NMJ Disorders

Question 1

what causes NMJ disorders in general

Answer 1

bacteria responsible for botulism

Question 2

discuss MG

Answer 2

autoimmune disorder that attacks Ach receptors; post synaptic

strength worse with cont contraction and improves c rest

thymoma

female

smooth and cardiac mms are spared

Question 3

clinical features of MG

Answer 3

ptosis - CN 3

jaw muscle weakness - mouth open

facial muscle weakness - buccinator

dysarthria, dysphagia

nasal regurgitation

trident tongue

neck muscle weakness - proximal more

Question 4

what is LEMS

Answer 4

autoimmune attack on Ca channels = no Ach go out; pre synaptic

males

assoc c bronchogenic carcinoma

Question 5

clinical features pf LEMS

Answer 5

weakness of proximal muscles sparing EOMS

hyporeflexia, dry mouth and normal sensory

2nd wind - improves with cont conctraction

increment response to 20 Hz RRNS

Question 6

discuss GBS

Answer 6

autoimmune attack p GI or respiratory illness

acute ascending symmetrical polyneuropathy

Question 7

clinical presentations of GBS

Answer 7

stock and glove paresthesia

symm LE->UE; proximal muna

respi prob and bulbar sx if secere

progresses: days-wks

can recover in 6mo to 2 yrs

Question 8

phases of GBS

Answer 8

tingling of hands and feet

diff in STS

areflexia, weakness and distal sensory loss

respi prob

mechanical vent

full recovery

Question 9

discuss acute inflammatory demyelinating polyneuropathy

Answer 9

AIDP

pure motor and common in pedia

Question 10

discuss acute motor-sensory axonal neuropathy

Answer 10

AMSAN

sesnsory and motor

marked msucle wasting = poor recovery

Question 11

discuss miller-fisher syndrome

Answer 11

triad: ataxia, areflexia, opthalmoplegia

1-3 mo recovery