ALS Flashcards
ALS
amyotrophic lateral sclerosis
ALS is also known as
lou gehrigs dz
what is ALS
most common form of adult onset progressive motor neuron dz
may be the most devastating of the neurologic degenerative dz
hallmark of ALS
combo of UMN and LMN signs and symptoms
UMN signs
spasticity
hyperreflexia
pathological reflexes
where do UMN signs stem from
destruction of motor neurons int he brain
causing degeneration of the corticospinal and corticobulbar tracts
LMN signs
muscle wasting
weakness
fasciculations
muscle weakness
amyotrophy
LMN signs stem from
destruction of the anterior horn cells of teh SC and cell bodies of the motor CN nuclei
90% of the CST fibers
decussate at the medulla
descend in the SC as the lateral CST
lateral sclerosis
destruction and scarring of the lateral CST
classification
sporadic
familial
sporadic
the most common form of ALS in the US
90-95% of all cases
familial
occurring more than once in family lineage (autosomal dominant)
accounts for very small number of cases in the US (5-10%)
familial is caused by
genetic mutation in a gene encoding an enzyme called copper-zinc superoxide dismutase
initial S&S
limb onset ALS
bulbar onset ALS
limb onset ALS
75% of cases
limb onset ALS is
difficulty walking or difficulty with the fine motor coordination of UEs
bulbar onset ALS
25% of cases