Amyotrophic Lateral Sclerosis (ALS)

ALS

Progressive neurodegenerative disease that attacks motor neurons in the brain and the spinal cord resulting in muscle

What underlying process leads to progressive motor neuron loss in ALS?

Degeneration linked to excess glutamate; motor neurons fail to activate muscles, causing atrophy.

Which systems are typically NOT affected by ALS?

Eye muscles; bowel/bladder external sphincters; internal organs; sexual function; the 5 senses; Mind and mental abilities (infrequent)- Recent research shows associations with dementia

What are the symptoms of ALS?

Weakness in limbs or bulbar muscles

Fasciculations and cramps (hands/feet)

Dysarthria (‘thick speech’)

Progressive dyspnea/dysphagia in advanced stages

Onset age of ALS?

55-70

Who gets ALS?

Anyone, 60% are men

ALS classification?

Sporadic (90-95%)

Familial (5-10%)

What is the prognosis of ALS?

Mean survival 3–5 years

30% >5 years

10–20% >10 years; rare remissions/misdiagnoses possible

What is the primary cause of death in ALS?

Respiratory failure as respiratory muscles weaken.

How is ALS diagnosed?

SERIES OF DIAGNOSTIC TESTS:

EMG

Blood and urine studies

Spinal tap

X-rays, MRI

Myelogram of cervical spine

Muscle and/or nerve biopsy

Thorough neurological examination

What medications are used to treat ALS?

Riluzole (Rilutek)

Riluzole (Rilutek)

Believed to reduce damage to motor neurons by decreasing the release of glutamate.

Lengthens survival by several (3) months.

Extends the time before needing ventilation support.

May have a greater survival benefit for those with a bulbar onset (speech and swallowing).

Adaptive devices and techniques for the beginning stages of ALS.

Button hook

Reacher

Key holder

Cane

Electric toothbrush

Hand‑held shower

Grab bars

Pullover shirts

Elastic waist pants

Slip‑on shoes

Velcro closures

Adaptive devices and techniques for the middle stages of ALS

Walker with wheels and hand brakes

Light-weight folding wheelchair to put in the car

Reclining chair with footrest

Chair with an electric rising seat or lift

Transfer board

Raised toilet seat

Shower chair

Urinal

Orthotics/Splinting

-Neck collar

-Ankle-foot splints

-Hand splints

Adaptive devices and techniques for the advanced stages of ALS

Electric bed

Highback wheelchair with headrest that reclines and has removable arms

Leg rests

Lap tray

Bedside commode

Hydraulic lift (manual versus powered)

Foot support boot

Arm supports

Suction machine

Communication devices

Durable medical equipment typically covered by Medicare?

Walker/cane OR wheelchair; hospital bed; bedside commode; manual Hoyer lift + sling; partial payment for power lift chair motor; pressure‑relief wheelchair cushion; alternating pressure pad; power wheelchair with tilt/recline/elevating legs and alternate controls (with documentation).

Commonly NOT covered equipment?

Shower chair, tub bench, feeding/dressing aids, raised toilet seat, grab bars, electric Hoyer, ceiling lift, exercise equipment, overbed tables, oscillating bed, catheters.

OT priorities across ALS progression?

Maintain function and safety, conserve energy, support communication and access, adapt environment/equipment, caregiver training, plan for progression and respiratory decline.

Early‑stage OT interventions (ALS)?

Energy conservation; ADL simplification; hand function supports (built‑up handles, button hooks); falls prevention; home mods; worksite adaptations.

Middle‑stage OT interventions (ALS)?

Transfer training with boards/lifts; seating/positioning; splinting; bathroom safety (shower chair, raised seat); mobility aids; communication access; caregiver instruction.

Late‑stage OT interventions (ALS)?

Power mobility with optimal supports; pressure management; environmental control units/AT; communication devices; safe transfers with lifts; caregiver body mechanics; hospice collaboration

OT role in respiratory considerations (ALS)?

Positioning to ease breathing; conserve energy during ADLs; coordinate with RT about timing of activities and ventilatory support; educate on signs of fatigue/distress

Caregiver training priorities (ALS)?

Safe transfers, equipment use, pressure relief, communication supports, emergency plans, pacing and respite strategies.