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3 Neurology > Neuromuscular Disorders > Flashcards

Neuromuscular Disorders Flashcards

1
Q

skeletal muscle fibres are innervated by __ neurons whose cell bodies arise in the __ horn of the spinal cord

A

motor

ventral/anterior

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2
Q

define the motor end plate

A

synapses formed between terminal motor neurons and skeletal muscle

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3
Q

this enzyme breaks down Ach into ___ and ____

A

acetylcholinesterase
acetone
choline

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4
Q

is acetate or choline sequestered into presynaptic vesicles?

A

choline

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5
Q

clostridium botulinum exists where?

A

soil

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6
Q

MoA of botox?

A

cleaves presynaptic proteins involved in vesicle formation

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7
Q

lambert eaton myaesthenic syndrome involves an inflammatory response against what structure?

A

presynaptic Ca channels

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8
Q

Tx of lambert eaton myeasthenic syndrome?

A

3,4- diaminopyridine

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9
Q

LEMS is associated with what condition?

A

small cell carcinoma

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10
Q

myaesthenia gravis is a __-synaptic disorder

A

post

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11
Q

most common disorder of the NMJ

A

myaesthenia gravis

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12
Q

myaesthenia gravis is an autoimmune condition T or F

A

T

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13
Q

pathophysiology of myaesthenia gravis

A

antibodies made against ACh receptors causing muscle weakness and fatigue

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14
Q

symptoms of MG begin when ACh receptors are reduced to __% of normal

A

30

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15
Q

what gland plays a particular role in myaesthenia gravis?

A

thymus

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16
Q

name the 2 peaks of incidence of MG and the genders they appear in

A

females in 30s

men in 60s/70s

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17
Q

clinical presentation of MG

A

fluctuating weakness worse during day
extraocular, facial, bulbar weakness
mainly proximal limb weakness

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18
Q

limb weakness is worst __lly

A

proximally

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19
Q

Tx of MG acutely?

A

acetylcholinesterase inhibitor eg pyridostignmine
IV Ig’s and/or plasma exchange
thymectomy if thymus involved

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20
Q

mainstay Tx of MG?

A

immunomodulators
steroids
azathioprine (immunosuppressant)

21
Q

what antibiotic should be avoided in MG?

A

gentamicin

22
Q

smallest contractile unit in skeletal muscle

A

muscle fibre

23
Q

which type of muscle has no sarcomeres?

A

smooth muscle

24
Q

actin myosin ratio of smooth muscle?

25
Q

fasciculations can be triggered by what?

A

stress
caffeine
fatigue

26
Q

fasciculations are indicative of disease where?

A

motor neurone (NOT muscle)

27
Q

where is the problem in myotonia?

A

chloride channels

28
Q

define myotonia

A

failure of muscle relaxation after use

29
Q

symptoms and signs of muscle disease?

A

myalgia
muscle weakness
wasting
hyporeflexia

30
Q

immune mediated muscle disorders?

A

dermatomyositis

polymyositis

31
Q

inherited muscle disorders?

A

muscular dystrophies
dystrophinopathies
myotonic dystrophies

32
Q

congenital muscle disorders?

A

myaesthenic syndromes

congenital myopathies

33
Q

describe the pattern of muscle weakness in polymyositis

A

symmetrical progressive proximal weakness that has a chronic onset

34
Q

best marker for polymyositis?

35
Q

slowly progressive weakness in a patient in their 60s with sparing of their thumb…

A

inclusion body myositis

36
Q

commonest MD

A

myotonic MD

37
Q

how is myotonic MD inherited?

38
Q

clinical presentation of myotonic MD

A 
myotonia
weakness
cataract
ptosis
frontal badling 
cardiac defects
39
Q

muscular dystrophies are non inflammatory T or F

40
Q

central and peripheral nerve dysfunction is a symptom of muscular dystrophy T or F

A

F, central and peripheral nerves not affected

41
Q

infective causes of MD?

A

coxsackie
trpanosomiasis
cistercercosis
borrelia

42
Q

patient with new onset myotonia after eating uncooked pork….

A

cistercercosis

43
Q

what electrolyte imbalance can cause a myopathy?

A

hypokalemia

44
Q

describe rhabdomyolysis?

A

skeletal muscle causes leakage of large quantities of toxic intracellular contents into plasma

45
Q

triad of symptoms of rhabdomyolysis?

A

myalgia
muscle weakness
myoglobinuria

46
Q

scoring a 4 on muscle grading means what?

A

movement against resistance but not full strength

47
Q

scoring a 3 on muscle grading means what?

A

movement against gravity but not against resistance

48
Q

scoring a 1 in muscle grading means what?

A

flicker of movement when attempting to contract muscle

3 Neurology (41 decks)

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