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Symmetrical polyneuropathies
Focal/multifocal neuropathies :
Cranial neuropathies(Bells Palsy,Ophthal-moplegia,Trigeminal neuralgia) Trunk & limb neuropathy(Carpal tunnel syndrome CTS- , Tarsal TS,Peroneal Palsy) Proximal motor neuropathy
Autonomic neuropathies:
Cardiovascular disturbances(postural hypertension, cardiac denervation) Thermoregulatory disorders(distal anhidrosis, gustatoory sweating, abnormal vasomotor response to change in temperature) Alimentory tract disorders(GItract atony) Genitourinary disturbances(bladder atony,impotence) Disturbances of respiratory control(cardiorespiratory arrest)
Neurogenic bladder
Failure to store urine due to a hyperactive bladder (CNS, cortical, subcortical disorders) Failure to evacuate urine due to PNS disorder (atonic bladder)
Guillain-Barre syndrome
Acute inflammatory demyelinating polyneuropathy(AIDP) Immunologically mediated An unclassical form of polyneuropathy Cause undetermined, often preceded by an infection, viruses, HIV, enteritis
Confirmatory tests
High cerebro-spinal fluid (CSF) protein, no pleocytosis (cells) = albumino cytologic dissociation. Electromyography (EMG) and Nerve conduction velocity (NCV) signs of demyelination. Treatment plasma exchange, plasma pheresis, Immunoglobulin I.V.
Most common in cervical (neck) and lumbar (LBP) regions, with Radiating pain, weakness, loss of tendon reflexes, opisthotonus, Lasegue and Kernig signs. Cervical root syndromes, C5-6 and C78 radiculopathies. Lumbar root syndromes, L4-L5-S1
Most common cause: herniated intervertebral disk/slipped disk, mass lesions, structural abnormalities. Symptoms and severity depends on: location and type of herniation (central disk herniation may result in conus cauda syndrome, lateral disk herniation may result in root syndromes)
Spinal cord
Spondylolisthesis
Brainstem lesions :
Mesencephalon, Pons, Medulla oblongata infarct, due to ischemic vascular occlusion of Circulus Willis cranial arteries at the base of the brain, Causing dysfunctions of cranial nerves.
Horner syndrome:
Ptosis,miosis, anhidrosis,enophthamos Due to disruption of sympathetic pathway in cervical region Cause not always detectable, most often due to vascular, infection, tumours, trauma.
Hemiplegia alternans :
Lesions of the bulb (med.obl.) of the last four cranial nerves IX,X,XI,XII, also called Wallenbergs syndrome. Contralateral hemiplegia due to crossing/decussation of lateral corticospinal tract in lower medulla oblongata.
Total tranverse lesion of myelum - cord spinal will cause disruption of motor, somato-sensory and autonomic fibers. Clinical features will be of upper motor neuron type. Cause: trauma,tumor,vascular obstruction. Brown-Sequard syndrome is a hemi transverse lesion of the myelum.
Cranial arteritis/vasculitis
Inflammation of bloodvessels, primary or systemic (rheumatologic disease or malignancies). Classification is complex and neurologic effects are protean. Well known is Temporal arteritis or giant cell arteritis.
Are demyelinating diseases ( white matter consisting of myelin, neural fibers and tracts). The course is relapsing, remitting, chronic and progressive. Etiopathogenesis remains an enigma, appear to be immune related.
Motor neuron disease, chronic, progressive, degenerative disease of unknown origin. Progressive loss of motor neurons in spinal cord, brainstem and cortex. Clinically LMN (atrophic) and UMN (spastic) involvement.
Syringomyelia :
A disease of spinal cord and brainstem of unknown cause associated with gliosis and cavitation of spinal cord and brainstem. Clinically muscular waisting and weakness, loss of pain and temperature sense but preservation of touch and pressure (dissociate anesth) Due to pain-temp. fibers crossing in spinal cord, dorsal column fibers crossing in M.Obl.
Headache disorders :
Extremely common, classification of International Headache Society. Some familiair forms are : migraine, cluster headache, tension headache, chronic paroxysmal hemicrania. Treatment are psychologic, physical and pharmacologic.
Migraine :
Unilateral, pulsating, moderate to severe headache, often increasing with physical activity and reduced by sleep. Often accompanied by nausea or vomiting, photophobia and phonophobia with or without auras.
Cluster headache :
Mostly in men, any age. Periodicity is the main feature. Each period lasts 2-3 months and recurs every year or two. Mostly nocturnal and characterized by a circadian regularity. Often precipitated by alcohol.
Tension-type headaches :
Episodic or chronic. Diffuse, bilateral, pressing or tightening in quality, mild to moderate in severity. Pain involves posterior aspects of head and neck. Photophobia, phonophobia, nausea may occur.
Nociceptive pain =
Somato-sensory system divided into : spinothalamic tract system (pain, temperature and viscero reception) and dorsal column lemniscal system (mechanoreception proprioception). Afferent impulses via 1)lateral thalamic tract to parietal cortex 2)medial thalamic tract to frontal lobe. Information coded by nociceptors via Adelta myelinated & C-unmyelinated fibers.
Spino thalamic tract-via Thalamic relay splits into Somato-sensory cortex Frontal cortex
Neuropathic pain =
Lesions of PNS/CNS pathways for pain can produce : 1)loss or impairment of pain sense 2)neuropathic pain like in H.zoster, Diabetes M. allodynia. This increased spontaneous activity is due to an increased conc. of sodium channels and N-epinephrine.
Axons of afferent nociceptors to cornu post.-spinal cord go to:1)Parietal lobe 2)Spinal neurons + input from viscera, deep muscles, skin spinal segments. This convergence often mislocalize the place by the same spinal segment.
Sensitization by:
Repeated, intense, prolonged stimuli can damage the threshold of nociceptors. Inflammatory mediators like Substance P, Bradykinin, Prostaglandin, Leukotrienes, low pH - can cause tenderness - hyperalgesia.
Sensory (p.major) skin face + anterior of head. Motor (p.minor) masseter + pterygoid muscles. Clinically: no objective sensory loss on examination (4.5 per 100.000 persons) sudden excruciating bouts for wks months with spontaneous remission. Cause: compression by a bloodvessel, demyelination, brainstem lesions, neoplasm Therapy: carbamazepine, phenytoin, surgical.
Location in Pons, close to N.VI via internal auditory meatus inner ear through for. Stylomastoid, gl. Parotis to facial muscles. Clinically: taste anterior 2/3 of tongue, hyperacusis (N.stapedius), deafness, tinnitus. Examination: MRI, EMG. Treatment: eye closing (tape,ointment), massage, prednison. Cause: vascular, infection, trauma, etc.
Myasthenia gravis
A disease affecting the neuromuscular junction (neurotransmitter-NT-Acethylcholin disorder). Characterized by fluctuating weakness, consisting of 2 forms, congenital and acquired.The latter is more common as the ocular and generalized form. An autoimmune disorder.
Myopathy - classification
Inflammatory (dermato-polymyositis) Endocrine, Metabolic, Toxic, Congenital, Muscular dystrophies (Duchenne type) Myotonic disorders (Myotonia) Periodic paralysis
Poly-& Dermato-myositis
Are inflammatory, sporadic myopathies Clinically: symmetric, painless, progressive with dominant proximal weakness of limbs, dysphagia or respiratory muscle weakness may occur, commonly with DM. Spontaneous exacerbations and remissions. 50% of Pts respond to corticosteroids.
Muscular dystrophy
2 forms, both are sex-linked, Duchenne type: acute, progressive,malignant. The benign Becker type is rarely progressive. Main features: family history, infantile & juvenile spinal muscular atrophy with slowly progressive proximal weakness to rapidly fatal disease.
Weakness, fatigue, pelvic & shoulder girdle muscle atrophy, next to a Focal facio-scapulo-humeral type. Confirmatory tests: history, laboratory biochemical aspects, muscle biopsies and electromyographic (EMG) exam. Etiology: genetic, metabolic, toxic, traumatic, inflammatory factors.
Neurofibromatosis
Also called von Recklinghausens disease is a neuroectodermal disorder due to genetic abnormalities with Skin caf au lait spots > 6 Neurofibromas of peripheral nerves Optic nerve or chiasmatic glioma Neurocutaneous syndromes produces defects in multiple organ systems.