Neurology - Neuromuscular Disorders and their Treatments Flashcards
proximal symmetrical weakness with reflexes spared until late indicates what
a.anterior horn disorder
b.peripheral neuropathy
c.myopathy
c.myopathy
possible causes of myopathy
autoimmune
endocrine - thyroid, `adrenal
toxic - corticosteroids, colchines, chloroquine , alcohol, statins
viral - HIV, HCV
METABOLIC - hyper/hypo ca/k
which of these is a cause of inflammatory myopathy
a.dermatitis
b.psoriasis
c.dermatomyositis
d.rheumatoid arthiritis
c.dermatomyositis
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
c.dermatomyositis
which of these malignancies is not associated with dermatomyositis
a.lung
b.breast
c.ovarian
d.lymphoma
e.brain
e.brain
how is dermatomyositis diagnosed
a.ct
b.ultrasound
c.culture
d.skin biopsy
e.muscle biopsy
e.muscle biopsy
perifascicular atrophy and perimysial atrophy
what is the acute treatment for dermatomyositis
a.methrotrexate
b.steroids
c.IVIG
d.ciclosporin
e.rituximab
f.cyclophosphamide
b.steroids
what is the long term immunosurpressant for dermatomyositis
a.methrotrexate
b.steroids
c.IVIG
d.ciclosporin
e.rituximab
f.cyclophosphamide
a.methrotrexate. or azathioprine
if patient has dermatomyositis and methotrexate is not effective / tolerated what is given
a.methrotrexate
b.steroids
c.IVIG
d.ciclosporin
e.rituximab
c.IVIG d.ciclosporin
what is the 3rd line drug for dermatomyositis
a.methrotrexate
b.steroids
c.IVIG
d.ciclosporin
e.rituximab
e.rituximab
the majority of myasthenia gravis is caused by autoantibodies to what
a.ACH r
b.MuSK
c.calcium channels
d.potassium channels
a.ACH r
blocking of the ach receptor by antibodies in myasthenia gravis leads to what
degradation and endocytosis of ach receptors
degeneration of the motor end plate and postsynaptic folds
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
c.young women and old men
what is the clinical hallmark of myasthenia gravis
FATIGUABILITY
worsens after movement
improves after rest
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
c.myasthenia gravis
in myasthenia gravis reflexes are….
a.increased
b.decreased
c.normal
c.normal
which antibodies are tested for in myasthenia gravis
a. AChR and MuSK
b.anti CCP
c.anti transglutaminase
d.fibrillin
a. AChR and MuSK
what is done to exclude thyoma in myasthenia gravis
a.achr antibody
b.muSK antibody
c. endophonium IV
d.CT chest
e.Ultrasound
d.CT chest
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
d.5
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
b.repetitive nerve stimulation
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
e.single fibre EMG
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
d.neuromuscular junction
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. pyridostigmine
given 1 with propantheline
excaerbating factors for mG
infection
stress
rapid increase of steroids
anaemia
drugs
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
e.myasthenic crisis
fvc less than what requires ICU support in MG crisis
a.2l
b.4l
c.5l
d.1l
e.0.75 l
d.1l
whats the treatment for myasthenic crisis
iv immunoglobulins
plasma exchange
ventilatory support
NBM and NG feeding
what happens to reflexes EARLY in neuropathies
a.increase
b.decrease
b.decrease
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
f.neuropathies
what are neuropathies broadly grouped into
demyelinating
axonal
distinguished by nerve conductiio studies
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
f.nerve conduction studies
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.conduction velocity reduced , amplitude of AP preserved
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
d.conduction velocity preserved, amplitude of AP reduced
length dependent (distal) symmetrical weakness and sensory loss indicates what
polyneuropathy
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
b.guillian barre
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
b.guillian barre
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
b.guillian barre
what is elevated in the csf in guillian barre
a.billirubin
b.protein
c.achr
d.musk
b.protein
what is given for GBS
IVIG
airway support
is MND fatal?
fatal and degenerative
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
f.motor neurone disease
subtypes of motor neurone disease
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
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.amytrophic lateral sclerosis
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
b.progressive muscular atrophy
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
c.progressive bulbar palsy
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
d.primary lateral sclerosis
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
f.motor neurone disease
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
g.MRI spine
