motor disorders Flashcards

1
Q

myasthenia gravis (MG)

A
  • painless weakness and fatiguability of skel muscles
  • disease of NMJ
  • bimodal age distribution: women 20-30, men 50-60
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2
Q

most common pathophys of MG

A
  • autoab directed against AcH receptors
  • decreased AcHR at post synaptic muscle membrane
  • causes weak contractions
  • AcH does not get repleated fast enough
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3
Q

pathophys of MG in 15% of pts

A
  • MuSK ab -> decreased AChR on membrane

- impacts trafficking of receptors

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4
Q

what is usu assoc with MG

A
  • thymus disorder
  • hyperplasia in young
  • thymoma in older: benign or malignant
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5
Q

si/sx of MG

A
  • weakness and fatiguability- worse with repetitive stim
  • exacerbations and remissions possible
  • infx or systemic disorders -> crisis
  • diplopia
  • ptosis
  • facial weakness
  • nasal timbre speech
  • difficulty swallowing -> aspiration risk
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6
Q

diagnosis of MG

A
  • Ab to AChR, MuSK, or Ipr4
  • electro diagnostic testing: repetitive nerve stimulation -> reduced amplitude
  • edrophonium -> improved muscle strength
  • CT or MRI to exclude intracranial lesions
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7
Q

treatment of MG

A
  • long acting anticholinesterase: pyridostigmine or neostigmine
  • immunosuppressants
  • surgical thymectomy
  • plasmapheresis in crisis
  • IV IG in crisis
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8
Q

immunosuppressants used in MG

A
  • steroids*
  • cyclosporine A
  • azathioprine
  • mycophenolate mofetil
  • MTX
  • cyclophosphamide
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9
Q

what is a tic

A
  • brief, rapid, recurrent, purposeless motor contraction

- motor vs sensory

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10
Q

types of motor tics

A
  • simple: individual muscle groups
    complex: multiple muscle groups
  • phonic ticks: simple vs complex
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11
Q

tourette’s syndrome

A
  • multiple motor tics often present with vocalizations/ phonic tics
  • mainly affects males
  • can suppress tic for short period of time but then experience irresistible urge to express them
  • varies in intensity
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12
Q

etiology and pathogenesis of tourette’s syndrome

A
  • thought to be genetic
  • no specific gene mutation
  • may have enviorn influence
  • possibly d/t decreased dompamine, opioids, second messenger systemics
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13
Q

treatment for tourette’s

A
  • education, counseling
  • no singular effective tx
  • alpha adrenergic agonists: clonidine, guanfacine
  • neuroleptics: typical vs atypical
  • botulism inj if focal tic
  • deep brain stimulation possible
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14
Q

guillain barre syndrome

A
  • acute, severe fulminant polyradiuclopathy
  • acute onset immune mediated demyelinating neuropathy
  • does not impact CNS
  • majority occur 1-3 weeks after acute respiratory or GI infx
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15
Q

causes of GBS

A
  • campylobacter jejuni most common in US
  • CMV, EBV
  • HIV
  • mycoplasma pneumonia
  • flu, rabies vaccine
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16
Q

si/sx of GBS

A
  • rapidly evolving ascending paralysis
  • weakness evolves over hours- days
  • asoc with tingling dysesthesias in extremities
  • legs more affected than arms
  • facial diapresis
  • pain
  • autonomic involvement -> fluctuation in BP, postural hypotension, cardiac dysrhythmias
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17
Q

subtypes of GBS

A
  • acute inflammatory demyelinating polyneuropathy- most common
  • acute motor axonal neuropathy
  • acute motor sensory axonal neuropathy
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18
Q

pathophys of GBS

A
  • demyelination -> conduction block -> flaccid paralysis and sensory disturbances
  • axonal conduction usu remains in tact
  • if axonal degeneration -> longer recovery and residual disability
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19
Q

diagnosis of GBS

A
  • based on rapidly evolving paralysis with areflexia, absence of fever, systemic sx, hx
  • CSF findings: increased proteins without pleocytosis
  • electrodiagnostics- slowed conduction velocity, conduction block, temporal dispersion
20
Q

treatment of GBS

A
  • IV IG or plasmapheresis
  • steroids not effective
  • 30% require vents
  • most fully recover within few months to a year
  • death secondary to pulm complications
21
Q

cerebral palsy

A
  • neuro dysfunction that occurs in developing brain
  • perm affects body mvmt, muscle coord, posture, balance
  • not progressive
22
Q

what is the most common cause of CP

A
  • antenatal causes
  • cerebral dysgenesis
  • congenital infections (TORCH)
  • substance abuse
  • prematurity
23
Q

other causes of CP

A
  • intrapartum: birth asphyxia, trauma

- post natal: intravent hemorrhage/ ischemia, meningitis, encephalitis, head trauma, hyperbilirubinemia

24
Q

types of CP

A
  • spastic- most common
  • dyskinetic
  • ataxia
25
spastic CP
- produces upper motor neuron type disease - d/t cortex damage - muscles stiffer than usual - may only be present in one part of body, half of body, or one side - extensors of UE - flexors of LE - tip toed walk
26
dyskinetic CP
- d/t basal ganglia damage - recurrent uncontrollable mvmnt - tone fluctuates
27
ataxic CP
- d/t cerebellum damage - least common type - generalized hypotonia - loss of muscle coord - wide gait
28
PE findings for CP
- delayed motor dev - persistent or late primitive reflexes - spasticity, hyperreflexia, ataxia - involuntary mvmt - microcephaly
29
other neuro disorders assoc with CP
- most have pain - bladder problems - seizure disorder - sleep disorder - varying degree of language, behavioral, vision, and sensory disorders
30
diagnosis of CP
- MRI | - genetic and metabolic testing
31
treatment for CP
- general mgmt- nutrition and proper care - pharm mgmt based on sx - surgery to relieve muscle tightness - physical aids - rehab services - family services
32
pharm options for CP
- botox, baclofen for muscle spasms and seizures - glycopyrrolate- control drooling - pamidronate- help with osteoporosis
33
multiple sclerosis
- autoimmune disease of CNS - chronic inflammation, demyelination, gliosis, and neuronal loss - can be relapsing or progressive - course is VERY variable
34
risk factors for MS
- genetic predisposition - vit D def - EBV exposure after early childhood - cigarette smoking - high salt diet
35
pathogenesis of MS
- perivenular cuffing by inflammatory mononuclear cells, predom T cells and macrophages - BBB gets disrupted - demyelination is hallmark - spares axons usually
36
si/sx of MS
- optic neuritis - exs induced weakness of limbs - facial weakness - spasticity - ataxia - vertigo - sensory sx: paresthesias, hypesthesia
37
optic neuritis
- often presenting sx of MS - diminished visual acuity - dimness - decreased color perception - occurs in central field of vision
38
clinical types of MS
- remission - relapsing remitting disease - secondary progressive disease - primary progressive disease
39
remission of MS
- remodeling of demyelinating axonal plasma membrane | - more Na channels to permit conduction despite myelin loss
40
relapsing remitting disease of MS
- progression with relapses of active disease | - complete recovery during remission
41
secondary progressive MS
- disease becomes more aggressive - consistent worsening of function - usu progression from relapsing remitting disease
42
primary progressive MS
- sx are progressive from onset - early disability - only about 15% of pts
43
diagnosis of MS
- 2 episodes of 2 that occur at different points in time - absence of other causes - MRI shows plaques - LP- mild pleocytosis and total protein normal - slowed or abnormal conduction in response to visual or auditory stimuli
44
treatment for acute attacks of MS
- IV methylprednisolone 3-5 d | - +/- PO taper over 2 weeks
45
long term tx for MS
- disease modifying therapy - spasticity: baclofen, cyclobenzaprine - optic neuritis: steroids - fatigue: amantidine, anti-depressants - pain: gabapentin, pregabalin - tremor: propranolol, primodone
46
what are the disease modifying agents for MS
- interferon beta - dimethylfumerate - natalizumab - alemtuzumab