ALS Flashcards
what is the pathology of ALS
progressive degeneration and loss of motor neurons in the spinal cord, brainstem, and motor cortex
compare structures lost in MS vs ALS
ALS = sensory and oculomotor CNs spared
MS - neither are spared
what is the breakdown of amyotrophic lateral sclerosis
muscle malnourishment due to scarring in the spinal cord where LMN are located
what areas of the spinal cord are effected by ALS
anterior horn
lateral CST
what CN nuclei in the brainstem is ALS related degeneration seen in
5,7,9,10,12
what is the incidence of ALS in relation to
- age
- gender
mid to late 50s
men > women
explain familial ALS? what can early age of onset lead us to?
5-10% of individuals have an inherited trait leading to ALS
younger onset can indicate genetic mutations
what are known risk factors for ALS
specific gene mutation
gender
family history
age
what are possible risk factors for ALS
lifestyle
neurotoxic exposure
trauma
diet
vigorous physical activity
occupation
explain clinical manifestations of ALS in relation to initial symptoms and percentages of cases associated with each
limb onset = 70-80%
bulbar/brainstem onset = 20-30%
explain initial onset s/s of ALS
asymmetrical and focal weakness in the distal LE or UE
or
bulbar muscle weakness like swallowing / speech
what muscle group is common in early stages of ALS
cervical extension weakness
what is the time frame from onset to death in those with ALS
27-43 months
major differences between ALS and other neurodegenerative diseases when comparing S/S
both UMN and LMN
- as well as bulbar
what are the most common bulbar s/s in those with ALS
dysarthria
dysphagia
sialorrhea
why does dysarthria/dysphagia occur
spastic/flaccid palsy causing weakness in:
tongue
lips
jaw
larynx
pharynx
why does sialorrhea occur
absence of automatic swallowing of excessive saliva
weakened lower facial muscles
what respiratory impairments are common in ALS
dyspnea on exertion
orthopnea
frequent wakening
___% of this predicted respiratory capacity value indicates _____ in those
Vital Capacity of <20-30% of predicted indicates risk of respiratory failure/death
what cognitive impairments are considered part of the ALS disease spectrum
frontotemporal dementia
what general cognitive issues are seen with ALS
impaired:
executive function
planning
organizing
personality / behavior changes
language comprehension
memory
reasoning / abstract reasoning
what are disease modifying agents for ALS
glutamate inhibitor
neuroprotective agents
what do glutamate inhibitors do in relation to prognosis
extend survival by 2-3 months
how is diagnosis of ALS completed
history
neuro exam
EMG / neuroimaging
muscle/nerve biopsies
genetic testing
for examination purposes, why would functional tests/measures be applicable vs non-applicable in the ALS population
would be used to justify medical necessity for assistive devices
– wouldn’t want to use them as benchmarks because of rapid progression of the disease
what is to be considered during initial exam for those with ALS
pt’s goals –> functional baseline
rate of progression
extent of involvement
stage of disease / education of prognosis
what is the ALSFRS-R
ALS functional rating scale revised
what does the ALSFRS-R consist of
bulbar involvement (SLP things)
fine motor tasks
ADLs
functional mobility / transfers
respiratory involvement
early in the ALS progression, what form of intervention strategy is applicable
preventative and restorative
– may be compensatory depending upon functionality
in the middle of ALS progression, what form of intervention strategy is applicable
compensatory and preventative
– may have restorative
late in the ALS progression, what form of intervention strategy is applicable
compensatory and preventative
– may have restorative
when considering exercise programming for those with ALS, what is best to consider
current functionality / progression and implementation of moderate to low intensity
- do not want to carry it to point of fatigue or overuse
what muscle types are more susceptible to overuse fatigue in those with ALS
weak/denervated
what is recommended for those with LE muscle weakness? why?
orthoses
- add support to muscles as well as decrease stress to surrounding tissue
what is important to understand when considering assistive device interventions in those with ALS
we need to anticipate equipment needs and be proactive
what does an ATP do? when would collaboration be necessary?
adaptive technology professional
- when writing letters of medical necessity they will understand all the options on the market for specific deficits
when addressing respiratory muscle weakness, what interventions can PTs do
education regarding
positioning during feeding
s/s of aspiration
manually assisted cough (via caregiver)
when addressing decreased mobility, what interventions can PTs do
environment modification
WC evaluation
caregiver education on mobility assistance / mechanical lift technique
when addressing muscle cramps and spasticity, what interventions can PTs do
gentle massage and stretching
PROM
splinting
