Med 1 EOR - Neuro Flashcards
autosomal dominant neurodegenerative d/o. Mutation chromosome 4. Neurotoxicity + cerebral, putamen + caudate nucleus atrophy. = behavioral change –> chorea –> dementia
huntingtons syndrome
CT/MRI of huntingtons shows what?
CT/MRI = cerebral + caudate nucleus atrophy.
prognosis + mgmt of huntingtons
fatal 15-20 yrs after presentation. Chorea mgmt: antidopaminergics (typical + atypical antipyschotics. Tetrabenzine, Benzos.
idiopathic dopamine (inhibitor) depletion → failure to inhibit Ach in basal ganglia. MC onset 45-65yo.
parkinsons
4 cardinal S+S of parkinsonism?
resting tremor (pill rolling)- better with intention
cogwheel rigidity
fixed facial expression
bradykinesia: shuffling gait
MRI: Cytoplasmic inclusions (lewy bodies), loss of pigment cells seen in substantia nigra.
parkinsons
3 txt options for parkinsons: what is the go-to? best for avoiding ADRs of the first line drug? for tremor predoninance?
- *levodopa/carbidopa (sinemet): most effective - “wearing off of bradykinesia” w/ longterm use
- *Dopamine agonists: bromocriptine, pramipexole, ropinirole. Used in young pts to delay use of levodopa (ADRs)
- Anticholinergics: trihexyphenidyl, benztropine - for <70yo w. Tremor predominance.
MC kids from rheumatic fever (group A strep) = antibodies against N-actyel-beta-glucosamine - involve basal ganglia + cortical structures.
syndeham chorea (post-infectious chorea)
txt options for syndenham chorea
chronic ABX for Group A strep - PCN G. severe symptoms =D2 blocker, carbamazepine, or valproic acid
pathologically enlarged ventricular size with normal opening pressures on lumbar puncture
normal pressure hydrocephalus
causes of normal pressure hydrocephalus (primary and secondary)
Causes: idiopathic (maybe congenital that shows symptoms later in life, cerebrovasc dz, decr CSF abs etc.
Secondary: MC intraventricular or subarachnoid hemorrhage
triad: 1. Dementia 2. gait disturbance 3. Urinary incontinence. Dont need all 3 but gait must be primary - “glued foot” - small steps + wide base.
normal pressure hydrocephalus
Dx of normal pressure hydrocephalus
Dx of exclusion then high-volume LP (30-50mL) + document gait before and after procedure.
MRI/CT = enlarged ventricles
txt for normal pressure hydrocephalus
reversible w/ VP shunt (lateral ventricle to abdomen)
necrosis of BOTH upper and lower motor neurons → progressive motor degeneration.
S+S: muscle weakness, loss of ability to initiate + control movements.
Upper: spastic, stiff, hyperreflexive
Lower: progressive bilat fasiculations, muscle atrophy, hyporeflexia, muscle weakness.
amyotrophic lateral sclerosis (ALS) - lou gherigs disease
prognosis + txt options for ALS?
riluzole (glutamate blocker): reduces progression up to 6mo. Fatal 3-5yrs after onset.
spasticity/hyperreflexive, varying degrees of motor deficits. Often associated w/ intellectual/learning disabilities.
cerebral palsy (brain injury during perinatal or prenatal period)
autoimmune peripheral nerve d/o (against Ach - nicotinic- postsynaptic receptor). MC young women. HLA-DR3. 75% have thymic abnormality (hyperplasia or thymoma).
myasthenia gravis
S+S of myasthenia gravis
progressive weakness w/ repeated muscle use + recovery after rest
Ocular weakness: diplopia, ptosis
generalized weakness, bulbar, respiratory!
MG crisis = flaccid paralysis + resp failure
Dx of MG ?
+ Ach receptor Antibodies
what is the Edrophonium (tensilon) test?
rapid response to short-acting IV edrophonium in limb MG
how do you distinguish cholinergic crisis from myasthenic crisis?
if flaccid paralysis improves w/ tensilon test = myasthenic crisis, worse = cholinergic crisis.
what is lambert-eaton syndrome and how do you distinguish it from MG?
Lambert eaton: MC assoc w/ small cell lung CA - antibodies against presynaptic voltage gated Ca+ channels prevents Ach release.
Unlike MG - weakness IMPROVES w/ repeated use.
what is guillan-barre? (what causes it and what is the patho?)
ACQUIRED inflam demyelinating polyradiculopathy of peripheral nerves. = slow nerve impulse = symmetric paresthesias and weakness. MC campylobacter jejuni, also respiratory of GI infections.