Neuromuscular

Question 1

Anterior interosseus nerve

Answer 1

All C7, C8. Comes off AFTER pronator teres. Innervates pronator quadratus - Flexor Dig Profundus (Digits 2+3) - Flexor Pollicis Longus

Question 2

Posterior interosseus nerve

Answer 2

All C7, C8. ABductor Pollicis Longus (APL) (fake APB)
Ext Carpi Ulnaris
Ext Digitorum
Ext Poll Brevis
Ext Poll Longus

Question 3

Serratus anterior

Answer 3

inn. long thoracic nerve. C5-7.

Comes off C5-6 before the upper trunk.

Question 4

Inversion of foot

Answer 4

Tibialis posterior. L4-S3. Tibial nerve.

Question 5

Eversion of foot

Answer 5

Peroneus longus. L5-S1. Superficial peroneal nerve.

Question 6

Extension of foot

Answer 6

Tibialis anterior. L4, L5. Deep peroneal nerve.

Question 7

Extension of great toe

Answer 7

EHL. L5, S1. Deep peroneal nerve.

Question 8

Hypokalemic periodic paralysis

Answer 8

AD. 
Calcium channels (DHP channels)
paralysis after sleep + REST POST-exercise
POST CARB PARALYSIS
Can also occur after sleep

Question 9

Hyperkalemic periodic paralysis

Answer 9

AD.
Sodium channels.
Paralysis with INCREASE K+, COLD, EXERCISE.
Rx = glucose or diamox/thiazide

Question 10

Myotonia congenita

Answer 10

Thomsen disease.
Autosomal dominant
Chloride channels
Myotonia w INACTIVITY - improve with exercise

Question 11

Paramyotonia congenita

Answer 11

AD,
Sodium channels
Myotonia with COLD, EXERCISE, LOW K+

Question 12

Central core disease

Answer 12

AR
ryanodine Ca channel mutation
\+/- Mal hyperthermia
lucent core (NADH diaphorase stain)
non-progressive weakness

Question 13

Centronuclear myopathy

Answer 13

MYF6 (AD), BIN1 (AR), myotubularin(XR)
“central myonuclei (H&E stain)
stellate sarcoplasmic reticulum (NADH diaphorase stain)”

Question 14

Emery Dreifuss

Answer 14

Emerin (XR), lamin A/C (AD+AR)
Cardiomyopathy
Biceps+ triceps weakness then spreading to legs

Question 15

Facioscapulohumeral dystrophy (FSHD)

Answer 15

AD; 
D4Z4 on chromosome 4q; encodes polyA binding P
Facial weakness + drooping shoulders
SN Hearing LOSS
MR, seizures

Question 16

Fukuyama muscular dystrophy

Answer 16

AR
Fukutin 
Contractures
Polymicrogyria + Cobblestone lissencephaly
Mental retardation
SEIZURES

Question 17

Hirayama

Answer 17

Aka monomelic amyotrophy
“Japan/Indian/Hawaiian origin
single-limb or UE-only ALS type picture”

Question 18

Nemaline rod myopathy

Answer 18

Mutations in:
alpha-actin (MC) + troponin
Path = EM with rods parallel to myofibers

Question 19

Polymyositis

Answer 19

endomysial + perimysial +perivascular inflammation
association with MALIGNANCY (not as much as DM)
responds to steroids

Question 20

Dermatomyositis

Answer 20

Characteristic rash, assoc w malignancy
path = peri-fascicular atrophy + periVASCULAR infl.
Anti Jo1 associated with ILD (do not not use MTX)

Question 21

pediatric myasthenic syndromes

Answer 21

Neonatal transitory = passive Xfer of AB’s
Congenital MG = AR mutations in ACH (R) epsilon subunit
familial infantile = chromosome 17 polymorphism

Question 22

HNPP

Answer 22

deletion in PMP-22 gene (also impl. in CMT1A)

Question 23

Lewis Sumner

Answer 23

aka MADSAM - basically asymmetric CIDP