Neurol - MedEd - Weakness

Question 1

Two types of disease categories for weakness

Answer 1

1) Demyelinating - MS, GB

2) NMJ diseases - endplate diseases - MG, EL

Question 2

Multiple sclerosis - pathophys

Answer 2

Autoimmune disease
Female
Demyelinating

Question 3

MS - S/S

Answer 3

20s-40s, often female
Anywhere in brain and spinal cord
Any neural symptom separated by time and space - so different episodes of symptom/asymptomatic and different lesions in the CNS

Question 4

One presentation of MS in eye is…

Answer 4

Optic neuritis

-Painful eye and blurry

Question 5

Diagnosis of MS

Answer 5

MRI
Periventricular white matter, plaques (shiny white)

• Can also see lumbar puncture - oligoclonal IgG
• Evoked potentials - demyelination
• But MRI is still best!

Question 6

Treatment of MS

Answer 6

MS flare - steroids, but need to rule out infection

Chronic MS meds - interferon, glatiraner, fingolimod

Question 7

Complications of MS

Answer 7

Urinary retention - bethanecol
Urinary incontinence - amitryptiline
Spasms - baclofen
Neuropathic pain - gabapentin

Question 8

Guillane bare - pathophys

Answer 8

Demyelinating
Autoimmune
Ascending paralysis
Starts distally and reaches trunk, eventually to diaphragm - stops breathing and die

Question 9

Guillane bare - s/s

Answer 9

Watery diarrhea - campylobacter
Flu shot
Then develop ascending paralysis 
Hyperreflexia
Paresthesias

Question 10

Diagnosis of GB

Answer 10

Lumbar puncture - lots of protein, immunoglobulins that cause immune reaction, few cells

• Could do MRI, not helpful for GB
• EMG will see diagnosis of demyelination

Question 11

Treatment of GB

Answer 11

Intubation! esp if have trouble breathing

• IVIG (binds up immunoglobulin), plasmapharesis (removes immunoglobulins)
• Never give steroids (worsen outcome)

Question 12

Myasthenia gravis - pathophys

Answer 12

Autoimmune disease against AChR
Antibodies block AchR
So need more ACh to compete with antibodies

Question 13

Myasthenia gravis

Answer 13

50+ patient
Fatigability - muscles fatigued are: eyes, throat (difficulty swallowing), fine movement of distal extremities (lack of hand coordination) - worse in PM, these are usually muscles that are used the most

Question 14

Diagnosis of Myasthenia gravis

Answer 14

Need antibodies - anti-AChR antibodies
Need EMG - great amplitudes that begin to decrease and baseline, because deplete ACh
-Might get a CT scan to look for thymoma

Question 15

Treat Myasthenia gravis

Answer 15

Treat by increasing ACh - use acetylcholinesterase inhibitors
Want to decrease autoimmune component - use steroids (in resistant disease)
Myasthenia crisis - use IVIG/plasmapharesis
If there is a thymoma - do thymectomy for cure

Question 16

Eaton-Lambert - pathophys

Answer 16

Autoimmune disease
Often paraneoplastic syndrome
against presynaptic calcium channels (harder to release vesicles)

Question 17

S/S of Eaton-Lambert

Answer 17

50s or older
Will have improvement with use (not fatigability)
Muscles that are used the least will be worst - usually proximal muscles (i.e. inability to rise from chair)
Associated with SCLC

Question 18

Eaton-Lambert - diagnosis

Answer 18

Antibodies
EMG - start with little contraction, which increase with more attempts
CT - for small cell lung cancer

Question 19

Eaton-Lambert - treatment

Answer 19

Treat lung cancer - chemoradiation

Can use immunosuppresants

Question 20

ALS - pathophys

Answer 20

Sporadic mutation (superoxide dimutase)

Question 21

ALS - S/S

Answer 21

UMN and LMN symptoms - multiple lesions throughout CNS

Spares sensation changes

Question 22

Diagnosis of ALS

Answer 22

EMG

Question 23

Treatment of ALS

Answer 23

Supportive
Die because eventually all muscles fail and they cannot breathe
Riluzole - affords them 3 months to live