DEFINITION

Headache is a pain in all part of the head that may also be felt in the face and neck. A
classification system developed by the International Headache Society characterized headache
as primary or secondary.

Primary headaches are those in which headache and its associated features are the
disorder itself. Primary headaches may be episodic which means they occur occasionally or they
may be chronic. Chronic headaches strike at least four times a month and usually affect a
person for most or all of his or her life. The most common are migraine ,tension-type headache
and cluster headache.

Secondary headache are those cause by exogenous disorders. Infectious diseases such
as the common cold, influenza and pneumonia often produces secondary headaches as does an
allergic reaction or sinus infection. Secondary headaches are also a symptom of the eye disease
glaucoma, high blood pressure, stroke, head injury ,a brain tumor and a teeth-grinding
condition known as temporomandibularjoint (TMJ) syndrome.

A classification system developed by the primary International Headache Society

characterizes headache as primary or secondary ( Table 1.1).

PRIMARY HEADACHE SECONDARY HEADACHE

Type % Type %
Tension-type 69 Systemic infection 63
Migraine 16 Head injury 4
Idiopathic stabbing 2 Vascular disorders 1
Exertional 1 Subarachnoid hemorrhage <1
Cluster 0.1 Brain tumor 0.1

PATHOPHYSIOLOGY

Headache is caused by traction, displacement, vascular spasm, or distention of the pain

sensitive structures in the head or neck. Isolated involvement of the bony skull ,most of the
dura or most regions of brain parenchyma does not produce pain.

Few cranial structures are pain-producing and these include the venous sinuses, the
anterior and midline meningeal arteries ;the dura at the base of the skull ; the trigeminal (V)
,glossopharyngeal (IX)and vagus (X) nerves ; the proximal portion of the internal carotid artery
and its branches near the Circle of Willies ; the brainstem periaqueductal gray matter ; and the
sensory nuclei of the thalamus. Extracranial pain sensitive structures include periosteum of the
skull ; the skin ; the subcutaneous tissues, muscles and arteries ; the neck muscles ; the second
and third cervical nerves ; the eyes, ears , teeth, sinuses and oropharynx and the mucous
membranes of the nasal cavity.

The trigeminal(V) nerve carries sensation from intracranial structures in the anterior and
middle fossae of the skull abpve the cerebellar tentorium.Discrete intracraniallesions in these
locations can produce pain that radiates in the trigeminal nerve distribution.

The glossopharyngeal (IX) and vagus (X) nerves supply part of the posterior fossa ; pain
originating in this area may also be referred to the ear or throat (eg :glossopharyngeal
neuralgia).

The upper cervical nerves transmit stimuli arising from the infratentorial and cervical
structures ; therefore pain from posterior fossa lesions projects to the second and third cervical
dermatomes.

The key structures involved in primary headache appear to be :

 The larger intracranial vessels and dura mater and the peripheral terminals of the
trigeminal nerve that innervate these structures.
 The caudal portion of the trigeminal nucleus which extends into the dorsal horns of the
upper cervical spinal cord and receives input from the first and second cervical nerve
roots.(the trigeminocervical complex)
 Rostral pain-processing regions such as the venteroposteromedial thalamus and cortex.
 The pain modulatory system in the brain that modulates input from trigeminal
nociceptors at all levels of the pain processing pathways.
ETIOLOGY

Headache

Muscle
Vascular Inflammatory Distortion Idiopathic
contraction

Tension Giant cell Trigeminal

Migraine Brain tumor
headache artritis neuralgia

Meningitis

Figure 1 Etiology of headache

Figure 2 Etiology of headache

HISTORY

An approach to the headache history:

A. How many different headache types does the patient experience?

B. Time questions
 Why consulting now?
 How recent in onset?
 How frequent and what temporal pattern (especially distinguishing between
episodic and daily or remitting)?
 How long lasting?
C. Character questions
 Intensity of pain?
 Nature and quality of pain?
 Site and spread of pain?
D. Cause of questions
 Predisposing and/or trigger factors?
 Aggravating and/or relieving factors?
 Family history of similar headache?
E. Response to headache questions
 What does the patient do during the headache?
 How much is activity (function) limited or prevented?
 What medication has been and is used and in what manner?
F. State of health between attacks
 Completely well,or residual or persisting symptoms?
 Concern, anxieties , fears about recurrent attacks and/or their cause?

Warning features in the history warranting investigation

 Acute thunderclap headache (intense headache with abrupt or “explosive” onset)

 Headache with atypical aura (duration>1 hour, or including motor weakness)
 New onset headache in a patient younger than 10 years or older 50 years.
 Progressive headache , worsening over weeks or longer
 Fever
 Symptoms of raised intracranial pressure:
Drowsiness
Postural-related headache
 Vomiting
 New onset seizures
 History of cancer or HIV infections
 Cognitive or personality changes
 Progressive neurologic deficit:
Progressive weakness
Sensory loss
Dysphasia
Ataxia
 Pain inducedby bending,lifting,coughing
 Pain that disturbs sleep or presents immediately upon awakening
 Pain associated with local tenderness eg: region of temporal artery

Classification and Approach to the Differential Diagnosis

a. Acute Headaches and Facial Pain

Headaches that are new in onset and different from any patient has experienced
previously commonly a symptom of serious illness.The sudden onset of “worst
headache in my life”(classic subarachnoid hemorrhage), diffuse headache with neck
stiffness and fever (meningitis) and head pain centered about one eye (acute glaucoma).

b. Subacute Headaches and Facial Pain

Occurs over a period of weeks to months.May signify serious medical disorders if the
pain is progressive or when it develops in elderly patients.Inquiries should be made on
recent head trauma, history of malaise fever or neck stiffness,other neurologic
abnormalities,weightloss,symptoms of vasculitis and medical conditions.

c. Chronic headaches and facial pain

Headaches that have occurred for years (migraine) usually have benign cause.

Characteristics of pain

Pulsating, throbbing Migraine , Tension headache

Tightness or pressure Tension headache
Dull, Steady Intracranial mass lesion
Sharp, Lancinating Trigeminal neuralgia
Ice pick-like Migraine, cluster headache ,Giant cell arteritis
Location of pain

Unilateral Cluster headache, Migraine

Ocular or retroocular Primary opthalmologic disorder
eg : Acute iritis, glaucoma,optic neuritis, also
common in migraine or cluster headache
Paranasal Acute infection, outlet obstruction
Focal Intracranial mass lesions
Bandlike or occipital Tension headaches, meningeal irritation
1st division of trigeminal nerve Postherpetic neuralgia
2nd and 3rd division of trigeminal nerve Tic douloureux
Pharynx and external auditory meatus Glossopharyngeal neuralgia

Other features of headache

A.Temporal pattern of headaches

Headaches from mass lesion  Maximal on awakening

 Increase in severity over time
Cluster headaches  Awaken patient from sleep
 Recur at the same time each day or
night
Tension headaches  Stressful situation occurs
 Maximal at the end of the day
Migraine  Episodic
 Worse during menses

B.Conditions relieving headaches

Migraine  Darkness
 Sleep
 Vomiting
 Pressing on the ipsilateral temporal
artery
 Often diminished during pregnancy
Post-lumbar puncture and low pressure  Recumbency
headaches
Intracranial mass lesion  Less severe with patient standing
C. Conditions exacerbating headaches

Intracranial mass lesions  Rapid changes in head position

 Coughing
 Sneezing
Migraine,tension headaches  Anger,excitement,irritation
 Coughing, sneezing
Sinusitis  Stooping
 Bending forward
 Sneezing
 Blowing the nose
Postural headache  Maximal when upright
( low CSF,Lumbarpuncture,head injury)  Nearly absent when lying down
Subdural hematoma  Fluctuations in intensity and duration
with no obvious cause
 Fluctuation in mental status

PHYSICAL EXAMINATION

Vital Signs

A. Temperature
Fever suggests a viral syndrome, meningitis, encephalitis or brain abscess ,headachese from
these causes can occur without fever.

B. Pulse
Tachycardia can occur in a tense ,anxious patients with a tension headache or accompany any
severe pain. Paroxysmal headache is associated with tachycardia .

C. Blood pressure
Chronic hypertension is major risk factor for stroke and can be associated with acute headache.
Subarachnoid hemorrhage commonly followed by marked acute blood pressure.

D. Respiration
Hypercapnia from respiratory insufficiency can elevate intracranial pressure and produce
headache.
General Physical Examination

A. Weight Loss
Weight loss or cachexia in a patient with headache suggests the presence of cancer or chronic
infections.

B. Skin
Focal cellulitis of the face may be the source of intracranial abscess or venous sinus
thrombosis.Cutaneous abnormalities may suggest vasculitis, endocarditis or cancer.The café au
lait spot may associated with benign or malignant intracranialtumors that can produce
headache.

C. Scalp, face and head

Scalp tenderness Migraine, subdural hematoma, giant cell arteritis,

post herpetic neuralgia
Nodularity, erythema , tenderness over temporal Giant cell arteritis
artery
Localized tenderness Acute migraine, recent head trauma, mass lesion
Skull tenderness Paget disease,myeloma,met.cancer of skull
Sinus tenderness Sinusitis
Tongue laceration Postictal headache
Ipsilateral conjunctival injection, lacrimation, Cluster headache
Horner syndrome,Rhinorrhea

D. Neck
 Cervical muscle spasms occur with tension and migraine headaches, cervical spine injuries,
cervical arthritis or meningitis. Carotid bruits may be associated with cerebrovascular disease.
 Meningeal irritation cause nuchal rigidity in anteroposterior direction, cervical spine disorders
restrict movement in all directions. Brudzinski sign indicated meningeal irritation.
 Lhermitte’s phenomena produces an electric shock feeling when the neck is forward flexion or
extension.This indicates cervical pathology usually dyemyelination.

NEUROLOGIC EXAMINATION

Mental status

The mental status evaluation begins with the basic determination of using A,V,P,U method.
A=alert,V=Responsive to verbal stimuli or commnds, P=Responsive to pain stimuli only and
U=Unresponsive.The Glasgow Coma Scale (GCS) was developed for traumatic brain injury.

Neurologic Examination

In general, CN II - VIII are of the most value in the neurologic screening exam ,testing the other cranial
nerves is complaint based.
CN II:
Testing the optic nerve involves visual acuity (central vision), gross visual fields (peripheral
vision), and the ophthalmoscopic exam. Visual acuity must be checked individually in each eye. Changes
in visual acuity are due to either refractive error or optic nerve dysfunction. Optic nerve function is
tested using the swinging flashlight test. Peripheral vision is checked by a visual field exam, testing each
eye individually using the confrontation method. Blindness in one eye suggests a lesion in front of the
chiasm; bitemporal hemianopsia suggests a lesion at the optic chiasm; a quadrant deficit suggests a
lesion in the optic tracts; bilateral blindness suggests cortical disease. The ophthalmoscopic exam
evaluates the optic disc, retinal arteries, and retinal veins. Pearls in diagnosing papilledema include
blurring of the lateral margin of the optic disc, loss of blood vessels as they cross the edematous disc,
distended veins, and loss of venous pulsations.

CN III, IV, VI:

CN III, innervates the extraocular muscles effecting primarily adduction and vertical gaze. CN III
function is tested in conjunction with CN IV, which aids in internal depression via the superior oblique,
and CN VI, which controls abduction via the lateral rectus. Extraocular muscle function is tested by
holding a pen vertically in front of the patient. Instructing the patient to follow while the pen is moved
horizontally, the patient is asked how many pens he sees (split images suggests diplopia). This is
repeated in the horizontal plane. Diplopia requires binocular vision and thus will resolve when one eye is
occluded (dislocated lens or retinal detachment can cause distorted images but not split images). While
checking the extraocular movements, the presence of nystagmus is noted. Slight nystagmus on extreme
lateral gaze may be normal. Marked nystagmus on lateral gaze or any nystagmus on vertical gaze is
abnormal, suggesting a peripheral or central nervous system lesion. Vertical nystagmus is seen in
brainstem lesions or intoxication with phencyclidine. Pendular nystagmus (equal to and fro movements)
is generally a congenital condition.

The pupillary light reflex is mediated via the parasympathetic nerve fibers which run on the
outside of CN III. The pupillary response is tested by shining a light individually into each eye.In the
swinging flashlight test a light in shined from one eye to the other; when the light is shined directly into
a normal eye, both eyes constrict via the direct light response and the consensual light response. A
Marcus-Gunn pupil is a pupil that constricts to the consensual response but dilates when light is shined
directly into it indicating an afferent nerve defect; this is seen in optic nerve disease, e.g. multiple
sclerosis.
Pupillary size must be observed and documented. Asymmetric pupils of <1mm are of no
pathologic significance. A larger difference in pupil size suggests a CN III compression; this can result
from compression of the nerve by aneurysms, or, in patients with an altered mental status, by cerebral
herniation. Bilateral pupillary dilation is seen with prolonged anoxia or due to drugs (anticholinergic,
sympathomimetic agents). Unilateral pupillary constriction in seen in sympathetic chain dysfunction, e.g.
Horner’s syndrome or carotid artery dissection. Bilateral pupillary constriction is seen with pontine
hemorrhage or as the result of drugs (e.g. opiates, organophosphates, clonidine).
CN V:
The trigeminal nerve has both sensory and motor function. A central nervous system lesion
affecting CN V usually involves all three branches, making individual testing unnecessary. Sensory
function is most reliably tested using double simultaneous stimulation, see below in the section on
sensory testing. CN V also supplies the muscles of mastication which are tested by palpating the
masseter muscles while the teeth are clenched.

CN VII:
The facial nerve innervates the motor function to the anterior scalp and face, andnsensory to
the anterior two-thirds of the tongue, to the ear canal and behind the ear. Weakness is often apparent
as a facial droop or flattening of the nasolabial fold. In more subtle cases, weakness becomes apparent
by asking the patient to show his teeth or whistle. Central lesions cause contra-lateral weakness of the
face below the eyes but with forehead sparing due to the crossing of central fibers; thus with central
lesions the eyebrows can be elevated and the eyes tightly closed. Patients with peripheral VIIth nerve
lesions, including Bell’s Palsy, may have a complete facial weakness that includes the forehead. Of note,
an isolated central VIIth lesion is rare, therefore absence of brow and forehead involvement does not
necessarily indicate a central lesion, especially if the patient is otherwise neurologically intact.

CN VIII:
The acoustic nerve has a vestibular and a cochlear component. The vestibular nerve supplies the
semi-circular canals, the cochlear nerve supplies the Organ of Corti (hearing). Decreased hearing is due
to either a conductive defect (e.g. wax in the ear canal) or a sensorineural defect. An easy screening test
for hearing defects is accomplished by asking the patient to hum; in conductive defects, the affected ear
hears the hum louder. In sensorineural defects it is the normal ear which hears the louder hum. When
vestibular nerve defects are suspected, patients are assessed for nystagmus and a Nylen-Barany
maneuver is performed by rapidly taking the patient from a sitting to supine position where the head
lies 45 degrees below the horizontal. The test is repeated with the patient’s head turned 30-45 degrees
to the right and then to the left. The test is considered positive with either the onset of nystagmus,
reproduction of vertigo, or both, and suggests a peripheral cause of vertigo. The nystagmus of
peripheral vertigo has a brief period of latency prior to onset, is suppressible by visual fixation, is
fatigable, and worsens with changes in position. Central vertigo has nystagmus of immediate onset and
is not suppressible, fatigable, or positional.

Motor exam
The motor screening exam focuses on detecting asymmetric strength deficits since this may be
an indication of an acute CNS lesion. Testing motor strength is difficult or impossible in the
uncooperative patient, and results may be significantly affected by pain. In general, it is not necessary to
test every muscle group individually but instead to test for the presence of a "drift". The patient, with
eyes closed, holds his arm out horizontally, palms up, for 30 seconds. If weakness is present, the hand
and arm on the affected side will slowly drift or pronate. Lower extremity drift can be checked by having
the patient lie prone with knees bent 90 degrees and legs pointing vertically up. A weak leg will tend to
drift downwards within 30 seconds. Other motor tests for extremity weakness include hand grasp, and
foot plantar and dorsiflexion. Hand grasp testing of the ulnar aspect of the hand is more reliable than
the radial aspect. In the case of possible peripheral nerve or muscle injury, or in the case of abnormal
results with motor testing as described above, more formal testing of the extremity is in order.

Reflexes
Reflex testing in the screening exam includes the major deep tendon reflexes and the planter
reflex (Babinski’s Sign). The major deep tendon reflexes are the patellar reflex (L3,4), Achilles
reflex (S1,2), the biceps reflex (C5,6), and the triceps reflex (C7,8). The response is graded from
O (no reflex) to 4+ (hyperreflexia). Symmetrical hyporeflexia may be normal, or indicative of
metabolic derangements or of a peripheral neuropathy. Symmetrical hyperreflexia may also be
systemic in origin, frequently resulting from hypocalcemia or hyperthyroidism. Unilateral
hyperreflexia suggests an upper motor neuron process. Asymmetrical reflexes are generally
pathologic. There are several reflexes which indicate upper motor neuron disease, the most
commonly elicited is the planter flexion/extension reflex (Babinski reflex). A normal response to
stroking the planter surface of the foot is plantar flexion of the toes; a positive Babinski reflex is
characterized by dorsiflexion of the big toe with fanning out of the smaller toes.

Sensory exam

The screening sensory exam consists of testing pain and light touch; the spinothalamic
tract in the anterior cord carries temperature and both pain and light touch fibers, while the
posterior column carries fibers for light touch thus explaining preservation of light touch in
patients with anterior cord syndromes. Testing for proprioception, stereognosis and vibration is
generally reserved for patients with suspected neuropathies or further evaluation of sensory
complaints. Dermatomal testing is performed in patients with suspected radiculopathies since
central lesions do not present with dermatomal deficits. Evaluating sensation in patients with
vague complaints is best done by testing for extinction on double simultaneous stimulation. In
this test, the patient closes his eyes and two sharp objects are simultaneously applied. If the
patient reports sharpness on one side only, a sensory deficit on the other side is present. If both
sides are initially reported as sharp, repeated testing is performed looking for extinction of
response on the affected side.

Coordination and Balance

Coordination is a function of the cerebellum combined with input from vision,
proprioception, and vestibular sense. Cerebellar function can be easily evaluated by asking the
patient to close his eyes and rapidly alternate touching the finger from one hand between a
finger on the other hand and his nose. A variation of this test involves the patient rapidly
alternating touching his finger from his nose to the examiner’s finger though in this case the
patient must open his eyes and therefore visual problems may interfere with performance.
Heel-to-shin testing is another frequently used test of cerebellar function. Difficulty in
performing rapid alternating movements may also demonstrate cerebellar pathology. Balance
requires an integration of cerebellar, visual, vestibular, and proprioception input. It is evaluated
by the Romberg test and tandem gait (heel-to-toe). In the Romberg test, the patient is asked to
stand with his feet together (proprioceceptive input), without support; first with eyes open
(visual input) and then with eyes closed. Closing the eyes eliminates vision, but with
proprioception and vestibular sense intact, the patient will not sway. If there is a proprioceptive
deficit, the patient will keep his balance with the eyes open and lose his balance when the eyes
are closed. When this occurs, the Romberg test is positive. If the patient sways with the eyes
open or closed, this is suggestive of a cerebellar lesion which is not compensated by sensory
input from the other systems.