Neuromuscular

Question 1

Motor Neuron disease could have normal amplitude MUAP

Answer 1

on NCV

Question 2

Familial ALS gene (FLAS) . AD

Answer 2

SOD1 and C9orf72

Question 3

Spinal Muscular Atrophy (SMA). AR
Type I: <6 month age
TypeII: 6-18 month age
Type III: >18 month age

Answer 3

SMN Gene Chromosome 5

Question 4

GMA1 Ab

Answer 4

a/w multifocal motor neuropathy.
Dramatically elevated in MMN (Pathognomonic)
ddx of ALS

Question 5

Hexosaminidase A deficiency

Answer 5

ALS mimc but has Psych features and cerebellar features

Question 6

Kennedy’s dz (X-linked SBMA)

Answer 6

X-Linked
Gynecomastia and Facial Fasic otherwise like ALS
normal life expectency

Question 7

West Nile

Answer 7

Meningoencephalitis in late summer/early fall
Acute Flaccid paralysis
Long duration: only 1/3 recovered at 1 year

Question 8

Vit B6 (Pyridoxin) TOXICITY
Affects Dorsal root ganglion (Sensory Neuron dz)
AKA sensory Neuronopathy

Answer 8

All modality sensory loss
Ataxia (early Ataxia—–very important finding)
Areflexia
Normal motor

Question 9

Sensory Neuron Disease

Answer 9

Pure sensory even on EMG/NCV
Autonomic dysfxn is common
Contrary to Sensory Neuropathy:
Early Ataxia
non length dependent sensory loss

Question 10

Sensory Neuronopathy causes

Answer 10

Paraneoplastic:** may preceed the cancer by months
Anti Hu Ab
Anti CRMP5 Ab——-Small cell lung ca

Non Malignant:
SJOGRAN------Most common******
MGUS associated
Cisplatin-------chemotherapy, ********
B6 Toxicity********

Question 11

Vasculitis Neuropathies

Answer 11

Enlarged Hypoechoic nerves on US—-Pathognomonic
Usually asymmetric neuropathy (Mononeuritis Multiplex) plus other systemic symptoms
eg: Kidney, GI, Skin, fatigue, etc.

Question 12

Foot INVERSION

Answer 12

Tibial Nerve (L4-L5)(Tibialis Posterior muscle)

Question 13

Foot Eversion and Dorsiflexion

Answer 13

Peroneal Nerve

Question 14

Myokymic discharges on EMG needle exam

Answer 14

Radiation induced Plexopathy

Question 15

Patient had lung Cancer, Had XRT, now has plexopathy

Cancer recurrence or XRT?

Answer 15

If there is Myokymia on EMG it is XRT

Question 16

Erb’s Palsey: (Waiter’s Tip)
Most common Plexopathy in new born
Common in MVA eg: Motor cycle accident head goes one way and arm the other way.

Answer 16

Upper Trunk; C5, C6
Weakness: 
Shoulder Abd (Deltoid/Axillary N.)
Elbow flexion(Bicep/Musculocutanous N.)
Arm Supination
Numbness:
LATERAL forearm

Question 17

Klumpke Palsey:

Usually occur when arm and shoulder are pulled up

Answer 17

Lower Trunk; C8, T1
Weakness:
Severe Hand weakness
Numbness:
MEDIAL forearm

Question 18

Femoral Nerve

Same for Obturator nerve

Answer 18

Lumbar Plexus; L2, L3, L4

Question 19

Sciatic Nerve

Answer 19

Lumbosacral Plexus; L4, L5, S1

Question 20

Alpha Motor Neurons

Answer 20

Stimulate the muscle with Acetylcholine
Nerve Itself is:
Stimulated by Glutamate
Inhibited by GABA and Glycine

Question 21

Young patient with:
Hand weakness and
ALS symptoms but NO UMN signs

Answer 21

Monomelic Amyotrophy
Normal life
Tx: PT

Question 22

SMA type I

Answer 22

Onset age <6 month
Never sits
No Tremor
life expectancy 1-2 years; rarely 10 years

Question 23

SMA type II

Answer 23

Onset age 6-18 months
Sits but NO walking
postural hand tremor
Life expectancy up to 25 years

Question 24

SMA type III

Answer 24

Onset age >18 months
Sits, Stands, Walks
Postural Hand Tremor
Life expectancy 50’s or normal