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Flashcards in NMJ Disorders Deck (28):
1

MC NMJ disorder

Myasthenia Gravis

2

Three types of myasthenia

-generalized
-ocular
-bulbar

3

Age/Sex MC seen in myasthenia

females: teens-30s
males: 50-70
(Bimodal distribution)

4

Three pathophys mechanisms that lead to myasthenia

1) Direct blocking of the nicotinic AchR site
2) Accelerated internalization of the AchR by crosslinking of IgG
3)Complement mediated lysis of the muscle end plate

5

How commonly is thymus involved in myasthenia?

50% have thymic hyperplasia
10-15% have thymoma

(Thymocytes produce the AchR Ab)

6

Classic symptom description for myasthenia:

-fatigable weakness
-diurnal variation (worse at night)

**Normal sensation, reflexes

7

Most common Ab assc with MG

AchR BINDING Ab
(1st screening test for myasthenia, may not ALWAYS be present especially in ocular MG)

8

Myasthenia Ab that is assc with thymoma

Anti-striated muscle
(seen in 75-80% of those with thymoma, also sometimes those without)

9

In addition to screening for Ab's, what other tests are diagnostic for MG:

-repetitive nerve stimulation
-edrophonium/tensilon test

10

How sensitive/specific is tensilon test for MG?

"71-95%" sensitive
not very specific
not reliable in anti-MuSK MG

11

Side effects assc with the tensilon test

bradycardia, hypotension (treat with atropine)

12

What must be done to ensure there is no bias in tensilon testing?

-patient+ docs blind, only nurse may know in which order patient receives placebo vs tensilon

13

MC treatment for symptom reduction in myasthenia

-pyridostigmine (mestinon)
-AchEi

14

Effect of mestinon on end plate potential

increases size and length of end plate potential

15

ADRs assc with mestinon

liquid out of all the places.
(diarrhea, runny nose, sweating, vomiting etc)

16

Short term therapies to suppress immune system in MG?

-plasma exchange
-IVIG

17

Long term therapies to suppress immune system in MG?

-thymectomy
-corticosteroids
-azathioprine/ mycophenolate

18

Why must plasma exchange be done every OTHER day for 3-6 days as opposed to EVERY day?

-It depletes clotting factors

19

When are Plasma exchange and IVIG used for MG

-exacerbations, resistant cases

20

What patients should have prophylactic thymectomy in MG?

-Most patients under 50

21

Lambert Eaton:
-MC sex
-paraneoplastic of what cx?
-how commonly is LEMS paraneoplastic?

-males=females
-small cell, half of LEMS patients have small cell, 3% of small cell patients have LEMS

**IF YOU DIAGNOSE LEMS YOU HAVE TO SCREEN FOR SMALL CELL!!!!!!!

22

Non small cell LEMS is most commonly assc with?

-younger females with other autoimmune disease

23

What antibody is responsible for lambert eaton?

-IgG antibody against voltage gated calcium channel

(Ca is responsible for the release of Ach at NMJ)

24

How does LEMS effect the motor end plate potential?

-decreased --> failed transmission --> low CMAP

25

Typical initial presentation of LEMS

-proximal leg/arm weakness that improves w/ exercise
-MILD CN sx
-dry eyes/mouth, metallic taste
-absent reflexes (UNLIKE MG)
-normal sensation

26

Subtle signs of LEMS (2)

-weakness if given CCB for HTN
-prolonged paralysis following intubation

27

How does repetitive stimulation testing vary in LEMS vs MG?

-increased end plate potential over time in LEMS
-decreased EPP over time in MG

28

Three possible MG antibodies

-antiMUSK
-antiAchR
-anti striated muscle