Neuromuscular disease

Question 1

How does botulinum toxin work?

Answer 1

Cleaves proteins involved in vesicle formation and docking

Question 2

What causes Lambert Eaton myasthenic syndrome?

Answer 2

Antibodies to presynaptic calcium channels

Question 3

What is Lambert Eaton associated with?

Answer 3

Small cell carcinoma

Question 4

What is the underlying aetiology of myasthenia gravis?

Answer 4

Impairment of neuromuscular junction transmission usually due to autoantibodies against the nicotinic acetylcholine receptor

Question 5

75% of MG patients have…

Answer 5

Thymoma or hyperplasia of the thymus

Question 6

Two peaks of incidence

Answer 6

Females- 3rd decade

Males- 6 or 7th

Question 7

Clinical features of MG (4)

Answer 7

Muscle fatigability, improves after rest:
proximal muscle weakness
extra-ocular muscle weakness
ptosis

Question 8

Bulbar symptoms (3)

Answer 8

“Myasthenic snarl”
Hypernasal speech
Dysphagia

Question 9

Symptomatic treatment of MG (1)

Answer 9

Pyridostigmine (acetylcholinesterase inhibitor)

Question 10

Potential side effects of pyridostigmine (3)

Answer 10

Increased salivation
Bradycardia
Diarrhoea

Question 11

Potential surgical treatment of MG

Answer 11

Thymectomy

Question 12

Immunomodulating therapy for MG (1)

Answer 12

Prednisolone

Question 13

Management of myasthenic crisis

Answer 13

Plasmapharesis
Intravenous immunoglobulin (works for unknown reason)

Question 14

Features of LEMS

Answer 14

Autonomic dysfunction
Hyporeflexia
Weakness