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21 Cards in this Set

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  • Back
Guillain-Barre Syndrome
Acute polyneuropathy. Aquired, acute onset inflammatory peripheral demylinating neuropathy with axonal sparing. Usually preceded by infection 2 weeks prior. Occurs in spinal roots and proximal nerve fibers. Leads to slow conduction velocity and decreased amplitude.
Presents with symmetric progression starting in arms and legs. Eventual lung paralysis can be fatal.
Spinal fluid protein is elevated and acellular.
Charcot-Marie Tooth Disease
Hereditary polyneuropathy resulting in demylination of sensory and motor axons.
MS
Acquired demylinating disease in CNS with an immunologic cause. Get demylinated plaques. Characterized by cycles of relapse and remission.
Weber syndrome
When a lesion involving the pyramidal tract of the cerebral crux also hits the occulomotor nerve. Get spastic hemiplegia on the contralateral side as well as a down and out eye with ptosis on the ipsilateral side.
Duchennne muscular dystrophy
Calves and deltoids enlarge but are weak, due to replacement of muscle tissue with fat.
Myotonia congenita
Kids have herculean appearance but are actually weak.
Eaton-Lambert syndrome
Disease preventing the presynaptic release of Ach. This is due to an autoimmune reaction against voltage-gate calcium channels.
Presents with proximal weakness of lower extremities and dry mouth. Weakness improves with exercise.
Gowers maneuver
Proximal weakness leading to difficulty getting up from the floor.
Cerebral palsy
Nonprogressive neonatal CNS disorder effecting motor system and mental function. Dyskinesia.
Dermatomyositis
Usually prebubecent or 40 yr old females. Associated with lung/breast cancer.
Presents with rash and proximal weakness. Will have small blood clots in nail beds.
Duchenes muscular dystrophy
Kids looked jacked but are weak because muscle is replaced with fat. Use gowers maneuver to get up b/c of hip weakness.
Tourette Syndrome
Extrapyramidal movement disorder:
-OCD
-Hyperactivity
-Sleep disorders
-movement rituals
-coprolalia (uncontrolled swearing)
-copropraxia (lewd gestures)
Can treat with dopamine blocking agents
Progressive supranuclear palsy
Neural degn of pons/midbrain, depigmentation of nigra
-lower cranial nerve hyperreflexia, weakness
-loss of voluntary by maintanence of reflex eye movements
-rigidity
-distonia
-gait problems
-dementia
Wilsons disease
In born error of copper metabolism, auto recessive. Associated with liver cirrhosis and basal ganglia degeneration. Reduced levels of ceruloplasmin which normally bind copper. Kasyser-Fleisher ring - yellow brown cornea
dystonia, wing beating tremor, sardonic grin.
treat with chelation, penicillamine, and zinc
Huntington's disease
autodom, CAG trinucleotide repeat leading to over production of huntingtin. Loss of striatal GABA.
Chorea, dementia, personality disturbance, milkmaid grip sign. Neuroimaging has butterfly appearance due to loss of caudate.
Essential tremor
Most common movement disorder. Onset occurs during emotional stress. Frequency starts high, becomes lower with increased amplitude. Beta blockers and primidone are main treatments.
Friedreich's Ataxia
hereditary progressive ataxia, autorecessive. GAA trinucleotide expansion, encodes for protein frataxin. Gross atrophy of spinal cord.
High arch feet, hammertoes, kyphoscoliosis. Gait ataxia, upper limb ataxia, wide based gait, explosive speech, romberg sign, areflexia.
Syrnigomyelia
Cavitation of the spinal cord
Brown -Sequard syndrome
Lesion involving right or left half of spinal cord. Get loss of ipsilateral touch and contral lateral pain/temp below lesion.
Acoustic Neurinoma
Schwann cell tumor from a vestibular nerve in the internal acoustic meatus. Compression of the nerves of the meatus leads to the symptoms:
vertigo - vestibular nerve
tinnitis/deafness - cochlear nerve
factial paralysis/loss of taste facial nerve and nervus intermedius
Benign familial neonatal convulsions
Auto dom, due to mutations in voltage gated k channels, begin @ 3-5 doL, normal neuro exam, good prognosis, 5-10% get epilepsy.