ALS pt. 2 Flashcards
classical ALS more common in who
men
2/3 of ALS population
classical ALS involves what
UMN and LMN
either limb onset or bulbar onset
early signs of classical ALS
progressive limb weakness, reduced dexterity, wasting of hands, muscle fasciculations
if bulbar - wasting of the tongue and muscles for speech and swallowing
limb onset first symptoms are due to what
result from damage to motor neurons in motor cortex - corticospinal
limb onset first symptoms look like what
distal to proximal
fasciculations, atrophy, weakness, spasticity
bulbar onset progression
faster than limb onset
bulbar onset first symptoms are due to what
damage to motor neurons in brainstem for corticobulbar tracts
bulbar onset first symptoms are due to what
CN IX to XII causing dysarthria and dysphagia
tongue atrophy, tongue fasciculations
vocal cord spasms
bulbar onset is more common in who
older females
primary lateral sclerosis
UMN SxS only
can progress to classical
key examination findings for primary lateral sclerosis include
spasticity
UMN pattern weakness
pseudobulbar findings
prognosis for primary lateral sclerosis
relatively good with life expectancy
progressive spinal muscular atrophy mainly affects who
men
21 y/o
progressive spinal muscular atrophy (SMA IV)
LMN only
mainly limb
may limit to one region
SMA IV progression
variable - slow or rapid
SMA IV survival rate
if slow progressed - 5-10 yrs
survival duration 12 months longer
flail arm syndrome
SMA IV
cervical
man in a barrel
flail leg syndrome
SMA IV
lumbosacral
how common is ALS with frontotemporal dementia
20-30% show cog symptoms
what does the clinical course look like with ALS with FTD
more rapid
familia ALS
combined effects of multiple predisposing + environmental & random effects leading to disease onset
familia ALS onset
late forties or early fifties
