Extras: PNS & Skeletal Muscle Flashcards
1
Q
what NMJ condition tends to have thymic hyperplasia too?
A
myasthenia gravis
2
Q
treatment for MG and LEMS
A
AchE inhibitors
3
Q
two Ab types in seronegative MG
A
MUSK, LRP4
4
Q
key presenting features of LEMS
A
dry mouth
5
Q
botulinum MOA vs curare
A
- botulinum: blocks Ach release
2. curare: blocks AchR
6
Q
histo of dermatomyositis
A
perifascicular atrophy
7
Q
Abs in dermatomyositis
A
anti-Jo1, anti-Mi2
8
Q
Abs in Immune-Mediated Necrotizing Myopathy
A
HMG-CoA reductase As
9
Q
tx of inclusion body myositis
A
respond poorly to steroids
10
Q
presentation of inclusion body myositis
A
weakness of hands + quads
11
Q
DMD gene
A
Xp21
12
Q
protein mutated in Limb-Girdle MD
A
sarcoglycan
13
Q
Emery-Dreifuss triad
A
- humeroperoneal weakness
- cardiomyopathy
- contractures
14
Q
SMA gene is on what chromosome
A
Chr 5
15
Q
Barth syndrome
A
mitochondrial dz -> cardioskeletal myopathy
