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79 Cards in this Set

  • Front
  • Back
pelvic tilt indicates weakness of ___ muscles,
especially___
hip girdle
gluteus medius
as hip extensor weakness progresses ___ occurs
this is characteristic of ___
increased lumbar lordosis
MD
myotonia can be detected by ___ on exam, which triggers ___
percussion of thenar eminence
sustained contraction
4 kinds of neuromuscular disorders
motor neuron disease (MND)
peripheral neuropathy (PN)
neuromuscular junction (NMJ)
myopathy
MNDs have ___ bulk,
___ tone
___ strength
___ sensation
___ reflexes
___ babinski, and
___ pattern
low
high
low
normal
high or low
positive
asymmetric
PNs have ___ bulk,
___ tone
___ strength
___ sensation
___ reflexes
___ babinski, and
___ pattern
low
low
low
low
low
negative
symmetric or asymmetric
MNDs have ___ bulk,
___ tone
___ strength
___ sensation
___ reflexes
___ babinski, and
___ pattern
normal
normal
low
normal
normal
negative
symmetric
MNDs have ___ bulk,
___ tone
___ strength
___ sensation
___ reflexes
___ babinski, and
___ pattern
low
normal
low
normal
normal or low
negative
symmetric
3 kinds of MND
UMN only
LMN only
UMN and LMN
MND affecting UMNs only
PLS
2 kinds of LMN-only MNDs
SMAs
polio-like syndromes
6 kinds of SMA
infantile
juvenile
kennedy's
distal
scapuloperoneal
segmental
infantile SMA is aka
Werdnig-Hoffman
juvenile SMA is aka
Kugelberg-Welander
distal SMA is aka
neuronal CMT
6 polio-like syndromes
polio
other viruses
post-radiation MND
paraneoplastic MND
toxin-induced MND
idiopathic LMND
MND affecting upper and lower motor neurons
ALS
T/F: most ALS is sporadic
true (90%)
2 criteria for ALS dx
UMN + LMN involvement
progression over time
in addition to spasticity and hyperreflexia, ___ is a sign of UMN dysfunction
loss of dexterity
main prognostic factor for ALS
age at onset (older is worse)
typical survival time for ALS
2--3 years
appearance of ___ or ___ with suspected ALS should suggest other diagnoses
sensory sx
autonomic sx
___ is the main study used to support dx of ALS
needle EMG
___ is present in 70% of sporadic ALS cases
this may cause ___
Glu transporter defect
excitotoxicity
___ is a drug approved for ALS
it works by ___ing
survival benefit from its use is ___
riluzole
inhibiting Glu release
2--3 months
2 kinds of neuropathic disorders
neuronopathy
peripheral neuropathy (PN)
neuronopathies affect ___
PNs affect ___
cell body
distal process
2 kinds of PN
myelinopathy
axonopathy
MCC of neuropathy in industrialized countries
DM
5 key diagnostic questions for PNs
what systems are involved
what is the distribution of weakness
what is the nature of the sensory involvement
what is the temporal evoluation
7 neuropathic disorders which may present with motor dysfunction only
MNDs
multifocal motor neuropathy
GBS
CIDP
Pb intoxication
porphyria
CMT
4 neuropathic disorders which may present with motor dysfunction only,
but commonly present WITH sensory sx
GBS
Pb intoxication
porphyria
CMT
neuropathic disorders which commonly have dysautonomia
PNs
4 kinds of dysautonomia
orthostasis
heat intolerance
bowel/bladder dysfunction
sexual dysfunction
7 PNs which commonly cause dysautonomia
DM
amyloidosis
GBS
vincristine-induced
porphyria
HIV dysautonomia
idiopathic pandysautonomia
PNs often associated with pain
cryptogenic sensory PN (CSPN)
DM
vasculitis
GBS
amyloidosis
pain from CSPN and DM is ___ (2)
symmetric
worse in feet
pain in vasculitis is ___ (2)
distal
asymmetric
GBS pain is commonly in ___
it is ___
back
symmetric
if asymmetric loss of proprioception is present, consider ___ (2)
neuronopathy
ganglionopathy
6 causes of neuronopathy + ganglionopathy
cancer
sjogren's
idiopathic sensory neuronopathy
cisplatin
B6 toxicity
HIV sensory neuronopathy
symmetric weakness in proximal and distal muscles implicates ___
demyelinating disorders (GBS, CIDP)
symmetric but only distal weakness implicates
axonal PN
5 kinds of causes of asymmetric weakness
MND
radiculopathy
plexopathy
mononeuropathy multiplex
compressive mononeuropathy
given symmetric proximal and distal weakness
with sensory loss,
consider ___ (2)
GBS
CIDP
given symmetric distal weakness with sensory loss,
consider ___ (5)
DM
drugs
toxins
CMT
amyloidosis
given asymmetric distal weakness with sensory loss at MULTIPLE nerves,
consider ___ (4)
vasculitis
infection
sarcoid
HNPP
3 infections causing asymmetric distal weakness with sensory loss at multiple nerves
Lyme disease
HIV
leprosy
given asymmetric distal weakness with sensory loss at SINGLE nerve/region,
consider ___ (2)
compressive mononeuropathy
radiculopathy
given asymmetric distal weakness without sensory loss,
consider ___ (2)
MND
multifocal motor neuropathy
given asymmetric proximal and distal weakness with sensory loss,
consider ___ (4)
polyradiculopathy
plexopathy
meningeal carcinomatosis
HNPP
given symmetric sensory loss without weakness, consider ___ (4)
DM
drugs
toxin
CSPN
treatment of choice for GBS
plasmapheresis
full recovery rate for GBS
80%
___% of ocular MG have anti-AChR___% of generalized MG have anti-AChR
overall, ___% of MG have anti-AChR
50
90
75
___% of ocular MG progresses to generalized
65
once MG dx is made, you must do ___
CXR
CXR in MG is to check for ___ (2)
thymic hyperplasia
thymoma
thymectomy is indicated for MG if ___ or ___
age < 60 and generalized
thymoma
5 nonsurgical interventions for MG
anti-ChE
azathioprine
CS
plasmapheresis
IVIg
3 kinds of MD
X-linked
AD
AR
3 X-linked MDs
DMD
Becker's MD
Emery-Dreifuss MD
5 AD MDs
myotonic dystrophy
scioscapulohumeral d.
scapulohumeral d.
distal myopathy
oculopharyngeal d.
4 AR MDs
limb-girdle dystrophy
distal myopathy
congenital MD
scapulohumeral MD
lab study for DMD
CPK
___ is the most common dystrophy affecting adults
myotonic d.
incidence of myotonic d.
1/10,000
2 cortical sx of myotonic d.
hypersomnia
intellectual decline
6 non-neurologic sx of myotonic d.
heart block
cataracts
gonadal atrophy
respiratory insufficiency
GI sx
endocrine abnormalities
3 channelopathic myotonic disorders
hyperkalemic periodic paralysis (HPP)
paramyotonia congenita
myotonia congenita
HPP is a defect in a ___ channel
it is a ___ disorder
Na+
AD
HPP presents as ___ which is triggered by ___ (5)
tansient paralysis
rest
exercise
stress
large meals
K+
HPP may be txed with ___
acetazolamide (K+ wasting)
paramyotonia congenita is a defect in a ___ channel
it is a ___ disorder
Na+
AD
unlike myotonia, in paramyotonia sx ___
get worse with repeated muscle use
myotonia congenita is a defect in a ___ channel
it is a ___ disorder
Cl-
AD or AR
AD form of myotonia congenita is aka
AR form of myotonia congenita is aka
Thomsen's disease
Becker's myotonia congenita
3 myotonia tx
quinine
procainamide
phenytoin