ALS

Question 1

what is the pathology of ALS

Answer 1

progressive degeneration and loss of motor neurons in the spinal cord, brainstem, and motor cortex

Question 2

compare structures lost in MS vs ALS

Answer 2

ALS = sensory and oculomotor CNs spared

MS - neither are spared

Question 3

what is the breakdown of amyotrophic lateral sclerosis

Answer 3

muscle malnourishment due to scarring in the spinal cord where LMN are located

Question 4

what areas of the spinal cord are effected by ALS

Answer 4

anterior horn
lateral CST

Question 5

what CN nuclei in the brainstem is ALS related degeneration seen in

Answer 5

5,7,9,10,12

Question 6

what is the incidence of ALS in relation to
- age
- gender

Answer 6

mid to late 50s
men > women

Question 7

explain familial ALS? what can early age of onset lead us to?

Answer 7

5-10% of individuals have an inherited trait leading to ALS

younger onset can indicate genetic mutations

Question 8

what are known risk factors for ALS

Answer 8

specific gene mutation
gender
family history
age

Question 9

what are possible risk factors for ALS

Answer 9

lifestyle
neurotoxic exposure
trauma
diet
vigorous physical activity
occupation

Question 10

explain clinical manifestations of ALS in relation to initial symptoms and percentages of cases associated with each

Answer 10

limb onset = 70-80%
bulbar/brainstem onset = 20-30%

Question 11

explain initial onset s/s of ALS

Answer 11

asymmetrical and focal weakness in the distal LE or UE

or

bulbar muscle weakness like swallowing / speech

Question 12

what muscle group is common in early stages of ALS

Answer 12

cervical extension weakness

Question 13

what is the time frame from onset to death in those with ALS

Answer 13

27-43 months

Question 14

major differences between ALS and other neurodegenerative diseases when comparing S/S

Answer 14

both UMN and LMN
- as well as bulbar

Question 15

what are the most common bulbar s/s in those with ALS

Answer 15

dysarthria
dysphagia
sialorrhea

Question 16

why does dysarthria/dysphagia occur

Answer 16

spastic/flaccid palsy causing weakness in:
tongue
lips
jaw
larynx
pharynx

Question 17

why does sialorrhea occur

Answer 17

absence of automatic swallowing of excessive saliva

weakened lower facial muscles

Question 18

what respiratory impairments are common in ALS

Answer 18

dyspnea on exertion
orthopnea
frequent wakening

Question 19

___% of this predicted respiratory capacity value indicates _____ in those

Answer 19

Vital Capacity of <20-30% of predicted indicates risk of respiratory failure/death

Question 20

what cognitive impairments are considered part of the ALS disease spectrum

Answer 20

frontotemporal dementia

Question 21

what general cognitive issues are seen with ALS

Answer 21

impaired:
executive function
planning
organizing
personality / behavior changes
language comprehension
memory
reasoning / abstract reasoning

Question 22

what are disease modifying agents for ALS

Answer 22

glutamate inhibitor
neuroprotective agents

Question 23

what do glutamate inhibitors do in relation to prognosis

Answer 23

extend survival by 2-3 months

Question 24

how is diagnosis of ALS completed

Answer 24

history
neuro exam
EMG / neuroimaging
muscle/nerve biopsies
genetic testing

Question 25

for examination purposes, why would functional tests/measures be applicable vs non-applicable in the ALS population

Answer 25

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

Question 26

what is to be considered during initial exam for those with ALS

Answer 26

pt's goals --> functional baseline rate of progression extent of involvement stage of disease / education of prognosis

Question 27

what is the ALSFRS-R

Answer 27

ALS functional rating scale revised

Question 28

what does the ALSFRS-R consist of

Answer 28

bulbar involvement (SLP things) fine motor tasks ADLs functional mobility / transfers respiratory involvement

Question 29

early in the ALS progression, what form of intervention strategy is applicable

Answer 29

preventative and restorative -- may be compensatory depending upon functionality

Question 30

in the middle of ALS progression, what form of intervention strategy is applicable

Answer 30

compensatory and preventative -- may have restorative

Question 31

late in the ALS progression, what form of intervention strategy is applicable

Answer 31

compensatory and preventative -- may have restorative

Question 32

when considering exercise programming for those with ALS, what is best to consider

Answer 32

current functionality / progression and implementation of moderate to low intensity - do not want to carry it to point of fatigue or overuse

Question 33

what muscle types are more susceptible to overuse fatigue in those with ALS

Answer 33

weak/denervated

Question 34

what is recommended for those with LE muscle weakness? why?

Answer 34

orthoses - add support to muscles as well as decrease stress to surrounding tissue

Question 35

what is important to understand when considering assistive device interventions in those with ALS

Answer 35

we need to anticipate equipment needs and be proactive

Question 36

what does an ATP do? when would collaboration be necessary?

Answer 36

adaptive technology professional - when writing letters of medical necessity they will understand all the options on the market for specific deficits

Question 37

when addressing respiratory muscle weakness, what interventions can PTs do

Answer 37

education regarding positioning during feeding s/s of aspiration manually assisted cough (via caregiver)

Question 38

when addressing decreased mobility, what interventions can PTs do

Answer 38

environment modification WC evaluation caregiver education on mobility assistance / mechanical lift technique

Question 39

when addressing muscle cramps and spasticity, what interventions can PTs do

Answer 39

gentle massage and stretching PROM splinting