Neuromuscular Junction Disorders

Question 2

Define a neuromuscular junction disorder.

Answer 2

Question 3

What is the gender distribution and peak age of onset of neuromuscular junction disorders?

Answer 3

Question 4

Explain the pathophysiology of myasthenia gravis. What is the main RF ?

Answer 4

Question 5

Explain the pathophysiology of LEMS. What is the main RF ?

Answer 5

Question 6

What is the normal physiology of nerve transmission at the NMJ?

Answer 6

Question 7

List the common symptoms of NMJ disorders and the questions you would like to ask in a history.

Answer 7

Question 8

Explain the distribution of muscle weakness in a patient with a NMJ disorder.

Answer 8

Question 9

You are asked to perform a focused exam on a patient with a neuromuscular junction disorder. What would you expect on general inspection?

Answer 9

Question 10

What cranial nerves are mostly affected by neuromuscular junction disorders?

Answer 10

Question 11

You are asked to perform a focused exam on a patient with a neuromuscular junction disorder. What would you expect on cranial nerve exam?

Answer 11

Question 12

You are asked to perform a focused exam on a patient with a neuromuscular junction disorder. What would you expect on motor and sensory exam?

Answer 12

Question 13

You are asked to perform a focused exam on a patient with a neuromuscular junction disorder. What special tests would you offer and what would expect to find?

Answer 13

Question 14

List some ddx for NMJ disorders.

Answer 14

Question 15

What are all the exam finding of a NMJ disorder?

Answer 15

Question 16

What cancers are associated with NMJ disorders?

Answer 16

Question 17

How would you grade MG?

Answer 17

Question 18

What is the edrophonium tensilon test and what is it used for? What are some SEs of the test?

Answer 18

Question 19

What are the diagnostic investigations for MG and LEMS? What are the expected findings?

Answer 19

Question 20

How would you determine if to start a patient with a dysphagia-causing disease on mech ventilation or intubation?

Answer 20

Question 21

What are the supportive investigations for MG and LEMS? What are the expected findings?

Answer 21

Question 22

Outline the acute management of NMJ disorders and specifically, myasthenic crisis.

Answer 22

Question 23

Outline the long term management of NMJ disorders.

Answer 23

Question 24

Outline the full management of a NMJ disorder.

Answer 24

Question 25

NMJ disorders typically peaks within several years then stabilizes, with some patients having remissions. What are the complications of NMJ disorders?

Answer 25