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Clinical Neurology > Neuromuscular Disorders > Flashcards

Neuromuscular Disorders Flashcards

1
Q

What is the motor end plate?

A

The synapse formed between motor neurones and muscle

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2
Q

An AP causes calcium channels to open causing what to be released into the synaptic cleft?

A

Vesicles of ACh

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3
Q

When ACh diffuses across the synapse, the membrane becomes permeable to what?

A

Na/K ions

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4
Q

Depolarisation of the membrane of motor neurones causes what?

A

Action potential at motor end plate

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5
Q

What can be used to stop ACh travelling across the synaptic cleft?

A

Acetylcholinesterase

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6
Q

Give some presynaptic neuromuscular disorders

A

Botulism

Lambert Eaton Myasthenic Syndrome

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7
Q

What occurs in botulism?

A

The clostridium botulinum toxin cleaves presynaptic proteins involved in ACh vesicle formation

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8
Q

What occurs in LEMS?

A

Antibodies to presynaptic Ca channels are formed

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9
Q

LEMS is strongly associated with what?

A

Small cell carcinoma

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10
Q

What is the main postsynaptic disorder?

A

Myasthenia gravis

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11
Q

What occurs in myasthenia gravis?

A

Antibodies to acetyl choline receptors (AChR) are formed

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12
Q

Symptoms of MG start when ACh receptors are reduced to what % of normal?

A

30%

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13
Q

What is main test used for MG?

A

Test for anti-AChR

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14
Q

What structure is commonly affected in MG?

A

Thymus

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15
Q

Give some clinical features of MG

A

Extraocular/facial/bulbar weakness

Proximal limb weakness

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16
Q

What is the acute treatment for MG?

A

AChesterase inhibitor (pyridostigmine)
IV immunoglobulin
Thymectomy

17
Q

What is the general treatment of MG?

A

Immunomodulating
Steroids (prednisolone)
Steroid-sparing agents (azathioprine)

18
Q

What drug must be avoided in MG?

A

Gentamicin

19
Q

What is the smallest contractile unit of skeletal muscle?

A

Muscle fibre

20
Q

What are fascicles formed from?

A

Muscle fibres surrounded by thin layer endomysium

21
Q

What are fasciculations?

A

Visible fast spontaneous twitches of muscle

22
Q

When can fasciculations occur pathologically?

A

Denervated muscle which becomes hyperexcitable

23
Q

What is myotonia?

A

The failure of muscle relaxation after use

24
Q

How does inclusion body myositis present?

A

Slowly progressive proximal weakness with thumb sparing

25
Q

Give some symptoms of myotonic dystrophy

A

Myotonia
Weakness
Cataracts
Ptosis

26
Q

What is the main drug class that causes myopathy?

A

Statins

27
Q

What occurs in rhabdomyolysis?

A

Damage to skeletal muscle causes leakage of toxic intracellular contents into plasma

28
Q

What triad of symptoms is seen in rhabdomyolysis?

A

Myalgia
Muscle weakness
Myoglobinuria

29
Q

When do men and women tend to get MG?

A

Females - 20s

Males - 50-70

Clinical Neurology (20 decks)

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