ALS Flashcards
explain ALS
fatal, motor neuron disease that leads to degeneration and loss of motor neurons in the spinal cord, brainstem, and brain
pt presents with signs of UMN, LMN, or both?
both
this is a hallmark sign
is limb onset ALS more common than bulbar onset?
limbs: 70-80%
bulbar: 20-30%
what causes ALS?
etiology unknown unless inherited
anterior horn cells are affected along with which cranial nerves? (5)
V: trigeminal
VII: fascial
IX: glossopharyngeal
X: vagus
XII: hypoglossal
Is sensation spared or affected in pts with ALS
SPARED; minimal axon atrophy
explain the progression and spread of denervation of ALS
spread throughout a region before moving to diff region
Cervical –> bulbar faster then rostral to caudal
clinical manifestation of ALS includes (focal/regional) and (asymmetrical/symmetrical) muscle weakness
focal; asymmetrical
3 cardinal signs of ALS
muscle weakness
LMN symptoms
UMN symptoms
what are signs of bulbar involvement in ALS (3)
dysarthria
dysphagia
sialorrhea
what % of vital capacity is assoc. w respiratory symptoms?
50%
what % of vital capacity requires ventilation and can lead to resp. failure?
<25-30%
pt with ALS present w. decline in cognitive function 35% will exhibit what type of dementia?
frontotemporal dementia
(unusual behaviors, emotional problems, trouble communicating, difficulty with work, or difficulty with walking)
what are 3 things that are often spared with ALS?
sensation
ocular muscles
sphincter m for bowel & bladder
T/F 50% of pts with ALS present with pseudo-bulbar affect
T
what 4 things must be confirmed to diagnose ALS?
LMN signs
UMN signs
progression within a region or multiple regions
other disease are ruled out
what criteria points to Possible ALS?
LMN and UMN signs only in 1 region
LMN + UMN in 2 regions indicates
probable ALS
if pt has LMN + UMN in 3 regions this indicates
definitive ALS
life expectancy of pts with ALS and age of diagnosis?
2-4years but can live up to 15yrs; usually in mid 50s
most common cause of death from ALS is a result of?
respiratory failure
what is a strong predictor of better survival rate
onset at age <35-40
(better 5 yr survival rate)
what correlates with 6.8x inc of death in <5yrs?
psychological distress
There is no cure for ALS, however, what is the only available drug that can extend life by 2-3mo?
Riluzole
Glutamate inhibitor
