Amyotrophic Lateral Sclerosis❗️❗️

Question 1

Etiology of ALS

Answer 1

• unknown cause
• progressive, degenerative disease in which the motor neurons in the brain, spinal cord, and peripheral system are destroyed and replaced by scar tissue → progressive muscle atrophy

Question 2

SSx of ALS

Answer 2

Rapidly progressive weakness, muscle atrophy and fasciculations (LMNL signs)

Muscle spasticity and hyperreflexia (UMNL signs)

Difficulty speaking (dysarthria) and difficulty swallowing (dysphagia)
due to cranial nerve involvement (CN IX, X, XII)

• Bulbar palsy: oral motor dysfunction due to weakness or paralysis of muscles of tongue, larynx and pharynx

Decline in breathing ability due to respiratory muscle weakness

Significant pain due to MSK related issues

Question 3

Stages of ALS

Answer 3

Stage I: can walk, is independent with ADLs, and has some weakness.

Stage II: can walk and has moderate weakness.

Stage III: can walk but has severe weakness.

Stage IV: requires a wheelchair for mobility, needs some assistance with ADLs, and has severe weakness in the legs.

Stage V: requires a wheelchair for mobility, is dependent for ADLs, and has severe weakness in the arms and legs.

Stage VI: unable to get out of bed and is dependent for ADLs and most self-care tasks.

Question 4

Information collected during Ax

Answer 4

• Gross Motor (ROM, MMT)
• Fine motor (Nine hole peg test, Purdue pegboard)
• Balance (Berg balance)
• Cognitive screening (MMSE, MoCA, Trails A&B)
• Fatigue (Modified Fatigue Impact Scale)
• Depression (Beck depression Inventory)
• Functional performance (BI, FIM, COPM)
• Home evaluations and home safety assessments
• Reevaluations as needed as the disease progresses

Question 5

Occupational performance issues

Answer 5

1. Decreased mm strength, ROM
2. Impaired balance, gait disturbance
3. decreased cognitive ability
4. Fatigue
5. Difficulty in workplace
6. Dysphagia
7. Communication difficulties
8. Psychological issues

Question 6

Tx goal and Tx plan (Decreased mm strength, ROM, Impaired balance, gait disturbance)

Answer 6

1. Safety, including positioning, transfers, and skin integrity

i. High backed and reclining; lightweight; small turning radius; and supports the head, trunk, and extremities.
ii. Later stage: consider a power wheelchair with adaptable controls that is easily maneuvered with tilt or recline and head, trunk, and extremity support

2. Exercise, AROM–>PROM (progressively decline)

Question 7

Tx goal and Tx plan (Communication difficulties)

Answer 7

• Augmentative communication equipment e.g. computerized communication device

Question 8

Tx goal and Tx plan (dysphagia)

Answer 8

1. Minimize distractions during mealtime.
2. Adapt food consistency (thicken liquids, downgrade diet to soft foods, in conjunction with the speech-language pathologist).
3. Use manual swallowing techniques.
4. Allow sufficient time for meals.
5. Ensure that nutritional needs are met, and refer to health care provider for alternatives to feeding if issues arise (e.g., feeding tube)

Question 9

Tx goal and Tx plan (Difficulty in workplace)

Answer 9

Allow work to continue as the disease progress

• sit while working
• w/c accessible, ergonomic work station
• medication/high energy time –> energy-demanding task
• adapt work hour
• work remotely
• chunking: break down task in manageable steps

Question 10

Tx goal and Tx plan (Psychological issues)

Answer 10

Self management (teach before cognitive decline)
- regulate behaviors, emotion, thoughts –> identify problem, brainstorm solutions and execute)

Support group