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77 Cards in this Set
- Front
- Back
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4 kinds of motor neuron disease
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ALS
PLS PMA PBP |
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2 kinds of MND which can affect UMN and LMN
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ALS
PBP |
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PBP is ___
it is limited to ___. |
progressive bulbar palsy
bulbar musculature |
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PLS is ___
it affects ___MNs only |
1ary lateral sclerosis
upper |
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PMA is ___
it affects ___MNs only |
progressive muscular atrophy
lower |
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main presentation of PLS is ___
it progresses faster/slower than ALS, with more/less bulbar involvement |
spasticity
slower less |
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bulbar sx in PBP
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dysarthria
dysphagia dyspnea |
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5 signs of PBP
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bulbar affect
tongue fasciculations jaw jerk jaw/neck weakness low FVC |
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___ is the most common inherited MND. it is a ___ trait.
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SMA
AR |
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3 kinds of neuropathy
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mononeuropathy simplex
mononeuropathy multiplex polyneuroapathy |
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weakness in polyneuropathy starts ___ly
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distally
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first reflex affected in polyneuropathy is ___
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ankle jerk
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3 kinds of acute polyneuropathy
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GBS
porphyria toxin |
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weakness in GBS is symmetric/asymmetric
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symmetric
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T/F: GBS weakness can involve cranial nerves
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true
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T/F: bowel and bladder continence are commonly affected in GBS
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false
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GBS commonly follows ___
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infection by C. jejuni
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GBS Tx: either ___ or ___
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plasmapheresis
IVIg |
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5 causes of subacute polyneuropathy
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vasculitis
paraneoplastic CIDP toxins drugs |
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4 kinds of chronic polyneuropathy
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metabolic
nutritional infection inherited |
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4 metabolic causes of polyneuropathy
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DM
CRF chronic liver failure thyroid disease |
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nutritional cause of polyneuropathy
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B12 def
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2 infections which cause polyneuropathy
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HIV
leprosy |
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workup of polyneuropathy includes ___
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EMG
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given a polyneuropathy is present, EMG does ___ (2)
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distinguishes axonal from demyelinating disease
measure severity |
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top 5 MG sx
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diplopia
ptosis dysarthria lower extremity weakness dysphagia |
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in MG, weakness is/isn't localized to single nerve root
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isn't
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T/F: MG affects pupillary reflexes
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false
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MG patients show ___ after exertion during physical exam
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worsening weakness
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most common diagnostic test for MG
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tensilon (edrophonium) test
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edrophonium is a ___
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short-acting AChEI
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if MG is diagnosed, a ___ study is indicated
the reason is ___. |
chest CT
exclude thymoma |
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2 EMG findings in MG
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decremental response to repetitive stimulation
increased jitter in SFEMG |
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MG Tx (5)
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AChEI
plasmapheresis IVIg thymectomy immunomosuppresant |
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3 immunosuppressants used in MG
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prednisone
azathioprine mycophenolate mofetil |
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Lambert Eaton (LEMS) is caused by ___
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autoantibodies against presynaptic cholinergic Ca2+ channel
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LEMS affects ___ (3) primarily
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shoulder girdle
pelvic girdle legs |
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in contrast to MG, in LEMS ___
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later contractions may be stronger
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LEMS is usually part of ___
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paraneoplastic syndrome
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LEMS is most strongly associated with ___ cancer
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small cell lung
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LEMS Tx (3)
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see MG tx
guanidine 3,4 diaminopyridine |
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diaminopyridine mechanism
this helps because ___ |
presynaptic K+ channel blocker
depolarization is extended |
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3 kinds of muscle disease
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dystrophy
congenital myopathy acquired myopathy |
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2 dystrophies
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muscular dystrophy
myotonic dystrophy |
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DMD is a ___ trait. It presents by age ___ and is associated with ___.
Life expectancy is <___. |
XLR
5 mental retardation 20 |
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death in DMD is due to ___ (2).
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respiratory failure
cardiomyopathy |
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weakness in DMD is ___
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proximal
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DMD is associated with the ___ sign, in which ___.
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Gowers
patient uses upper limbs to support standing up |
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in contrast to DMD, in Becker MD ___ (2)
Becker MD is more/less severe than DMD |
dystrophin is produced
dystrophin is altered less |
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myotonic dystrophy usually presents at ___th decade
it is a ___ trait |
3rd or 4th
AD |
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myotonic dystrophy is a ___ genetic disorder. therefore it can exhibit ___.
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trinucleotide repeat
anticipation |
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myotonic dystrophy presents with (4)
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distal weakness
ptosis facial muscle atrophy (hatchet face) tongue weakness |
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3 conditions associated with myotonic dystrophy
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cardiac conduction problems
fertility problems DM |
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3 inflammatory myopathies
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polymyositis
dermatomyositis inclusion body myositis |
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inflammatory myopathies commonly present with (4)
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fatigue
weakness muscle pain high CPK |
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polymyositis usually occurs after age ___
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18
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weakness in polymyositis is ___
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proximal
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4 sx of polymyositis
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Raynaud
arthralgia weight loss low-grade fever |
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polymyositis is associated with ___
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slight increase in cancer risk
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polymyositis tx (2)
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prednisone
MTX |
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T/F: polymyositis affects lower extremities more than upper
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true
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in contrast to polymyositis, dermatomyositis ___s
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commonly affects kids
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dermatomyositis affects ___ primarily
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proximal muscles
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dermatomyositis can have fulminant presentation with ___ (2)
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rhabdomyolysis
ARF |
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dermatomyositis can have ___ changes, which commonly precede ___
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skin
muscle |
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most common skin change in dermatomyositis
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heliotropic rash
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dermatomyositis is associated with ___
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large increase in cancer risk
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4 cancers associated wtih dermatomyositis
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ovarian
lung pancreatic CRC |
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tx of dermatomyositis
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IVIg
prednisone MTX |
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IBM affects people over age ___
it has ___ predominance |
50
male (3:1) |
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T/F: weakness in IBM is symmetric
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false
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IBM affects ___ extremities primarily
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lower
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in contrast to polymyositis, ___ (2) is common in IBM
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dysphagia
facial weakness |
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tx for IBM
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???
(immunosuppression is ineffective) |
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workup for suspected muscle disease (4)
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blood test
genetic test EMG muscle biopsy |
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in blood test for muscle disease, do (3)
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CPK
aldolase ESR |
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genetic tests for muscle disease
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DMD
myotonic dystrophy |
