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77 Cards in this Set

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