Neuromuscular Disorders Flashcards
myelin is composed of ___ cells in the PNS
Schwann
what do small fibers sense?
pain and temperature
what do large fibers sense?
light touch and vibration
how do the nerves exit the SC?
cervical: above vertebral level
T-S: below
what is the hallmark of ALS disease?
UMN & LMN sx
motor involvement ONLY
LMN signs in ALS
weakness
atrophy
fasciculations all over body
UMN signs in ALS
pathologic spread of reflexes
clonus
spasticity
T/F: there is no sensory loss or cognitive decline with ALS
T
how effective are ALS meds?
only prolong life for about 3 months
meds for ALS
anyone: riluzole & edaravone
genetic: tofersen
which intervention prolongs life for ALS pts better?
BIPAP
____ physical activity increases risk for ALS patients
intense
what is the most common motor neuron disease?
ALS
West Nile sx
weakness in B legs
fever, drowsy
stiff neck & HA
absent reflexes in LE
july-october
how to differentiate neuron injury d/t acute flaccid paralysis vs. Guillain-Barré
AFP: B/B involvement and no image changes
sx of neuro involvement d/t West Nile
aseptic meningitis
meningoencephalitis
acute flaccid paralysis
B/B involvement
post-polio syndrome diagnostic criteria
- hx of paralytic poliomyelitis
- period of partial or complete RECOVERY followed by interval of stable function
- gradual or sudden onset of gradual or sudden progressive and persistent MUSCLE WEAKNESS/FATIGUE
- sx for > 1 year
- exclude other causes
post-polio syndrome causes _____ neuron syndrome
LMN
what is the most common level for radiculopathy?
L5
what is the most common level for nerve injury?
C7
sx of radiculopathy
pain, numbness and tingling dermatomal
weakness w/ corresponding myotome
reduced reflexes
T/F: 95% of cervical radiculopathies d/t herniated disc improve w/o surgical intervention
T
pain difference b/w radiculopathy and LMN lesion?
radic = shooting pain
ABCDs of radiculopathy vs. LMN lesion
A - weakness
B - radicular pain in dermatomal dist
C - muscle atrophy
D - hyporeflexia
what is the most common cause of plexopathy?
trauma
polyneuropathies are _____ dependent
length
plexopathy sx
severe pain then weakness
what is meralgia parasthetica?
lateral femoral cutaneous compressed at inguinal ligament
sensory loss ONLY
carpal tunnel is an example of
mononeuropathy
most myelinated fibers are _____ diameter fibers
large
what is a strong characteristic of demylination?
weakness w/o atrophy
axons regrow _____ per year
2-3 mm
what is the most common axonal polyneuropathy? 2nd?
most - diabetes
2nd - alcohol
T/F: EMG can be normal for small fiber polyneuropthies
T
what sense composes small fiber neurons?
sensory - pain and temp
autonomic
small fiber neuropathy sx
reduced temp discrimination
allodynia
burning, neuropathic pain
LE discoloration, reduced sweating, & hair loss
autonomic dysfunction
what type of nerves are large fibers?
motor
sensory - balance & proprioception
what is the 1st sign of autonomic neuropathy?
erectile dysfunction
sx of autonomic neuropathy
orthostatic hypotension
arrythmias
severe constipation, urinary retention
erectile dysfunction
abnormal sweating
early satiety, lightheaded with meals
what are the rapidly progressive inflammatory demyelinating polyneuropathies?
AIDP
Guillain-Barre
what are the slowly progressive inflammatory demyelinating polyneuropathies?
CIDP
weakness progressing > 2 months
how does acute inflammatory demyelinating polyneuropathy progress?
- tingling, paresthesias w/o sensory loss
- severe radicular pain
- weakness (starts in legs –> up)
gets worse over 4 weeks then plateus
no B/B involvement
where is the most common area of weakness in acute inflammatory demyelinating polyneuropathy?
face
what labs are elevated with acute inflammatory demyelinating polyneuropathy?
protein
T/F: steroids are beneficial for Guillain Barre
F
Guillain Barre is a demyelinating ____ illness
monophasic
which fibers are lost with chronic inflammatory demyelinating polyneuropathy?
large motor
sx of chronic inflammatory demyelinating polyneuropathy
motor loss
symmetric weakness proximal AND distal
HYPO and Areflexic
sensory loss in LE (not disabling)
T/F: diabetic neuropathy should NOT cause weakness
T
are UEs or LEs affected first with diabetic neuropathy?
LE
T/F: the pain and numbness can be treated for diabetic neuropathy
F: just pain
presentation of diabetic lumbosacral radiculoplexus neuropathy (DLRPN) aka diabetic amytrophy
begins abrupt
sharp or aching pain asymmetrically in hip and thigh —> leg and foot
pain –> weakness thigh and knee –> weakness progresses distally
often becomes bilateral
Charcot-Marie-Tooth Disease clinical manifestations
distal weakness and atrophy
length dependent sensory loss
foot deformities
absent or decreased reflexes
chronic slowly progressive
when is Charcot-Marie-Tooth Disease most commonly onset?
children
Lambert Eaton is a ___synaptic disorder
PRE
Myasthenia gravis is a ____synaptic disorder
POST
diff dx/hallmark of MG
fluctuating, fatigable weakness especially of bilateral eyes that has a quick improvement with rest
myopathies usually have ___ weakness
proximal
polymyositis and dermatomyositis presentation
subacute/chronic onset of prox weakness
variable cardio and pulm involvement
d/t malignancy, autoimmune disorders, HIV
1st line of treat for dermatomyositis
steroids
2nd line of treatment for dermatomyositis
mild chemo
3rd line of treat dermatomyositis
usually related to cancer - chemo
how does weakness present in inclusion body myositis?
proximal leg and distal arm
T/F: steroid help with inclusion body myositis
F: none
what is the most common myopathy in adults?
myotonia
which muscular dystrophy is common in children?
Duchene & Becker’s
