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
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
3
Q
pathophys of MG in 15% of pts
A
- MuSK ab -> decreased AChR on membrane
- impacts trafficking of receptors
4
Q
what is usu assoc with MG
A
- thymus disorder
- hyperplasia in young
- thymoma in older: benign or malignant
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
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
7
Q
treatment of MG
A
- long acting anticholinesterase: pyridostigmine or neostigmine
- immunosuppressants
- surgical thymectomy
- plasmapheresis in crisis
- IV IG in crisis
8
Q
immunosuppressants used in MG
A
- steroids*
- cyclosporine A
- azathioprine
- mycophenolate mofetil
- MTX
- cyclophosphamide
9
Q
what is a tic
A
- brief, rapid, recurrent, purposeless motor contraction
- motor vs sensory
10
Q
types of motor tics
A
- simple: individual muscle groups
complex: multiple muscle groups - phonic ticks: simple vs complex
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
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
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
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
15
Q
causes of GBS
A
- campylobacter jejuni most common in US
- CMV, EBV
- HIV
- mycoplasma pneumonia
- flu, rabies vaccine
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
17
Q
subtypes of GBS
A
- acute inflammatory demyelinating polyneuropathy- most common
- acute motor axonal neuropathy
- acute motor sensory axonal neuropathy
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
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
Q
spastic CP
A
- 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
Q
dyskinetic CP
A
- d/t basal ganglia damage
- recurrent uncontrollable mvmnt
- tone fluctuates
27
Q
ataxic CP
A
- d/t cerebellum damage
- least common type
- generalized hypotonia
- loss of muscle coord
- wide gait
28
Q
PE findings for CP
A
- delayed motor dev
- persistent or late primitive reflexes
- spasticity, hyperreflexia, ataxia
- involuntary mvmt
- microcephaly
29
Q
other neuro disorders assoc with CP
A
- most have pain
- bladder problems
- seizure disorder
- sleep disorder
- varying degree of language, behavioral, vision, and sensory disorders
30
Q
diagnosis of CP
A
- MRI
- genetic and metabolic testing
31
Q
treatment for CP
A
- general mgmt- nutrition and proper care
- pharm mgmt based on sx
- surgery to relieve muscle tightness
- physical aids
- rehab services
- family services
32
Q
pharm options for CP
A
- botox, baclofen for muscle spasms and seizures
- glycopyrrolate- control drooling
- pamidronate- help with osteoporosis
33
Q
multiple sclerosis
A
- autoimmune disease of CNS
- chronic inflammation, demyelination, gliosis, and neuronal loss
- can be relapsing or progressive
- course is VERY variable
34
Q
risk factors for MS
A
- genetic predisposition
- vit D def
- EBV exposure after early childhood
- cigarette smoking
- high salt diet
35
Q
pathogenesis of MS
A
- perivenular cuffing by inflammatory mononuclear cells, predom T cells and macrophages
- BBB gets disrupted
- demyelination is hallmark
- spares axons usually
36
Q
si/sx of MS
A
- optic neuritis
- exs induced weakness of limbs
- facial weakness
- spasticity
- ataxia
- vertigo
- sensory sx: paresthesias, hypesthesia
37
Q
optic neuritis
A
- often presenting sx of MS
- diminished visual acuity
- dimness
- decreased color perception
- occurs in central field of vision
38
Q
clinical types of MS
A
- remission
- relapsing remitting disease
- secondary progressive disease
- primary progressive disease
39
Q
remission of MS
A
- remodeling of demyelinating axonal plasma membrane
- more Na channels to permit conduction despite myelin loss
40
Q
relapsing remitting disease of MS
A
- progression with relapses of active disease
- complete recovery during remission
41
Q
secondary progressive MS
A
- disease becomes more aggressive
- consistent worsening of function
- usu progression from relapsing remitting disease
42
Q
primary progressive MS
A
- sx are progressive from onset
- early disability
- only about 15% of pts
43
Q
diagnosis of MS
A
- 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
Q
treatment for acute attacks of MS
A
- IV methylprednisolone 3-5 d
- +/- PO taper over 2 weeks
45
Q
long term tx for MS
A
- disease modifying therapy
- spasticity: baclofen, cyclobenzaprine
- optic neuritis: steroids
- fatigue: amantidine, anti-depressants
- pain: gabapentin, pregabalin
- tremor: propranolol, primodone
46
Q
what are the disease modifying agents for MS
A
- interferon beta
- dimethylfumerate
- natalizumab
- alemtuzumab
