Neuromuscular Diseases - Cohen

Question 1

fluctuating dementia

Answer 1

lewy body

Question 2

fatiguing weakness

Answer 2

myasthenia gravis

Question 3

immunology of myasthenia gravis

Answer 3

tumor of thymus - or very active thymus gland

Question 4

path of MG

Answer 4

autoAbs - to ACh receptors

-blocked and destroyed

Question 5

myasthenia gravis

Answer 5

age 20-30yo
first - around eyes
-these muscles have few ACh receptors to begin with

also - fatiguing weakness of speech and swallowing

later on - weak limbs

Question 6

epidemiology of MG

Answer 6

younger patients - women - pregnancy or childbirth - 20-30yo

older patients - men - 50-70yo

MC if pt has other autoimmune disorder

Question 7

older MG patients

Answer 7

men - more thymomas

Question 8

course of MG

Answer 8

slow decline

some myasthenic crisis
-severe impairment swallowing - secretions, resp impairment, aspiration pneumonia - intubation and mechanical ventilation

Question 9

diagnosis of MG

Answer 9

B/L ptosis - worse in one eye -extraocular palsies
-weak voice, swallowing, facial expression

strong in morning - then weaker throughout course of day

no change reflexes and no sensory changes or cerebellar involvement

Question 10

circulating Abs

Answer 10

not in all MG patients

to ACh receptors - of skeletal muscles

Question 11

nerve conduction studies

Answer 11

decremental response

-declining muscle response

Question 12

thymoma

Answer 12

take it out - can cure MG

CT of MRI of chest

Question 13

tensilon

Answer 13

short acting cholinesterase inhibitor

allows ACh to last longer at NMJ
-briefly improve MG patients

Question 14

edrophonium

Answer 14

tensilon

AChE inhibitor

short acting - 5-10min t-1/2

Question 15

tx of MG

Answer 15

oral cholinesterase inhibitor
-pyridostigmine (long lasting)

can also give corticosteroids

• initially get worse
• look out - its immunosuppressants

Question 16

adverse of pyridostigmine

Answer 16

intestinal cramp, diarrhea, nausea, bradycardia, sweating

Question 17

IVIG

Answer 17

IV IgG - tx of severe of MG

Question 18

ALS

Answer 18

UMN and LMN disease

no involvement of extraocular muscles

also - motor CNs

atrophy of muscles

Question 19

extraocular eye muscles

Answer 19

not affected by ALS

Question 20

dysarthria

Answer 20

motor speech disorder

feature of ALS

can’t move tongue very well - difficulty to speak

Question 21

path of ALS

Answer 21

cell death UMN and LMN
-from denervation

-weakness, fasciculations, atrophy, increased DTRs

UMN - motor frontal cortex, corticospinal tracts

LMN - anterior horn spinal cord to skeletal muscles

Question 22

bulbar

Answer 22

brainstem

Question 23

variation of ALS

Answer 23

bulbar only
UMN only
LMN only
UMN and LMN - MC form ALS

Question 24

ALS prognosis

Answer 24

fatal all cases - w/in 5 years - especially if bulbar symptoms present

Question 25

hand in ALS

Answer 25

lateral side - abductor pollicis brevis - more impaired

Question 26

ALS diagnosis

Answer 26

criteria

• both UMN and LMN fxn impairment
• at least 2 levels
• BS, cervical, thoracic, lumbo-sacral

confirmed by evidence of UMN and LMN at 3 levels

increased DTR, spasticity, babknski - UMN

LMN - weak, atrophy, fasciculations

Question 27

pseudobulbar affect

Answer 27

labile moods - crying then laughing

Question 28

SOD1

Answer 28

mutation in ALS

Question 29

diagnosis of ALS

Answer 29

EMG - denervation of at least 3 limbs

MRI of brain

blood test - SOD1, TFT, lyme, B12, ANA, PTH, west nile, heavy metals, HIV, HTLV-1

Question 30

tx of ALS

Answer 30

glutamate antagonist - riluzole

prolongs life - time to resp failure

damages liver

Question 31

lou gehrigs disease

Answer 31

ALS