Neuromuscular Disease Flashcards

1
Q

How does curare affect cholinergic transmission and muscle contraction?

A

Competes for and blocks ACh receptor so no muscle stimulation occurs

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2
Q

Curare is only toxic when administered how?

A

Intravenous

Intramuscular

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3
Q

How does Botox affect cholinergic transmission and muscle contraction?

A

Cleaves proteins involved in ACh vesicle formation and blocks vesicle docking to prevent exocytosis

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4
Q

What happens molecularly in Lambert Eaton myasthenic syndrome?

A

Antibodies attack presynaptic Ca channels resulting in less NT release

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5
Q

Which cancer is associated with Lambert Eaton syndrome?

A

Small cell carcinoma

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6
Q

Which disease is the most common neuromuscular junction disorder?

A

Myasthenia gravis

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7
Q

Autoantibodies attack what in myasthenia gravis?

A

ACh receptors

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8
Q

When do symptoms start in myasthenia gravis?

A

When ACh receptors reduced to 30% of normal

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9
Q

A patient must have Autoantibodies to suggest myasthenia gravis. True/False?

A

False

Patient with fatigue without antibodies may still have it

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10
Q

Which organ must be screened/assessed in people with myasthenia gravis?

A

Thymus (arrange CT chest to check for thymoma)

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11
Q

Females are more affected than males by myasthenia gravis. True/False?

A

True

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12
Q

List clinical features of myasthenia gravis

A

Fluctuating weakness
Tiredness at end of the day
Ocular signs (ptosis)

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13
Q

What is the treatment for myasthenia gravis?

A
Acetylcholinesterase inhibitor (pyridosigmine)
Thymectomy
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14
Q

What are muscle fasciculations?

A

Visible fine fast spontaneous twitches that occur in denervated muscle that become excited

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15
Q

What is myotonia?

A

Failure of muscle relaxation after use

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16
Q

What is the commonest muscular dystrophy?

A

Myotonic dystrophy

17
Q

List clinical features of myotonic dystrophy

A

Weakness
Cataracts, ptosis
Frontal balding
Cardiac defects