Neuro - myaesthenia gravis

Question 1

what makes this condition better and worse?

Answer 1

better with rest and worse with activity

Question 2

typical patients

Answer 2

women under age of 40

men over age of 60

Question 3

what condition is there a strong link with?

Answer 3

thymoma

Question 4

what neurotransmitter is involved?

Answer 4

acetylcholine

Question 5

what antibodies are produced?

Answer 5

Ach receptor

Question 6

what system do these antibodies activate?

Answer 6

complement

Question 7

what other antibodies can be produced?

Answer 7

MuSK and LRP4

Question 8

what % of people have Ach receptor antibodies?

Answer 8

85

Question 9

proximal or distal muscles affected worst?

Answer 9

proximal

Question 10

what muscle affected worse? proximal muscles and..

Answer 10

head and neck muscles

Question 11

eye symptoms

Answer 11

dipolpia and ptosis

Question 12

other symptoms

Answer 12

fatigue in jaw when chewing
weak facial movements
slurred speech
progressive weakness with repetitive movements

Question 13

examination

Answer 13

fatiguability - repeated blinking, upward gazing and arm abduction
check thymectomy scar
FVC

Question 14

diagnosis

Answer 14

test for antibodies
CT or MRI thymus
edrophonium test

Question 15

what is an edrophonium test?

Answer 15

IV dose of edrophonium chloride/neostigmine
block enzymes that break down Ach
briefly and temporarily relieves weakness

Question 16

treatments

Answer 16

acetylcholinesterase inhibitors
immunosuppression - steroids, azathioprine
thymectomy
monoclonal antibodies

Question 17

myaesthenia crisis

Answer 17

acute worsening of symptoms
resp infection can cause
need NIV and BiPAP

Question 18

medical treatment myaesthenic crisis

Answer 18

IVIg

plasma exchange