ALS Flashcards
what is the etiology of ALS?
not well understood
The pathology of ALS may be located where? (5)
- motor cortex
- corticobulbar pathway
- cranial nerve nuclei (brainstem)
- SC
- anterior horn cells
define the condition associated with ALS in the corticobulbar pathway
pseudobulbar palsy - UMN PATHOLOGY - spasticity and poor control over face/mouth/throat muscles as well as emotional lability
define the condition associated with ALS in the cranial nerve nuclei (brainstem)
progressive bulbar palsy - LMN PATHOLOGY - flaccid, atrophied face/mouth/throat muscles without change in affect
Re-innervation can compensate for progressive degeneration until motor unit loss is ~__%
50%
Where can degeneration occur in Primary Lateral Sclerosis?
cortex and CST (UMN pathology)
which is more aggressive, ALS or PLS?
ALS
where can degeneration occur in progressive bulbar palsy
CN IX-XII (LMN pathology)
Where do we typically see degeneration in Progressive Muscular Atrophy (aka SMA)
anterior horn cells (LMN) and brainstem (UMN)
ALS or PLS: stiffness
PLS
ALS or PLS: limb wasting
ALS
ALS or PLS: duration
PLS longer duration
ALS v PLS: mortality
ALS higher mortality
how do you differentiate ALS from PLS, primarily
PLS = spasticity without wasting x 3 years
Sxs of ALS depend on the pathology, but what are the five things that are typically spared?
- eye movement
- sensation
- bowel and bladder
- coordination
- cognition
cardinal sign of ALS
weakness (beginning distally and moving proximally)
T/F: ALS weakness is symmetrical
false: usually asymmetrical
LMN signs with ALS
fatigue, fasciculations, hyporeflexia, decreased tone, cramping
UMN signs with ALS
spasticity, hyperreflexia, pathological reflexes, and weakness
how do you dx ALS
primarily EMG, location of symptoms at multiple sites
EMG & NCV test demonstrate UMN or LMN disease?
EMG and NCV demonstrate LMN disease
what is the threshold for perceived weakness in ALS patients?
50% motor neuron loss
limb or bulbar onset of ALS leads to a poorer prognosis
bulbar onset leads to a poorer prognosis
median survival of ALS
4 years
