Neuromuscular Disorders

Question 1

How is the diagnosis of MG made?

Answer 1

Positive antibodies (80% have them)

Tensilon Test

Repeated stimulation

Question 2

What type of gait might you observe is a patient with duchenne’s muscle weakness?

Answer 2

Trendellenberg’s

Question 3

What is the classical constellation of signs typical of myopathies in terms fasciculations, muscle wasting, weakness, reflexes, and sensation?

Answer 3

Fasciculations: Absent

Muscle wasting: No

Weakness: Proximal > Distal

Reflexes: Normal

Sensation: Normal

Question 4

What are some treatment options for MG?

Answer 4

Acetylcholinesterase antagonists

Plasma exchange

IV Ig

Immunosuppresion - Steroids or azathioprine

Thymectomy

Question 5

In which type of NM disease are fasciculation common?

Answer 5

Anterior horn cell disease

(Motor neuron)

Question 6

Which diseases would you attribute a ptosis to?

Answer 6

MG

Ocular nerve palsy

Thyroid eye disease

Question 7

Which muscles are spared in motor neuron disease?

Answer 7

Ocular

Question 8

In terms of location of symptoms, how do myopathy and neuropathy differ?

Answer 8

Myopathy - proximal

Neuropathy - distal

Question 9

What is the classical constellation of signs typical of neuropathy in terms fasciculations, muscle wasting, weakness, reflexes, and sensation?

Answer 9

Fasciculations: Infrequent

Muscle wasting: Late

Weakness: Distal>Proximal Localised

Reflexes: Reduced or absent early

Sensation: Glove and stocking

Question 10

What is the tensilon test?

Answer 10

IV injection of short acting acetylcholinesterase antagonist

Question 11

How does motor neuron disease progress?

Answer 11

Generally local muscle wasting followed by more diffuse muscular involvement

Question 12

What are the most common causes of neuropathies?

Answer 12

DM

Alcohol/Drugs

Paraneoplastic

Hypothyroidism

Question 13

What is the classical constellation of signs typical of motor neuron disease in terms fasciculations, muscle wasting, weakness, reflexes, and sensation?

Answer 13

Fasciculations: Present

Muscle wasting: Early

Weakness: Diffuse, mixed picture

Reflexes: Increases or normal

Sensation: Normal

Question 14

What is the best test for myopathies?

Answer 14

CK levels

Question 15

What is the most common motor neuron disease?

Answer 15

Amyotrophic lateral sclerosis

Question 16

What is the classical constellation of signs typical of neuromuscular junction pathologies in terms fasciculations, muscle wasting, weakness, reflexes, and sensation?

Answer 16

Fasciculations: Absent

Muscle wasting: No

Weakness: Proximal>Distal, ptosis and eye muscles, fatiguable

Reflexes: Normal

Sensation: Normal

Question 17

When is CK elevated?

Answer 17

Muscular damage

Question 18

What is the key finding on physical examination of a MG patient?

Answer 18

Fatiguable muscle weakness

Question 19

What symptoms do patients with neuromuscular disease present with?

Answer 19

Weakness

Gait clumsiness

Sensation loss

Diplopia

Fasciculations

Cramps

Question 20

What is a strabismus?

Answer 20

Abnormal position of the eye eg lazy eye

Question 21

What type of dementia is associated with motor neuron disease?

Answer 21

Fronto-temporal

Question 22

What are the 4 types of neuromuscular disorders? Briefly describe them

Answer 22

Motor neuron disease - pathology of uppper or lower motor neurons

Myopathy - Pathology of muscle

Neuropathy - pathology of peripheral nerves

Disorders or neuromuscular junction

Question 23

What are the pre and post synaptic disorders of the neuromuscular junction?

Answer 23

Pre - MG

MuSK Myasthenia

Post - Lambert Eaton myasthenic syndrome

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