Amyotrophic Lateral Sclerosis (ALS) Flashcards
Though etiology is uncertain, increased levels of ____ is thought to clog up the synaptic cleft and suffocate what structure?
glutamate, alpha motor neurons
what is the average mortality for individuals dx with ALS?
3-5 years
the pathogenesis of ALS can be best described as a progressive degeneration/ loss of ____ in the SC, ____, and motor cortex.
loss of motor neurons, brain stem
T/F: Because ALS targets the anterior horn cells and descending corticoobulbar/corticospinal tracts, it’s a solely LMN pathology.
false. it also impacts UMN cell bodies in the motor cortex. but LMN»UMN weakness.
In addition to familial ALS that is autosomal dominant and makes up ___% of cases, additional RF include (5)
- 5-10%
- gender (M>F), age (55-75), race, geographic clusters, prior trauma/TBI
ALS is largely a dx of exclusion, but what diagnostic tools might help lead to its discovery? (4)
clinical examination
EMG/NCV (LMN impact)
MRI
CSF/ blood urine tests (can help rule out other neurologic ocnditions)
Clinical dx of ALS requires what 2 patterns?
-both UMN and LMN symptoms
-persistent decline in physical fn unexplained by other dx.
The 2 Criteria systems used to dx ALS are ___ and __. The main difference between them is
El Escorial Criteria for Dx & The Gold Coast Criteria for Dx of ALS.
the latter has more broadly defined regions (blubar/UE/LE vs bulbar/spinal levels)»_space; more sensitive and aids in IDing clinical trial candidates.
El Escorial Criteral for Dx of ALS leads to ___
suspected, possible, probable, and definitive ALS
The Gold Coast Criteria for Dx of ALS leads to
+ or - ALS
T/F The cardinal sign of ALS is muscle weakness and we often see extraoccular muscle, bowel, and bladder involvement as early indicators.
False. These are normally spared until terminal stages.
Fatigue, dyspnea on exertion, frequent sighing and morning HA are early s/s of respiratory involvement. They usually being to show up when VC reaches about __%. A decrease to ___% poses significant risk of repiratory failure or death
50%; 25-35%
Primary impairments related to ALS are:
* UMN/LMN/ bulbar motor dysfunction.
* respiratory impairments
* cognitive deficit (esp bulbar)
* and_____(3)
pain
fatigue
and ANS symptoms
The main clinical subtypes of ALS include classical ALS, ALS w/ frontotemporal dementia, familial ALS, and ___(2)
Primary Lateral Sclerosis
Progressive Spinal Muscular Atrophy (SMA)
T/F: In cases of limb-onset classical ALS, we might expect motor s/s such as fasciculations, weakness/atrophy and spasticity to appear in the calf and progress to the toes (following the direction of motor signals) .
false. symptoms progress distal>proximal
