NMJ Disorders

Question 1

What percent of MG is ocular? Generalized?

Answer 1

10-20% Ocular

80-90% Generalized

Question 2

What are the symptoms of generalized MG?

Answer 2

ptosis, diplopia, dysarthria (difficulty speaking, , dysphagia; respiratory, facial, neck and limb weakness

preserved sensation

Question 3

How are reflexes affected by MG?

Answer 3

They are preserved

Question 4

Which is the most specific test for MG?

Answer 4

Elevated serum antibody titer to AChR

Question 5

What percentage of people with MG have elevated serum antibody levels against AChR?

Answer 5

80-90% of people with generalized MG are +

50% of people with ocular MG are +

Question 6

Name three treatment strategies for MG

Answer 6

Anti Acetylcholinesterase drugs
Immunosuppressant drugs
Thymectomy

Question 7

Name two quick, yet transient fixes for MG

Answer 7

plasmapheresis and IVIG

Question 8

What is Lambert Eaton Myasthenic Syndrome (LEMS)?

Answer 8

Antibodies against the presynaptic voltage gated Ca2+ channels

Question 9

Where is weakness seen in LEMS?

Answer 9

proximal limbs and trunk

Question 10

How are reflexes affected by LEMS

Answer 10

hyporeflexia

Question 11

LEMS is commonly associated with what other disease process?

Answer 11

Cancer- most often small cell lung cancer

Question 12

What are the treatment strategies for LEMS?

Answer 12

• Find and treat other cancer and symptoms will resolve
• Drugs which enhance ACh release (guanidine, 3,4-diaminopyradine)
• Immunotherapy, although it is much less effective than MG