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Neurology 1 > Neurology - Neuromuscular Disorders and their Treatments > Flashcards

Neurology - Neuromuscular Disorders and their Treatments Flashcards

1
Q

proximal symmetrical weakness with reflexes spared until late indicates what

a.anterior horn disorder
b.peripheral neuropathy
c.myopathy

A

c.myopathy

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2
Q

possible causes of myopathy

A

autoimmune
endocrine - thyroid, `adrenal
toxic - corticosteroids, colchines, chloroquine , alcohol, statins

viral - HIV, HCV

METABOLIC - hyper/hypo ca/k

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3
Q

which of these is a cause of inflammatory myopathy

a.dermatitis
b.psoriasis
c.dermatomyositis
d.rheumatoid arthiritis

A

c.dermatomyositis

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4
Q

gottron papules, periorbital oedema, helitrope rash, v sign, shawl sign and mechanic hands are seen in what

a.dermatitis
b.psoriasis
c.dermatomyositis
d.rheumatoid arthiritis

A

c.dermatomyositis

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5
Q

which of these malignancies is not associated with dermatomyositis

a.lung
b.breast
c.ovarian
d.lymphoma
e.brain

A

e.brain

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6
Q

how is dermatomyositis diagnosed

a.ct
b.ultrasound
c.culture
d.skin biopsy
e.muscle biopsy

A

e.muscle biopsy

perifascicular atrophy and perimysial atrophy

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7
Q

what is the acute treatment for dermatomyositis

a.methrotrexate
b.steroids
c.IVIG
d.ciclosporin
e.rituximab
f.cyclophosphamide

A

b.steroids

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8
Q

what is the long term immunosurpressant for dermatomyositis

a.methrotrexate
b.steroids
c.IVIG
d.ciclosporin
e.rituximab
f.cyclophosphamide

A

a.methrotrexate. or azathioprine

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9
Q

if patient has dermatomyositis and methotrexate is not effective / tolerated what is given

a.methrotrexate
b.steroids
c.IVIG
d.ciclosporin
e.rituximab

A

c.IVIG d.ciclosporin

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10
Q

what is the 3rd line drug for dermatomyositis

a.methrotrexate
b.steroids
c.IVIG
d.ciclosporin
e.rituximab

A

e.rituximab

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11
Q

the majority of myasthenia gravis is caused by autoantibodies to what

a.ACH r
b.MuSK
c.calcium channels
d.potassium channels

A

a.ACH r

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12
Q

blocking of the ach receptor by antibodies in myasthenia gravis leads to what

A

degradation and endocytosis of ach receptors

degeneration of the motor end plate and postsynaptic folds

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13
Q

who is most at risk of myasthenia gravis

a.young women and old women
b. old men and old women
c.young women and old men
d. young men and old women

A

c.young women and old men

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14
Q

what is the clinical hallmark of myasthenia gravis

A

FATIGUABILITY

worsens after movement
improves after rest

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15
Q

ptosis, opthalmoplegia , weakness chewing and swallowing and neck weakness with preserved reflexes and NO sensory involvement indicates what

a.eaton lambert
b.myopathy
c.myasthenia gravis
d. duchenne muscular dystrophy

A

c.myasthenia gravis

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16
Q

in myasthenia gravis reflexes are….

a.increased
b.decreased
c.normal

A

c.normal

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17
Q

which antibodies are tested for in myasthenia gravis

a. AChR and MuSK
b.anti CCP
c.anti transglutaminase
d.fibrillin

A

a. AChR and MuSK

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18
Q

what is done to exclude thyoma in myasthenia gravis

a.achr antibody
b.muSK antibody
c. endophonium IV
d.CT chest
e.Ultrasound

A

d.CT chest

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19
Q

in repeptitive nerve stimulation decrement of >10% seen in how many responses is consistent with a diagnosis of MG

a.2
b.3
c.4
d.5
e.6

A

d.5

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20
Q

which test for MG is unreliable if the patient is cold or on acetycholineesterase inhibitors

a.achr antibody
b.repetitive nerve stimulation
c. endophonium IV
d.CT chest
e.single fibre EMG

A

b.repetitive nerve stimulation

21
Q

which test records from 2 muscle fibres in a motor unit and is more reliable for MG diagnosis

a.achr antibody
b.repetitive nerve stimulation
c. endophonium IV
d.CT chest
e.single fibre EMG

A

e.single fibre EMG

22
Q

increased variability in the interval between paired AP termed jitters / occasional blockage of one of the potentials in a single fire EMG indicates a defect in transmission where

a.anterior horn
b.posterior horn
c.nerve root
d.neuromuscular junction

A

d.neuromuscular junction

23
Q

which drug for MG inhibits ACh breakdown by acetylcholinesterase in the synaptic cleft thus increasing time for the synaptic response to ACh

a. pyridostigmine
b.steroids
c.azathioprine
d.methrotrexate
e.rituximab

A

a. pyridostigmine

given 1 with propantheline

24
Q

excaerbating factors for mG

A

infection
stress
rapid increase of steroids
anaemia
drugs

25
Q

increasing muscle weakness and diplopia accompanied by respiratory failure and often normal o2 saturation and normal blood gases (until late) indicates what

a.eaton lambert
b.myopathy
c.myasthenia gravis
d. duchenne muscular dystrophy
e.myasthenic crisis
f.addisonian crisis

A

e.myasthenic crisis

26
Q

fvc less than what requires ICU support in MG crisis

a.2l
b.4l
c.5l
d.1l
e.0.75 l

A

d.1l

27
Q

whats the treatment for myasthenic crisis

A

iv immunoglobulins
plasma exchange
ventilatory support
NBM and NG feeding

28
Q

what happens to reflexes EARLY in neuropathies

a.increase
b.decrease

A

b.decrease

29
Q

sensory aand motor symptoms early reflexia and wasting is seen in what

a.eaton lambert
b.myopathy
c.myasthenia gravis
d. duchenne muscular dystrophy
e.dermatyomyositis
f.neuropathies

A

f.neuropathies

30
Q

what are neuropathies broadly grouped into

A

demyelinating
axonal

distinguished by nerve conductiio studies

31
Q

how are demyelinating and axonal neuropathies distinguished

a.achr antibody
b.repetitive nerve stimulation
c. endophonium IV
d.CT chest
e.single fibre EMG
f.nerve conduction studies

A

f.nerve conduction studies

32
Q

which is true of demyelination neuropathies

a.conduction velocity reduced , amplitude of AP preserved

b.conduction velocity reduced, amplitude of AP reduced

c.conduction velocity preserved amplitude of AP preserved

d.conduction velocity preserved, amplitude of AP reduced

A

a.conduction velocity reduced , amplitude of AP preserved

33
Q

which is true of d axonal neuropathies

a.conduction velocity reduced , amplitude of AP preserved

b.conduction velocity reduced, amplitude of AP reduced

c.conduction velocity preserved amplitude of AP preserved

d.conduction velocity preserved, amplitude of AP reduced

A

d.conduction velocity preserved, amplitude of AP reduced

34
Q

length dependent (distal) symmetrical weakness and sensory loss indicates what

A

polyneuropathy

35
Q

which of these conditions is an acute inflammatory demyelinating polyradiculoneuropathy (aidp)

a.eaton lambert
b.guillian barre
c.myasthenia gravis
d. duchenne muscular dystrophy
e.dermatyomyositis
f.neuropathies

A

b.guillian barre

36
Q

proximal weakness with acute infective triggers 1-2 weeks prior eg cmv, m.pneum.campylobacter jejuni

a.eaton lambert
b.guillian barre
c.myasthenia gravis
d. duchenne muscular dystrophy
e.dermatyomyositis
f.neuropathies

A

b.guillian barre

37
Q

progressive weakness over hours- days, no reflexes , relatively symmetrical with more motor that sensory and facial involvement indicates what

a.eaton lambert
b.guillian barre
c.myasthenia gravis
d. duchenne muscular dystrophy
e.dermatyomyositis
f.neuropathies

A

b.guillian barre

38
Q

what is elevated in the csf in guillian barre

a.billirubin
b.protein
c.achr
d.musk

A

b.protein

39
Q

what is given for GBS

A

IVIG
airway support

40
Q

is MND fatal?

A

fatal and degenerative

41
Q

degeneration of pyrimidal cells , corticospinal pathways , brainstem motor nuclei and anterior horn cells occurs in what

a.eaton lambert
b.guillian barre
c.myasthenia gravis
d. duchenne muscular dystrophy
e.dermatyomyositis
f.motor neurone disease

A

f.motor neurone disease

42
Q

subtypes of motor neurone disease

A

amytrophic lateral sclerosis - most common mis of upper and lower mn signs

progressive muscular atrophy - LMN only, can starts with 1 limb

progressive bulbar palsy - confined to bulbar dysfunction (speaking , swallowing ) may progress to ALS

43
Q

which type of MND has a mix of upper and lower motor neuron signs and is most common

a.amytrophic lateral sclerosis

b.progressive muscular atrophy

c.progressive bulbar palsy

A

a.amytrophic lateral sclerosis

44
Q

which type of MND has LMN signs only and can start in one limb

a.amytrophic lateral sclerosis

b.progressive muscular atrophy

c.progressive bulbar palsy

A

b.progressive muscular atrophy

45
Q

which type of MND is confined to bulbar dysfunction (speaking and swallowing ) but may progress to ALS

a.amytrophic lateral sclerosis

b.progressive muscular atrophy

c.progressive bulbar palsy

A

c.progressive bulbar palsy

46
Q

Which type of MND has upper motor neuron signs only

a.amytrophic lateral sclerosis

b.progressive muscular atrophy

c.progressive bulbar palsy

d.primary lateral sclerosis

A

d.primary lateral sclerosis

47
Q

painless progressive weakness and wasting, jaw weakness, tongue fasciculations, head drop, umn and lmn in the limbs, cachexia and normal eye moevemnts and sensation indicates what

a.eaton lambert
b.guillian barre
c.myasthenia gravis
d. duchenne muscular dystrophy
e.dermatyomyositis
f.motor neurone disease

A

f.motor neurone disease

48
Q

what investigation is done to rule out other causes when a clinical MND diagnosis is made

a.achr antibody
b.repetitive nerve stimulation
c. endophonium IV
d.CT chest
e.single fibre EMG
f.nerve conduction studies
g.MRI spine

A

g.MRI spine

Neurology 1 (44 decks)

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