QUIZ 3 NSG 555 NEURO MODULE 7 + HEM MODULE 8
QUESTIONS WITH COMPLETE VERIFIED ANSWERS GRADED A+
+
ALS
lou gehrig's disease
incurable progressive muscle weakness and eventually death
affects upper motor neurons and lower motor neurons.
age 55-75 is typical onset
sporatic, 5-10% familial. thought to be known gene mutation
pathophysiology of ALS
unknown. gene mutation SOD1 or C9orf72
may be due to oxidative stress/free radicals. eventually leads to denervation and
atrophy of muscles b/c motor neurons in brainstem and spinal cord die
presentation of ALS
neuro exam is essential.
ASYMMETRIC weakness in limbs first (usually arms)
foot drop, difficulty walking, weakness in liftin garms.
UMN (upper motor neuron) s/s: hyperreflexia, sapsticity, babinski signs, incoordination
,and weakness
LMN (lower motor neuron) s/s: weakness, atrophy, fasciculations (twitching)
BULBAR s/s ALS: dysarthria, dysphagia, siallorhea, tongue atrophy and fasciculations
dementia may appear first.
bowel and bladder are spared
dx ALS
widespread UMN, LMN s/s and absense of any other disease process, absence of
neuroimaging evidence of other disease.
should get labs to rule out heavy metal poisoning, lyme disease, HIV, hereditary d/o.
MRI, ESR, protein, immunofixation electrophoresis, CSF analysis, b12, CK, EMG, nerve
cond. studies and MRI, chem, liver, thyroid tests
awaji-shima criteria
criteria for ALS diagnostics
EMG+clinical abnormalities (s/s LMN degeneration by clinical, electrophys and
neuropath exam, s/s UMN by clinical exam, progression of motor syndrome within a
region)
riluzole
for ALS, improves survival
antiglutamate slows progression of ALS
, s/e: asthenia, dizzy, gi distress, low WBC, elevated liver enzymes (check labs first and
then q 3mos)
Edaravone
free radical scavenger to reduce oxidative stress: decreases functional decline so
reduces symptoms progression)
INFUSION daily X14 days, then 14 days off, then daily X 10 days, 14 off, then repeat
s/s: contusion from injection, HA, gait change
SSRI, SNRI for ALS
improve depression, anxiety, and sleep s/s
SARI (trazadone). can improve emotional lability. Benzos may also be used and may
help with spasticity and muscle cramps
anticholinergics for ALS
TCAs, glycopyrrolate, atropine
for sialorrhea (saliva increase), depression, anxiety, sleep and pain
other meds for ALS
ALS: muscle relaxants (tizanidine, baclofen), dextromethorphan/quinidine for emotional
lability
modafinil--stimulant for fatigue
oxybutynin--bladder spasms
Bells Palsy
QUESTIONS WITH COMPLETE VERIFIED ANSWERS GRADED A+
+
ALS
lou gehrig's disease
incurable progressive muscle weakness and eventually death
affects upper motor neurons and lower motor neurons.
age 55-75 is typical onset
sporatic, 5-10% familial. thought to be known gene mutation
pathophysiology of ALS
unknown. gene mutation SOD1 or C9orf72
may be due to oxidative stress/free radicals. eventually leads to denervation and
atrophy of muscles b/c motor neurons in brainstem and spinal cord die
presentation of ALS
neuro exam is essential.
ASYMMETRIC weakness in limbs first (usually arms)
foot drop, difficulty walking, weakness in liftin garms.
UMN (upper motor neuron) s/s: hyperreflexia, sapsticity, babinski signs, incoordination
,and weakness
LMN (lower motor neuron) s/s: weakness, atrophy, fasciculations (twitching)
BULBAR s/s ALS: dysarthria, dysphagia, siallorhea, tongue atrophy and fasciculations
dementia may appear first.
bowel and bladder are spared
dx ALS
widespread UMN, LMN s/s and absense of any other disease process, absence of
neuroimaging evidence of other disease.
should get labs to rule out heavy metal poisoning, lyme disease, HIV, hereditary d/o.
MRI, ESR, protein, immunofixation electrophoresis, CSF analysis, b12, CK, EMG, nerve
cond. studies and MRI, chem, liver, thyroid tests
awaji-shima criteria
criteria for ALS diagnostics
EMG+clinical abnormalities (s/s LMN degeneration by clinical, electrophys and
neuropath exam, s/s UMN by clinical exam, progression of motor syndrome within a
region)
riluzole
for ALS, improves survival
antiglutamate slows progression of ALS
, s/e: asthenia, dizzy, gi distress, low WBC, elevated liver enzymes (check labs first and
then q 3mos)
Edaravone
free radical scavenger to reduce oxidative stress: decreases functional decline so
reduces symptoms progression)
INFUSION daily X14 days, then 14 days off, then daily X 10 days, 14 off, then repeat
s/s: contusion from injection, HA, gait change
SSRI, SNRI for ALS
improve depression, anxiety, and sleep s/s
SARI (trazadone). can improve emotional lability. Benzos may also be used and may
help with spasticity and muscle cramps
anticholinergics for ALS
TCAs, glycopyrrolate, atropine
for sialorrhea (saliva increase), depression, anxiety, sleep and pain
other meds for ALS
ALS: muscle relaxants (tizanidine, baclofen), dextromethorphan/quinidine for emotional
lability
modafinil--stimulant for fatigue
oxybutynin--bladder spasms
Bells Palsy
