in all components of the examination, an efficient screening exam must be developed for sensory testing. This should be more detailed when abnormalities are suspected or detected or when sensory complaints predominate. Basic testing should sample the major functional subdivisions of the sensory systems. The patient's eyes should be closed throughout the sensory examination. The stimuli should routinely be applied lightly and as close to threshold as possible so that minor abnormalities can be detected. Spinothalamic (pain, temperature and light touch), dorsal column (vibration,

proprioception, and touch localization), and hemispheric (stereognosis, graphesthesia) sensory functions should be screened. Pain (using a pin or toothpick), vibration (using a C128 tuning fork), and light touch should be compared at distal and proximal sites on the extremities, and the right side should be compared with the left. Proprioception should be tested in the fingers and toes and then at larger joints if losses are detected. Stereognosis, the ability to distinguish objects by feel alone, and graphesthesia, the ability to decipher letters and numbers written on skin by feel alone, should be tested in the hands if deficits in the simpler modalities are minor or absent. However, Significant defects in graphesthesia and stereognosis occur with contralateral hemispheric disease, particularly in the parietal lobe (since this is the somatosensory association area that interprets sensation). However, any significant deficits in the basic sensory modalities cause dysgraphesthesia and stereognostic difficulties whether the lesion or lesions are peripheral or central. Therefore, it is difficult or impossible to test cortical sensory function when there are deficits of the primary sensory functions. It may be surprising that the more basic modalities are usually not greatly affected by cortical lesions. With acute hemispheric insults (e.g., cerebral infarction or hemorrhage), an almost complete contralateral loss of sensation may occur. It is relatively short-lived, however; perception of pin and light touch, as routinely tested, returns to almost normal levels, whereas proprioception and vibration may remain deficient (though considerably improved) in most cases. This lack of a significant longterm deficiency in basic sensation following hemispheric lesions has no completely satisfactory explanation, although some basic sensations probably have considerable bilateral projection to the hemispheres.

Double simultaneous stimulation

Double simultaneous stimulation (DSS) is the presentation of paired sensory stimuli to the two sides simultaneously. This can be visual, aural or tactile. Light touch stimuli presented rapidly, simultaneously, and at minimal intensity to homologous areas on the body (distal and proximal samplings on extremities) may pick up very minor threshold differences in sensation. Additionally, this testing can also detect neglect phenomena due to damage of the association cortex. Neglect may be hard to distinguish from involvement of the primary sensory systems. However, neglect usually can be demonstrated in multiple sensory systems (i.e., visual, auditory, and somesthetic), confirming that this is not simply damage to one

sensory system. Association cortex lesions, particularly involvement of the right posterior parietal cortex, may become apparent only on double simultaneous stimulation. The face-hand test is a further modification of DSS. This test takes advantage of the fact that stimuli delivered to the face dominate over stimulation elsewhere in the body. This dominance is best illustrated in children and in demented and therefore regressed patients. Before the age of ten, most strikingly earlier than age five, stimuli presented simultaneously to the face and ipsilateral or contralateral hand are frequently (more than three in ten stimulations) perceived at the face alone. Perception of the hand, and, if tested, other parts of the body is extinguished. In an older child or adult, several initial extinctions of the hand may occur, but very quickly both stimuli are correctly perceived. In the patient with diffuse hemispheric dysfunction, dementia, a regression is frequently seen to consistent bilateral extinction of the hand stimuli. This test therefore can be doubly useful, first as an indication of diffuse hemispheric function and second by stimulating the face and opposite hand, a means of detecting minor hemisensory defects (e.g., if the patient consistently extinguishes only the right hand and not the left, a sensory threshold elevation due to primary sensory system or association cortex involvement on the left is suspect).

Patterns of sensory loss

Since one of the main goals of the sensory exam it is important to consider the principle patterns of sensory loss resulting from disease of the various levels of the sensory system. These patterns of loss are based on the functional anatomy of the various components of the sensory system and we will also briefly review some of these elements.

Peripheral neuropathy (polyneuropathy)

Peripheral neuropathy, that is, symmetrical damage to peripheral nerves, is a relatively common disorder that has many causes. Most of these can broadly be classified as toxic, metabolic, inflammatory or infectious. In this country, the most common causes are diabetes mellitus and the malnutrition of alcoholism, although other nutritional deficiencies or toxic exposures (either environmental toxins or certain medicines) are occasionally seen. Infections, such as Lyme disease, syphilis or HIV can cause this pattern and there are inflammatory and autoimmune conditions that can produce this pattern of damage. A more complete discussion can be found in chapter 12. Because this is a systemic attack on peripheral nerves, the condition produces symmetrical symptoms.

The initial symptoms are most often sensory and the longest nerves are affected (the ones that are most exposed to the toxic or metabolic insult). The receptors of the feet are considerably farther removed from their cell bodies in the dorsal root ganglia than are the receptors of the hands. The metabolic demands on these neurons is substantial which accounts for their being the first affected and for the early appearance of sensory loss in the feet in a "stocking" distribution. Later on, as the symptoms reach the mid-calf, the fingers are involved and a full "stocking-glove" loss of sensation develops. Even later, when the trunk begins to be involved, sensory loss is noted first along the anterior midline (Fig. 9-1). Vibration perception is often the earliest affected modality since these are the largest, most heavily myelinated and most metabolically demanding fibers. Usually the loss of pin, temperature, and light-touch perception follow, and conscious proprioception (joint position sense) is variably affected. Despite the fact that proprioception follows many of the same pathways as vibration it is usually not as noticeably affected because the testing procedure (i.e., moving the toes or fingers up or down), is quite crude and is not likely to pick up early loss. The peripheral deep-tendon reflexes are depressed early in most cases of peripheral neuropathy, particularly the Achilles reflex. This is because the sensory limb of this reflex depends on large myelinated fibers. As a rule, symptomatic motor involvement is late and, when it occurs, it affects the intrinsic muscles of the feet first.

Radiculopathy
Radiculopathy (nerve root damage) is the relatively common result of intervertebral disc herniation or pressure from narrowing of the intervertebral foramina due to spondylosis (arthritis of the spine). The most common presentation of this is sharp, shooting pain along the course of the nerve root (Fig. 9-2). Single-root usually does not have any sensory loss because of the striking overlap of dermatomal sensory distribution (Fig. 93). There may be slight loss, often accompanied with paresthesias (tingling or pins and needles) in small areas of the distal limbs where the sensory overlap is not great. Table 91 lists some of the common areas of paresthesia or decreased sensation with common nerve root injuries. Herpes zoster, which affects individual dorsal roots, nicely demonstrates dermatomal distribution because, despite the lack of sensory loss (attributable to overlap), vesicles ("shingles") appear at the nerve endings in the skin (see Fig. 9-3).

Nerve root damage in the cauda equina often produces a "saddle" distribution of sensory loss by affecting the lower sacral nerve roots. This saddle distribution of sensory loss can also be seen in anterior spinal cord damage (see the next section) and, in either case, must be taken quite seriously due to the potentially serious sequellae of spinal cord and cauda equina damage. Nerve root pain is often quite characteristic. It is often quite sharp and welllocalized to the dermatomal distribution and may be brought on by stretching of the nerve root (Fig. 1-5) or by maneuvers that load the intervertebral discs and compress the intervertebral foramina (Fig. 1-4). However, pain can also "refer" (see Chapt. 26). This referred pain is less localized and is often felt in the muscles (myotomal) or skeletal structures (sclerotomal) that are innervated by the nerve root. The person usually complains of a deep aching sensation. Myotomes should not be memorized but can be looked up easily by referring to the motor root innervations of muscles, which are essentially the same as their sensory innervations. Sclerotomal overlap is so great that localization on their basis is impractical.

Spinal cord
Spinal cord damage is characterized by both sensory and motor symptoms both at the level of involvement as well as below by affecting the tracts running through the cord. Symptoms referable to the level of injury appear in the pattern of dermatomes and myotomes and, when present, are very useful for localizing the level of spinal cord damage. The symptoms of damage to the long sensory tracts (the dorsal columns and the spinothalamic tract) are less helpful in localizing the lesion because it is often impossible to determine the precise level of the sensory loss and also because, particularly in the case of the spinothalamic tract, there is considerable dissemination of the signal in the spinal cord before it is relayed up the cord. Similar difficulties make it difficult to localize the level of spinal cord damage by examining for damage to the descending (corticospinal) motor tracts. Therefore, when long tract damage is identified, one can only be certain that the lesion is above the highest level that is demonstrably affected. Compression of the spinal cord from the anterior side first involves the spinothalamic paths from the sacral region, and a "saddle" loss of pin and temperature perception is usually the first symptoms even with lesions high in the spinal cord (Fig. 94). In this case, as symptoms progress with greater degrees of compression, symptoms progressively ascend the body up toward the level of the actual cord damage (see Fig. 94).

Intramedullary lesions of the spinal cord (such as syrinx, ependymoma, or central glioma) may present with a very unusual pattern of "suspended sensory loss". This consists of an isolated loss of pain and temperature perception in the region of the expanding lesion because of damage to the crossing spinothalamic tract fibers (Fig. 9-5). In this pattern of sensory loss due to expanding intramedullary lesions, there is "sacral sparing" of pain and temperature because the more peripheral spinothalamic fibers (the ones from the sacrum) are the last to be involved (see Fig. 9-4). With intramedullary lesions, the dorsal columns are also usually spared until extremely late in the course of expansion, leaving touch, vibration, and proprioception intact. The loss of one or two sensory modalities (such as pain and temperature sense, in this case) with preservation of others (such as touch, vibration and joint position sense) is termed a "dissociated sensory loss" and is in contrast to the loss of all sensory modalities associated with major nerve or nerve root lesions or with complete spinal cord damage. Complete hemisection of the cord is seen occasionally in clinical practice and is quite illustrative of the course of spinal cord sensory pathways. This lesion results in a characteristic picture of sensorimotor loss (Brown-Sequard syndrome), which is easily recognized due to the loss of dorsal columns sensations (vibration, localized touch, joint position sense) on the ipsilateral side of the body and of spinothalamic sensations (pain and temperature) on the contralateral side (Fig. 9-6).

Brain stem
Brain stem involvement, like involvement of the spinal cord, is characterized by longtract and segmental (cranial nerve) motor and sensory abnormality and is localized by the segmental signs. The picture of ipsilateral cranial nerve abnormality and contralateral long-tract dysfunction is quite consistent (Fig. 9-7). Both the dorsal columns and pyramids decussate at the spinomedullary junction (the spinothalamic system has already decussated in the spinal cord). This accounts for the typical crossed presentation of symptoms in the body. Below the level of the midbrain, the spinothalamic and dorsal column (medial lemniscus) systems remain separate and therefore lesions may involve the pathways separately (i.e., there may be a dissociated sensory loss). For example, an infarction caused by occlusion of the posterior inferior cerebellar artery typically involves only the lateral portion of the medulla. The ipsilateral trigeminal tract and nucleus and the spinothalamic tract are frequently included in the lesion, leaving a loss of pain and temperature perception over the ipsilateral face (see Chap. 5) and the contralateral side of the body from the neck down. The medial

lemniscus and its modalities (i.e., vibration, joint position and well-localized touch) are spared.

Thalamus
Thalamic lesions are associated with contralateral hemihypesthesia. Initially, if the lesion is acute, there is considerable loss bordering on anesthesia, but some recovery is expected over time, especially of touch, temperature, and pain perception. Vibration and proprioception remain more severely affected. Unfortunately, episodic paroxysms of contralateral pain may be a striking and not infrequent residual of thalamic destruction (this is one of the "central pain syndromes"). The pain can be controlled occasionally with anticonvulsants. An additional residual that may develop over time is marked contralateral hyperpathia in spite of the presence of diminished overall sensitivity of the skin. Stimulation of a site with a pin causes a very unpleasant, poorly localized and spreading sensation, which is frequently described as burning. This is presumably an irritative phenomenon of the nervous system, although it may also result from loss of normal pain-suppression mechanisms. It is seen most often after thalamic lesions, although it can occur as a residual of lesions in any portion of the central sensory systems. A hypersensitivity to cold sensation frequently accompanies the hyperpathia.

Cerebral cortex
As discussed earlier, cortical lesions tend to leave minimal deficits in basic sensation but, especially if the parietal lobe is damaged, there may be striking contralateral deficits in the higher perceptual functions (see Chap. 2). Stereognosis and graphesthesia are abnormal in spite of minor difficulties with vibration and proprioception and even less, if any, difficulty with pain, temperature, and light-touch perception. Of course, if there is significant deficit of primary sensations, it may be impossible to test for deficits of higher perceptual functions.

References
Brodal, A.: Neurological Anatomy in Relation to Clinical Medicine, ed.2, New

York, Oxford University Press, 1969.

Medical Council of the U.K.: Aids to the Examination of the Peripheral Nervous

System. Palo Alto, Calif., Pendragon House, 1978.

Monrad-Krohn, GH, Refsum, S.: The Clinical Examination of the Nervous

System, ed. 12, London, H.K. Lewis & Co., 1964.

 Wolf, J.: Segmental Neurology, Baltimore, University Park Press, 1981.

Questions
Define the following terms:

conscious proprioception, agnosia (sterioagnosia), graphesthesia, dermatome, sclerotome, myotome, radiculopathy, myelopathy, anesthesia/hypoesthesia, hyperpathia, allodynia, hyperesthesia, dysesthesia, paresthesia, polyneuropathy, subjective.
Conscious proprioception is the ability to tell where a body part is in space. It is largely based on joint position sense. Agnosia (sterioagnosia) ia the inability to recognize what a sensation is despite relatively normal perception of the sensation. When it is tactile it is termed sterioagnosia (or asteriognosis). It would be the inability to determine the denomination of a coin despite normal ability to perceive it, for example. Graphesthesia is the ability to identify letters or figures traced on the skin (without looking). Dermatome is the area of skin supplied by a nerve root. Sclerotome is the area of bone and joints supplied by a single nerve root. Myotome is the muscles supplied by a single nerve root. Radiculopathy this is damage to a nerve root (radiculitis is irritation). Myelopathy is damage to the spinal cord from any cause. Anesthesia/hypoesthesia is loss (or decrease) in sensation. Hyperpathia is the exaggerated perception of normally painful stimuli. Allodynia is the perception of normally innocuous stimuli as being painful. Hyperesthesia is excessive sensitivity to any modality. Dysesthesia is the perception of the pain when no stimulus is present. Paresthesia is the detection of a sensation in the absence of any stimulus. Polyneuropathy is generalized damage to peripheral nerves. This is usually due to a systemic cause. The sensory exam is by definition subjective, that is, relies on the patients report.

9-1. What are the steps involved in the sensory exam?

Answer 9-1. First, the exam needs to determine if the patient can detect modality; next, you need to know if it is the same on both sides; then you need to know if the patient can interpret the sensation.

9-2. How is it possible to lose some types of sensations and not others?
Answer 9-2. Different sensory modalities follow different types of nerve fibers and different pathways (tracts) through the nervous system.

9-3. What sensations are conveyed by the small-diameter sensory nerve fibers in a peripeheral nerve?
Answer 9-3. Small, unmyelinated or lightly-myelinated (slow) nerve fibers convey pain and termperature sense.

9-4. What sensations are conveyed by large-diameter sensory nerve fibers in a peripeheral nerve?
Answer 9-4. Large, heavily myelinated (fast) nerve fibers convey proprioception and well-localized touch sensation. They are also the sensory limb of the muscle stretch reflex.

9-5. What sensations are conveyed by the dorsal columns?

Answer 9-5. Dorsal columns convey vibration, 2-point discrimination and joint position sense.

9-6. What sensations are conveyed by the spinothalmic tract?

Answer 9-6. The spinothalamic tract conveys pain, temperature and very light (poorly localized) touch.

9-7. What is tested by double simultaneous stimulation?

Answer 9-7. Double simultaneous stimulation tests attention/neglect (parietal lobe).

9-8. Where would the lesion be if the patient was able to detect all modalities of sensation but could not recognize an object placed in the right hand?
Answer 9-8. The left parietal lobe (somatosensory association area).

9-9. What is the common sensory loss from damage to the spinal cord?
Answer 9-9. Spinal cord lesions often result in sensory level (loss of sensations below lesion) due to damage to ascending sensory tracts. .This loss (especially of pin sensation) usually begins at least several segments below the level of the lesion of the tract.

9-10. What would be the expected sensory loss from damage restricted to the left side of the spinal cord?
Answer 9-10. There would be ipsilateral loss of vibration and joint position sense and contralateral loss of pain and temperature sense below the level of the lesion. The pain and temperature sense loss would start at least several dermatomes below the injury.

9-11. What is the characteristic of sensory loss due to damage to peripheral nerves in a limb?
Answer 9-11. Peripheral nerve injury (mononeuropathy) usually results in well-localized sensory loss (often with appropriate motor loss).

9-12. What is the pattern of sensory loss seen in diffuse damage to peripheral nerves (polyneuropathy?

Chapter 10- Motor system examination

In this chapter we discuss the evaluation of the motor systems, that is the systems involved in generation and control of voluntary and reflex movements. The motor system can be divided into (1) the peripheral apparatus, which consists of the anterior horn cell and its peripheral axon, the neuromuscular junction, and muscle, and (2) the more complex central apparatus, which includes the descending tracts involved in control (i.e., the pyramidal system) and the systems involved in initiating and regulating movement (the basal ganglia and cerebellum). Dysfunction in individual components of the motor system systems results in fairly specific abnormalities that can be evaluated at the bedside. Although multiple components may be involved (particularly with diseases of the central nervous system) isolated involvement of the various components commonly occurs. We will consider these components in order to help you establish an orderly approach to motor system evaluation (Table 10-1). Examination for motor dysfunction includes assessment of strength, muscle tone, muscle bulk, coordination, abnormal movements and various reflexes. Many of these are best detected through simple (but careful) observation. However, a few maneuvers aid in the detection of abnormality. Table 10-2 lists the components of a comprehensive and efficient screening examination that will elicit and localize most motor system dysfunctions. If no abnormalities are found, this exam should only take two to three minutes in a cooperative patient. Table 10-3 lists the findings expected with diseases listed in Table 10-1, and Table 10-4 lists differentiating points between diseases affecting the nerves versus those primarily damaging muscle.

Elements of the examination

Examination of the motor system can be relatively objective and Tables 10-2 and 10-3 outlines an approach using isolated segments of the motor system as models. Mixedsystem involvements do occur with variable symptom and sign predominance, depending on such variables as the dominance of the various motor systems involved and the extent of the lesion(s) in each system. Lack of cooperation caused by patient fatigue, misunderstanding of the tasks demanded, or lack of physician-patient rapport must always be considered. Feigned or hysterical weakness, for example, usually can be distinguished by its bizarre localization, the absence of expected involvement of other systems (i.e., reflex, sensory, cranial), and the irregular ratchet-like giving way of muscles tested. It is always important to consider the implications of your findings and

what additional or confirmatory test can be done to clarify and document your conclusions about the patient's motor system abnormality.

Strength
Strength is conveniently tested by having the patient resist your force as you attempt to move their body part against the direction of pull of the muscle that you are evaluating. This is graded on a graded scale of 0-5, with "0" representing absolutely no visible contraction and 5 being normal. A grade of "1" means that there is visible contraction but no movement;"2" is some movement but insufficient to counteract gravity;"3" is barely against gravity (with inability to resist any additional force); and "4" being less than normal (but more than enough to resist gravity). Obviously, there is ample range between 3 and 5, making the determination somewhat subjective. Some examiners expand the 5 point scale into a 9 point scale by the addition of + symbols when strength seems to be between numbers. Still others add - symbols when a muscle seems to function just below a level. While there may be merit in having a scale beyond the original 5 points (particularly between 3 and 5) it must be remembered that the scale is quite arbitrary and lacks the precision suggested by the creation of many categories. Additionally, "normal" is a designation that takes into account the patients age and level of conditioning. This assessment can be made with greater precision when there is a normal side with which to compare it. In order to test strength at various levels of the nervous system, several muscles must be tested. The more common of these muscles, along with their particular peripheral nerve and nerve root levels, are found in Table 10-5. It is often very efficient to test patients using functional tasks rather than by manually testing each muscle. For example, the patient may be asked to hold the arms horizontally out in front with the palms up and eyes closed. Diffuse weakness of the upper limb often produces a pronator drift, i.e., downward drift of the weak limb with the hand pronating (turning in). If the limb drifts straight down, without pronation, this is not suggestive of physiologic weakness and the patient may have a conversion disorder or malingering. Erratic drift of the limb can be seen with proprioceptive sensory loss (confirmed by testing of proprioception). When testing strength in the legs it is helpful to have the patient attempt to walk on the toes and then heels. Other tests of the legs would include hopping on each foot, standing from a chair (without use of the hands) or climbing a stair. These latter tests examine more proximal muscles. When confronting the patient with weakness, some assessment of effort should be made. Poor effort is usually reflected as good initial contraction, followed by a collapse

(often termed "breakaway" or collapsing weakness). This is not a pattern seen in true neurologic injury where strength is typically inadequate but relatively constant. It is usually easy to detect the patient with "collapsing" weakness if you apply varying force during the muscle test. With true neurologic weakness, the maximum force that the patient applies does not vary appreciably. "Collapsing weakness" should not be graded. There are several potential causes of collapsing weakness, ranging from pain to the conscious embellishment of symptoms. When this pattern is seen, other more objective elements of the examination (such as reflex testing) become more important. The ultimate goal of strength testing is to decide whether there is true "neurogenic" weakness and to determine which muscles/movements are affected. In correlation with the remainder of the motor exam it should be possible to determine the particular part of the nervous system that is at fault to produce this weakness. Probably the most important decision is whether the weakness is due to damage to upper or lower motor neurons (UMN or LMN). As you may recall from chapter 8, upper motor neuron weakness is due to damage to the descending motor tracts (especially corticospinal) anywhere in its course from the cerebral cortex through the brain stem and spinal cord. UMN weakness is typically associated with increased reflexes and a spastic type of increased tone. On the other hand, LMN weakness is due to damage of the anterior horn cells or their axons (found in the peripheral nerves and nerve roots). This results in decreased stretch reflexes in the affected muscles and decreased muscle tone. Additionally, atrophy usually becomes prominent after the first week or two, and this atrophy is out of proportion to the amount of disuse produced by the weakness.

"Deep tendon" (myotatic) reflexes

The "deep tendon" (myotatic) reflexes are a critical part of the neurologic examination that is discussed in Chapt. 8. Testing reflexes is the most important element of determining whether weakness is of an upper or lower motor neuron type (limited only by the fact that only certain muscles actually have reliably tested stretch reflexes (include the biceps, triceps, brachioradialis (radial periosteal), quadriceps, hamstring and calf muscles). Since the reflex arc includes stretch receptors and sensory fibers, it is not necessary to damage motor axons to abolish reflexes. However, in the setting of the patient with known weakness, reflex testing is a powerful tool to investigate the cause. As you may recall from chapter 8, symmetry of reflexes is the most important consideration in determining normality. Pathological "spread of reflexes" (i.e., contraction of muscles that produce motions other than the one associated with the test muscle) is another objective sign of hyperactivity. You may recall that sustained clonus

(repeated muscle contraction when a muscle is passively stretched) is an indicator of hyperactive reflexes. Conditions that damage lower motor neurons decrease muscle stretch reflexes by interrupting the reflex arc (Fig. 8-1). Therefore, a diminished reflex in a weak muscle suggests damage to the lower motor neurons somewhere along the course to the muscle (i.e., anterior horn cells, motor nerve root, or peripheral nerve). Hyperactive reflexes are seen after damage to upper motor neurons (i.e., descending motor tracts). There are other confirmatory findings that may suggest upper or lower motor neuron disease. These signs include atrophy (LMN), fasciculations (LMN), spasticity (UMN), Babinski sign (UMN) or loss of superficial reflexes (UMN).

Superficial and "pathologic" reflexes

Superficial reflexes (abdominal, cremaster and plantar) are discussed in chapter 8. These reflexes are mediated above the spinal cord. Therefore, disruption of the spinal cord or brain stem can abolish these reflexes. Of course, the superficial reflexes can also be abolished if there is extensive damage to sensory nerves or lower motor neurons in the region. The "Babinski response" (upgoing toe) is the classic pathological reflex seen with upper motor neuron damage. This reflex replaces the normal plantar response. The findings upon testing of superficial reflexes should be placed in the context of the remainder of the motor exam when evaluating upper and lower motor neurons.

Muscle Bulk
Muscle bulk is primarily assessed by inspection. Symmetry is important, with consideration given to handedness and overall body habitus. Generalized wasting or cachexia should be noted and may reflect systemic disease, including neoplasia. Some areas can be adequately evaluated by inspection alone, such as the thenar and hypothenar regions or the shoulder contour. Some areas, like the thigh, leg, arm and forearm, may be better evaluated by measurement. These measurements can also permit assessment over time. Severe atrophy strongly suggests denervation of a muscle (such as with LMN lesions). This usually begins at least a week after acute injury and gets progressively worse with time (unless reinnervation takes place). Atrophy due to LMN damage must be distinguished from that which occurs secondary to disuse. However, there is usually a clear substrate for disuse (bedrest, cast, etc.) and there is little overall change in strength.

Unfortunately, patients who have limited functional reserve (such a those with prior neural disease or the elderly) can be severely affected by disuse and deconditioning.

Coordination
Coordination is tested as a part of a sequence of movements. Typically the patient is asked to hold his/her hands in front with the palms up, first with the eyes open and then closed (as when examining pronator drift, above). It is usually good form to instruct the patient to prevent movement of his/her hands, and to exert some force either toward the floor or in attempting to push the hands apart. This force can be used to assess the strength of the patient and then should be released suddenly and without warning. After a short excursion, the patient should check this movement, and this checking should be symmetrical. The patient may then be asked to touch his/her nose, and subsequently the examiners finger. This can be repeated a few times to assess the smoothness and accuracy of the movement. Further assessment can be obtained by having the patient perform a rapidly repeated movement such as tapping the thumb and forefinger together, or by having the patient clap his hands. This test can be made somewhat more difficult by having the patient repeatedly strike first the palmar and then the dorsal aspect of one hand against the palm of the other. This, of course, must be done with each hand, and you are evaluating rhythmicity and speed in performance of the movement. Lower extremity coordination can be tested in the supine position by having them attempt to place the heel of one foot on the opposite knee and subsequently tap or slide the heel down the shin to the ankle. This should be done with each leg. Other tests of lower limb coordination include tapping of the foot on the examiners hand, or attempting to draw a number in the air with his/her foot. If the patient can stand and walk, it is usually only necessary to evaluate gait in order to assess lower limb coordination. The patient who can stand on either foot for ten seconds without excessive sway does not need further testing of leg coordination. These maneuvers test several neurologic systems. Strength is required for all of these tests. Excessive rebound (or loss of checking) is suggestive of cerebellar injury on the side of the abnormality. Similarly, difficulty with rapid alternating movements (dysdiadochokinesia) or marked overshoot or undershoot when attempting to hit a target (intention tremor) suggests cerebellar problems on that side. Repetitive over and undershoot during voluntary movement may reflect as "intention tremor". Extreme slowness of movement can be produced by extrapyramidal disease (such as Parkinsons). Of course, problems with any part of the motor systems may affect coordination. For example, if there is a marked alteration in muscle strength, muscle tone, or if the patient

is having abnormal movements this can influence your perception of coordination. Therefore, although tests of coordination are mainly directed toward assessing cerebellar function, you must decide whether other problems in the motor system are affecting these tests.

Muscle Tone
Muscle tone may be increased or decreased, with increased tone being much easier to detect. Tone can be assessed by one of two means. The most common method is for the examiner to passively move the patients limb (especially at the wrist). The second method involves evaluating arm swing (with the patient standing). Tone is often easily checked by having the patient stand with his/her arms hanging loosely at their side. When the patients shoulders are moved back and forth or rotated the arms should dangle freely. Increased tone is usually reflected as the arms being held stiffly both in the standing position and when walking. The lower limbs can be evaluated with the patient seated with the legs dangling. Movement of the feet should result in gentle swinging of the legs of a brief duration. Increased tone results in abrupt restriction on the excursion of the feet. There are two common patterns of pathologically increased tone, spasticity and rigidity. Spasticity is found with upper motor neuron injuries and manifests as a marked resistance to the initiation of rapid passive movement. This initial resistance gives way and then there is less resistance over the remaining range of motion (clasp-knife phenomenon). Rigidity is an increase in tone that persists throughout the passive range of motion. This has been termed "lead pipe" rigidity and is common with extrapyramidal disease, especially Parkinsons disease. Many older individuals have paratonia. This is a phenomenon in which the patient is essentially unable to relax during passive movements. You will note that the resistance is irregular and generally greatest when you change the pattern of movement. Of note, most of these individuals have apparently normal tone when you test them in a standing position and move their shoulders about (as described above). Extreme paratonia is common in patients with dementia. Some types of increased tone appear to be prolongations of voluntary muscle contraction. Myotonia is a slowness of relaxation of muscles after a voluntary contraction or a contraction provoked by muscle percussion. This is a disorder of striated muscle and not an abnormality of innervation and may be seen in conditions such as myotonic dystrophy or congenital myotonia (a disorder of ion channels). Occasionally, metabolic diseases of muscle (such as hypothyroidism) can result in myotonic discharges.

Myotonia can be easily observed by asking the individual to reverse a muscle action quickly (i.e., trying to rapidly open a tightly clenched fist) or by tapping on a muscle belly (such as the thenar muscles). Neuromyotonia is a rare condition of irritability of the nerve (possibly autoimmune) where there is persistent contraction. Muscle contractions are not terminated and the patient becomes "stiff" with movement.

Abnormal movements
There are a number of types of abnormal movements including tremor, chorea, athetosis, dystonia, hemiballism and fasciculations. Each of these has clinical implications that require discussion. Tremor is the most common abnormal movement seen in practice. Three characteristics are of particular importance. These include the symmetry (or asymmetry) of the tremor, the rate of the tremor (basically, whether it is fast or slow, i.e., greater or less than 7 cycles per second) and the circumstances under which the tremor is present (i.e., whether it is worst at rest, during sustained postures or when moving). Physiological tremor comes in two types. Rapid (>7cps) tremor is characteristic of states with increased sympathetic function (think of the last time you had too much coffee). This is most commonly secondary to anxiety, but may occur with increased adrenaline (such a pheochromocytoma) or thyrotoxicosis. A slower tremor must be classified with regard to the conditions in which it is most evident. If it is present predominantly at rest, and decreases with movement, this suggests extrapyramidal disease such as Parkinsons disease (PD). In PD, the tremor is frequently asymmetrical and is usually associated with other signs (bradykinesia, rigidity or delayed postural corrections). Tremors which are severe on sustained postures (such as with the hands outstretched), but which may worsen slightly with action are characteristic of essential tremor (this is also seen in senile tremor or familial tremor). These tremors are absent at rest and are often worsened by anxiety. They are often asymmetrical and characteristically affect the use of writing and eating implements. Damage to cerebellar systems (particularly the hemispheres or dentate connections) often produces a tremor that is most pronounced during voluntary actions. The second most common type of abnormal movement that is seen in practice is fasciculation. These are twitches in muscle (actually, contraction of a single motor unit, i.e., all of the muscle fibers attached to a single motor neuron). These can be felt and often seen. These are random and involuntary occurrences and do not result in movement of a joint. Fasciculations may reflect damage to lower motor neurons, either the cell body or the motor axon located in the nerve root or peripheral nerve. Of course,

if the fasciculations were due to LMN lesions one would expect some weakness, decreased tone and (after a while) atrophy. Also, one would expect that the fasciculations would remain in a single group of muscles for more than transiently. Fasciculations may also be a finding in muscle overuse, or a sign of local muscle irritation. Also, there are some individuals who have benign fasciculations particularly in the calf muscles. Of course, these are not associated with weakness or other motor system abnormalities. There are several other, less common abnormal movements. Chorea is a rapid, fleeting, random and non-stereotyped movement which is worsened by anxiety and which can be suppressed for short periods by conscious effort. They differ from tics since tics are stereotyped and repeat within the same muscle groups. Tics may affect the voice, as well, and consist of repeated throat clearing, sniffing or coughing. Multiple vocal and motor tics are seen in Tourette syndrome. Athetosis is a slow, writhing, snakelike movement of a body part or parts. Dystonia is a sustained twisting of the body, usually the trunk or neck (where it is called torticollis). Hemiballism is a flinging motion of one side of the body, potentially resulting in falls. Involuntary movements are seen in a number of clinical situations. Chorea, athetosis and hemiballism are reflections of basal ganglia disease. This may be congenital (a type of cerebral palsy), post infectious (Sydenham's chorea), hereditary (Huntington's chorea), metabolic (Wilson's disease) or cerebrovascular.

Station
This is the ability to maintain an erect posture. One should be able to stand both with the eyes open and closed with a relatively narrow base of support (the feet close together). You should record excessive sway, falling to one side, or marked worsening in the ability to stand when the eyes are closed. Excessive sway with the eyes open is common with cerebellar or vestibular problems. This may be to one side (and commonly is with vestibular disorders) or may be to both sides (especially with conditions that effect the midline portion of the cerebellum, such as intoxication). You must consider the possibility of other explanation such as the patient not have enough strength to stay upright or severely delayed reactions to destabilization (such as with Parkinsons disease). Some patients can stand well with the eyes open, but have marked increase in instability with the eyes closed. This is suggestive of a disorder of conscious proprioception (i.e., joint position sense, as may be seen with peripheral neuropathy or dorsal column/medial lemniscus dysfunction). This is termed a Romberg sign. Proprioceptive problems on one side can be brought out with standing on one foot. Of course, there are other tests of conscious proprioception,

including evaluation of joint position and vibration sense in the feet. These data must be correlated with the findings on station.

Gait
This is an important part of any neurologic exam. It is particularly important to observe the symmetry of the gait, the ability to walk with a narrow base, the length of the stride when walking at a normal pace, and the ability to turn with a minimum of steps and without loss of equilibrium. When observing a normal person from behind, the medial parts of the feet strike a line and there is no space visible between the legs at the time of heel strike. This is a narrow-based gait and deviation from this can be measured in the amount of distance laterally each foot strikes from the line that their body is following. Tandem walking (the ability to walk on a line) may be used to evaluate for stability of gait, recognizing that many normal elderly patients have trouble with this. Damage to virtually any part of the nervous system may be reflected in gait. An antalgic gait, or the limp caused by pain is familiar to any practitioner. Patients with unilateral weakness may favor one side, and if the weakness is spastic (i.e., from upper motor neuron damage) the patient may hold the lower limb stiffly. S/he will drag the weak limb around the body in a "circumducting" pattern. A staggering or reeling gait (like that of the drunk) is suggestive of cerebellar dysfunction. Generally, the patient with true vertigo will tend to fall to the one side repeatedly (especially with the eyes closed). A patient with foot drop will tend to lift the foot high (steppage gait). Hip girdle weakness often results in a "waddle," with the hips shifting toward the side of weakness when the opposite foot is lifted from the floor (of course if both sides are weak the hips will shift back and forth as they take each step). Patients with Parkinson's disease often have difficulty initiating gait. The steps are usually short though the gait is narrow-based. If severe, the patient may be propulsive (they may even fall). Patients who are "glue footed" (sliding their feet along the ground rather than stepping normally) may be suffering from damage or degeneration of both frontal lobes or the midline portion of the cerebellum. When damage to these areas is severe the patient may be severely retropulsive (tending to fall over backwards repeatedly). Dorsal column injury may result in a gait in which the patient "stamps" his or her feet, and usually also needs to look at the feet in order walk. Patients with painful neuropathy of the feet may walk as if they are "walking on eggs" and patients with spinal stenosis may walk with a stooped posture (a "simian" posture).

Disorders of the motor systems

The reflection of motor system disease depends on the particular part of the motor system that is involved. Here we will discuss the characteristic deficits produced by each level of the motor system.

Muscle disease (see Chapt. 12)

Typically, muscle disease (myopathy) has its earliest and greatest effects on proximal musculature. There is little atrophy (until very late) and deep-tendon reflexes are decreased only in proportion to the weakness. Certain metabolic myopathies may result in cramping due to the fact that energy is required to relax muscles and myotonia may also produce difficulty in relaxation. There are no sensory changes in myopathy.

Neuromuscular disease (see Chapt. 12)

Myasthenia gravis is the prototypical neuromuscular disease. This condition results from autoimmune damage to acetylcholine receptors, which results in inefficient neuromuscular transmission. Initial contraction is strong but during sustained contraction, depletion of neurotransmitter results in progressive weakness. This can be seen during tonic actions (like simply holding up the eyelids or maintaining the arms out in front) or in actions that require sustained activity (like talking or swallowing a meal). For further information see Chapt. 12.

Lower motor neuron (LMN) disease (see Chapt. 12)

These conditions occur due to damage to the anterior horn cells, the ventral roots or the peripheral nerves anywhere along their course to the muscles. In the majority of cases, the weakness is distal. The best explanation for the predominantly distal weakness in neuronal disease is that longer motor (also sensory) nerve fibers are more exposed and vulnerable to the many processes that damage nerve. An exception to this rule is the diffuse polyneuropathy of Guillain-Barre syndrome (presumed to be an autoimmune process). In this case weakness may begin in the proximal muscles and this is presumable because the primary damage to nerves is occurring quite proximally (near the nerve root level). LMN disease results in weakness in the muscles connected with the affected nerve fibers. Understanding of the distribution of nerves and nerve roots to the individual muscles is essential to correct interpretation (Table 10-5). Additionally, there is atrophy (after the first week or two following an acute injury) that is out of proportion to simple disuse. Furthermore, reflexes are usually affected quite early and severely. This is because most conditions that damage LMNs also damage sensory nerve fibers that

represent the afferent limb of the muscle stretch reflex. Finally, when there is damage to LMNs in peripheral nerves, there is often an accompanying sensory loss that can aid in diagnosis of the nerve that is involved.

Upper motor neurons (UMN)

Historically, this has been associated with the corticospinal (pyramidal) tract. However, this is not quite accurate since voluntary motor pathways arising in the cerebral cortex can function by activating more primitive descending tracts from the brain stem. It is clear that the direct projections in the corticospinal tract are responsible for highly skilled movements, especially of the hands. In this section we will refer to direct and indirect corticospinal projections to distinguish the corticospinal tract itself from the indirect activation of other descending motor tracts by cerebral cortical input. Additionally, it must be understood that the motor cortex does not act independently, but rather under the influence of the premotor cortex (involved in planning and initiating movement) as well as "extrapyramidal" systems such as the basal ganglia and cerebellum (see below). The classic picture of acute damage to UMNs includes contralateral paralysis of distal limb movements, while proximal limb movements are severely weakened and trunk movement minimally involved. Muscle tone (measured as passive resistance to manipulation) is depressed in this initial phase. The deep-tendon reflexes are also likely to be absent, recovering over time to normal or hyperactive levels. The superficial reflexes (abdominal and cremasteric) opposite the lesion are depressed or absent. A Babinski response is often present on the weak side. Over weeks to months proximal strength improves to a significant degree, whereas distal movements make only a poor recovery. A rudimentary grasping capability is frequently all that remains in the hand. Extension, opposition, and individual finger movements remain severely affected or lost. Presumably, the recovery of proximal functions relates to some bilaterality of distribution of corticospinal fibers that innervate proximal muscles. The modest recovery of distal movements is suspected to relate to preserved motor pathways from the brain stem (presumably under extrapyramidal control). Damage to the precentral gyrus (primary motor cortex) or isolated damage to the medullary pyramid produces a rather pure corticospinal tract lesion. In these cases, the weakness of distal muscles is severe but there is little appearance of other findings such as spasticity and hyperreflexia that are hallmarks of most UMN lesions. Other UMN

lesions also damage indirect descending connections between the cerebral cortex and spinal cord. This happens with lesions of the premotor cortex, corona radiata, internal capsule, cerebral peduncle, basal pons, and lateral columns of the spinal cord. Invariably, lesions in these areas also involve other cerebrofugal pathways that are intermixed with the direct corticospinal (pyramidal) projection. In all of these cases (in addition to the weakness), there is a decrease in tonic inhibition of reflexes and an increase in resting muscle tone. This is accompanied by hyperactivity of the deep-tendon reflexes and development of what is traditionally called spasticity. Spasticity is elicited during passive manipulation of the muscles. The muscles at rest do not have excessive tone but any brisk stretch of a muscle group (particularly the flexors in the upper extremity and the extensors in the lower extremity) will result in a "catch" at about midlength of the muscle followed by a sudden release of the catch and relaxation of the muscle. The last two components, the catch and release, have been likened to a closing pen knife, which is the origin of the term "clasp-knife" spasticity. Hyperactive deep-tendon reflexes and spasticity have a similar mechanism (overactive muscle stretch reflexes). The giving away or release portion of the clasp-knife phenomenon is presumed to be caused by increased firing of the inhibitory Golgi tendon organs, which produce an overactive reflex to inhibit the muscle. If the lesion extends beyond the confines of the traditional corticospinal path, more descending pathways are involved and a greater degree of spasticity is noted; also there is a poorer recovery from weakness. This is presumably because of loss of more inhibitory influences on the segmental reflex arc and loss of more facilitatory influences on the motor neuron effector systems. After very acute lesions of the descending motor systems there is often initial flacid weakness that is sometimes followed by stereotyped movements and postures (decorticate posture, decerebrate posture or generalized withdrawal reactions)(Fig. 101). Acute destructive lesions of the descending motor pathways cause a transient shock state of flaccid, areflexic paralysis. When progressively greater amounts of the descending pathways are involved, a longer period of shock ensues. Acute cortical destruction may result in only hours to days of shock, whereas acute transection of the spinal cord can cause a shock state that persists for many weeks to months before spastic hyperreflexia and rudimentary spinal reflex behavior return. The precise pathophysiology of spinal shock is not clear, but it may complicate the evaluation of the patient following acute injury. It is always difficult to predict the final extent of the neurological injury in the setting of shock. Chronic or slowly progressive destruction of the descending motor pathways is not associated with a shock state. Presumably this is

because compensatory reorganization of the motor function occurs in pace with the losses. Lesions that extensively destroy the cerebral cortex and basal ganglia, and preserve at least some of the diencephalon (like those caused by severe hypoxia) may result in stereotyped motor responses that involve flexion of the upper extremities and extension of the lower extremities. Noxious stimulation is usually necessary to elicit this reflex activity, which has been called decorticate posturing (Fig. 10-1). It has been thought, on the basis of experimental data, that release of the rubrospinal motor system is, at least in part, responsible for decorticate posturing. Transection of the brain stem, for example by stroke, at the level of the midbrain or pons is followed after a period of neuraxis shock by severe spasticity and reflex extension and pronation of the upper extremities with extension of the lower extremities and trunk on noxious stimulation (see Fig. 10-1). This response is called decerebrate posturing and depends on preservation of the vestibular nuclei in the caudal brain stem, with the extension being produced by vestibulospinal pathways. Lesions transecting the lowest portion of the brain stem or the upper spinal cord result in quadriplegia and severe spasticity after a period of shock. In time, reflex flexion movements can be elicited with noxious stimulation (see Fig. 10-1). These probably represent primitive spinal withdrawal responses. As a rule, UMN lesions affect large areas of the body below the level of injury. It is often difficult to localize the specific level of damage by the pattern of weakness. Associated neurologic findings may clarify the level. For example, cranial nerve involvement or involvement of nerves or nerve roots may indicate a brain stem or spinal cord level of involvement, respectively, while cortical findings such as language difficulties, visual field abnormalities, dyspraxias, or other disorders of higher integrative function suggest cortical damage. In most UMN lesions, the whole side of the body below the lesion is affected (hemiparesis or hemiplegia). However, in the cerebral cortex the motor representation for the arm, face, and trunk lie within the supply of the middle cerebral artery, whereas the leg lies within the distribution of the anterior cerebral artery (Fig. 10-2). Loss of middle cerebral cortical perfusion therefore causes a greater degree of weakness of the upper extremity than of the lower extremity. Occlusion of the anterior cerebral artery, an uncommon event, is associated with greater weakness in the leg than in the arm. Because sensory and motor systems are near one another through the spinal cord, most of the brain stem and the cerebral hemispheres, it is common to have some sensory as well as motor symptoms. The sensory abnormality (see Chapt. 9) may help localize the

lesion. Pure involvment of UMNs without any sensory damage is most often seen with small lesions (usually vascular) in the posterior limb of the internal capsule or in the base of the pons.

Basal Ganglia
The abnormalities associated with lesions and degenerative processes in the basal ganglia are discussed in some detail in Chapter 18. The findings are generally categorized into "hyperkinesias" and "hypokinesias". The classic picture of parkinsonism (the most common cause being Parkinson disease) includes bradykinesia (slow movements), rigidity, difficulty initiating movements and delayed postural corrections. These symptoms all fall into the category of "hypokinesia". There may also be a tremor at rest (suppressed by movement), which is a form of hyperkinesia. The rigidity of parkinsonism is present in all ranges of passive manipulation and cannot be abolished by sectioning the dorsal roots. Therefore, it is not due to reflex overactivity (deep tendon reflexes are normal in parkinsonism). It is probably due to tonic overactivity of the descending motor pathways and it can be abolished by cutting descending motor tracts (see Chap. 18). Other types of "hyperkinesia" include chorea, athetosis, hemiballism, tic and dystonia. These are indicative of dysfunction of the basal ganglia (extrapyramidal) system. However, they are not diagnostic of a particular cause (see Chap. 18).

Cerebellum
Cerebellar disease produces predominantly motor symptoms. There are three main parts of the cerebellum, which have slightly different functions. The lateral cerebellar hemispheres (the neocerebellum) are involved in controlling distal limb movement of the ipsilateral limbs. The vermis of the cerebellum (midline) is involved in control of axial functions as well as the voice and eye movements. The posteroinferiorly-located vestibulocerebellum (floculonodular lobe; archicerebellum) is involved in vestibular functions and regulation of the vestibulo-ocular reflex (see Chapt. 6). Damage to the neocerebellum produces predominant symptoms of tremor, ataxia, and hypotonia. The tremor is of a particular type, consisting of rhythmic, variably 3-8 per second oscillations that occurs predominantly on voluntary activity and reaches its peak of oscillation toward the end of the movement. It disappears with posturing or at rest. It is noticed dramatically when reaching for objects (such as when performing finger-to-nose testing. The ataxia (incoordination) is manifest in several ways. There is dysmetria (past-pointing) with overshoot and/or undershoot of the target. Also, there

may be lack of checking (excessive rebound). For example, if the patient is asked to hold their hand extended out in front of them while pressure is applied and then suddenly released, there will be excessive movement before the patient "checks" the motion. Additionally, the patient will have difficulty performing a rapidly repeated motions (tapping fingers, patting hands or tapping feet) and this may be even more obvious if there is rapid alternating movements involved in the motion (such as pronation and supination of the hands). Ataxia of the legs is manifest in difficult in walking, often characterized by a broad-based and/or drunken gait. Diosrders affecting the midline cerebellum (vermis) effect axial motor activity. This is likely to be manifest as head and trunk instability as well as speech and eye movement problems. The problems with trunk stability are usually brought out during attempts to stand still or to walk. When there is both instability of the trunk and ataxia of the legs patients will have severe ataxia. After vermal lesions, the speech may sound drunken or inappropriately staccato and eye movements may be erratic and uncoordinated when patients have damage to the vermis. Because it is an important symptom of cerebellar disease, it would be appropriate to say a few more words about ataxia. Cerebellar ataxia is fairly easy to observe in the office and it has at least two origins: (1) intention tremor of the legs, giving a dysmetric gait, and (2) truncal imbalance. If it is advanced, the patient has a wide-based compensatory gait, and if there is lateralized limb involvement, they tend to lean and fall toward the affected side. A sensitive test for ataxia is heel-to-toe tandem walking; this should be part of any neurologic screening examination in a patient with gait or balance complaints because it detects early cerebellar dysfunction. If the trunk alone is involved, as in early alcoholic degeneration or with a tumor of the vermis, there is a tendency to fall to either side, forward or backward. Some persons with midline cerebellar damage may have a stronger tendency to fall backward. This is called retropulsion and can also be seen in basal ganglia dysfunction (particularly parkinsonism) and in frontal lobe disorders. When retropulsion is due to cerebellar involvement, it frequently has an involuntary tonic character, i.e., the patients actually appear to be actively pushing themselves backward. Even at rest, sitting or standing, there is a tendency to lean or fall backward. With frontal lobe dysfunction and parkinsonism, the retropulsion is usually passive rather than active, i.e., the patient has difficulty recovering from being pushed backward or from a backward-leaning position, but he has no active or forced retropulsion at rest. Damage to the vestibulocerebellum (flocculonodular lobe; archicerebellum) produces vestibular findings, including nystagmus that may be quite severe and in different directions depending on which way the patient is looking ("gaze-shifting

nystagmus"). This is often more severe than symptoms due to vestibular damage since vestibulocerebellar damage is more difficult to compensate for. Finally, cerebellar damage can occasionally be reflected in hypotonia. The examiner should check for tone abnormalities by asking the patient to relax and not resist. The limbs are then moved rapidly by the examiner in several ranges. A lack of resistance or a floppiness is noticed with hypotonia. Having the patient sit with his legs swinging free may test the legs. The leg is lifted by the examiner and released. Normally the leg swings back and forth several times and then stops, arrested by inertia and the normal resting muscle tone, which is a manifestation of the sensitivity of the normal muscle stretch reflex. With cerebellar hypotonia, the leg swings freely, unchecked, like a pendulum, arrested mainly by passive limb inertia.

References
Brodal, A.: Neurological Anatomy in Relation to Clinical Medicine, ed. 2, New

York, Oxford University Press, 1969.

Medical Council of the U.K.: Aids to the Examination of the Peripheral Nervous

System, Palo Alto, Calif., Pendragon House, 1978.

Monrad-Krohn, G.H., Refsum, S.: The Clinical Examination of the Nervous

System, ed. 12, London, H.K. Lewis & Co., 1964.

Wolf, J.K.: Segmental Neurology, A Guide to the Examination and Interpretation

of Sensory and Motor Function, Baltimore, University Park Press, 1981.

Questions
Define the following terms:

spasticity, rigidity, hemiparesis/plegia, bradykinesia, paraparesis/plegia, upper motor neurons, lower motor neurons, internal capsule, chorea, athetosis, dystonia, hemiballism, tic, fasciculation.
Spasticity is a resistance to passive movements that is greatest at the initiation of motion (particularly of a rapid movement). It is often a sign of overactive muscle stretch reflexes. Hemiparesis/plegia paralysis or paresis (weakness) of one side of the body. Rigidity is a smooth resistance to passive movement that occurs throughout the range of motion and usually results from extrapyramidal disorders such as Parkinson's disease. Bradykinesia is a pathological slowing of motor performance often seen with Parkinson's disease and parkinsonism. Paraparesis/plegia paralysis or paresis of both lower extremities.

Upper motor neurons are the principal descending motor pathways for voluntary movement, including the corticospinal and corticobulbar tracts (and some other associated tracts). Lower motor neurons are the anterior horn motor neurons and their axons that extend through the ventral nerve root and the peripheral nerves to reach the neuromuscular junction. The internal capsule is the primary locus through which the upper motor neuron (corticospinal and corticobulbar) pathways descend. Chorea a purposeless, involuntary, random twitching movement. Athetosis a purposeless, involuntary writhing movement. Dystonia is an involuntary, sustained twisting position of the body or a body part (torticollis, for example, when it involves the head). Hemiballism a repeated, involuntary, flinging or flipping movement of a part of the body on one side, usually due to damage to the subthalamic nucleus. Tic is a rapid movement, tending to be repeated in the same pattern over and over. Fasciculation an involuntary twitching of individual motor units, usually visible as a rippling of the skin but usually not resulting in any actual movement of the body part.

10-1. Describe the course of "upper motor neurons".

Answer 10-1. Upper motor neurons (coticospinal and corticobulbar tracts) arise in the motor cortex, traverse the internal capsule, cerebral peduncle and pyramids of the brain stem.

10-2. Over what functions do the upper motor neurons exert the greatest control (what movements are most effected by damage)?
Answer 10-2. They are mostly involved in control of distal movements (such as hand and fingers). Proximal functions (such as shoulder shrug) have bilateral control.

10-3. Where are sites of potential lesion producing lower motor neurons signs and symptoms?
Answer 10-3. Lower motor neuron damage can be anywhere along the pathway from the anterior horn motor neuron, ventral root, plexus or peripheral nerve.

10-4. What are the features of lower motor neuron damage?

Answer 10-4. Lower motor neuron damage results in decreased reflex and usually atrophy. It may also produce fasciculations.

10-5. What is the significance of fasciculations?

Answer 10-5. Diffuse, persistent and extensive fasciculations suggests motor neuron disease or damage (transient fasciculations are common and benign if unaccompanied by weakness or reflex change).

10-6. What are the characteristics of peripheral nerve damage?

Answer 10-6. Effects of nerve damage are most often seen distally, reflexes are affected early and atrophy is often present.

10-7. What are the characteristics of muscle disease?

Answer 10-7. Symptoms are usually most evident proximally, there is no sensory loss, reflexes only affected late and atrophy is not severe.

10-8. What are the characteristics of basal ganglia disease?

Answer 10-8. Muscle tone, postures, and patterned movements are most affected. Parkinsonism is common, with bradykinesia, difficulty initiating movements, delayed postural reflex responses and rigidity. Abnormal movements at rest are common: resting tremor, chorea, athetosis, dystonia, hemiballism.

10-9. What are the characteristics of cerebellar disease?