Neuro ICU > ALS

Amyotrophic Lateral Sclerosis (ALS)

What is it?

  • ALS is a“progressive degeneration of motor neurons on the brainstem, anterior horn cells of the spinal cord, and the motor cortex that eventually affects all voluntary muscles” (Hamby, 2024). The onset of ALS typically occurs between the ages of 40 and 70 (the average age is 55). It is 20% more common in men. However, as age increases, the incidence equalizes between genders.

Types

  • Sporadic: 90-95% of cases occur randomly without a genetic cause or family history

  • Familial: a small percentage of people are thought to inherit ALS

How is it diagnosed?

  • Generally, a diagnosis of exclusion. The patient must have both upper and lower motor neuron symptoms. Electromyography (EMG) and muscle/nerve biopsy are used.

Symptoms & Stages

  • The onset of symptoms is generally gradual, and initial symptoms can vary. During the early stage, patients experience muscle weakness and/or stiffness with progressive weakness and paralysis to follow. For each patient, the order of symptoms and rate of disease progression will vary.

    • Gradual onset of progressive muscle weakness is the most common first symptom. Other early signs include: tripping, dropping things, abnormal fatigue, slurred speech, muscle cramps, muscle twitching, and/or uncontrolled periods of laughing or crying.

    • Once breathing is affected, ventilatory support will be required.

Treatment

  • There is no cure for ALS, and treatment is primarily focused on symptom management and supportive care

  • Riluzole and edaravone are FDA-approved medications that may slow disease progression modestly, though neither is curative.

Life Expectancy

  • The average life expectancy after diagnosis is 3 years, with overall life expectancy varying based on age and symptoms present at diagnosis. ~20% live for 5 years, 10% for 10 years, and 5% live for 20+ years.

Why ALS Patients Are in the ICU

ALS patients are typically admitted to the ICU for one or more of the following:

  • Respiratory failure — as diaphragm and respiratory muscles weaken, non-invasive ventilation (BiPAP) or invasive mechanical ventilation may be required. This is often a pivotal decision point in the disease course.
  • Aspiration pneumonia — bulbar dysfunction leads to dysphagia and aspiration risk. SLP involvement is critical.
  • Tracheostomy or PEG placement decisions — ICU admission often coincides with goals-of-care conversations about ventilatory support and feeding tube placement.
  • Intercurrent illness — infections or other acute events can accelerate functional decline in ALS patients.

Goals-of-care conversations are central to ALS ICU management. Rehabilitation teams play an important role in supporting function and quality of life within the patient's expressed wishes — not in reversing the disease course.

Cognitive and Behavioral Involvement

In approximately 40–50% of patients with ALS, cognitive, behavioral, or language deficits will be present. Executive dysfunction is typically the first sign of cognitive impairment. In 10–15% of patients, deficits are severe enough to meet criteria for frontotemporal dementia (ALS-FTD). Memory deficits are uncommon until late-stage disease.

Caregivers report that cognitive and behavioral deficits cause more anxiety and caregiver burden than physical deficits. Common behavioral changes include apathy, compulsive behaviors, and social cognition changes (difficulty recognizing emotions, impaired humor comprehension, social faux pas).

Implications for therapy: Identify cognitive and behavioral deficits early. Adapt communication strategies, provide memory aids, and involve family in strategies to mitigate behavioral changes. Educate nursing staff on the presence of and strategies to address cognitive and behavioral deficits.

ALS Nonmotor Symptoms — Prevalence

(Hamby, 2024)

Depression and low mood — 92%
Drooling — 52%
Fatigue — 82%
Cognitive deficits — 40–50%
Pseudobulbar affect — 68%
Suicidal ideation — 34%
Pain — 57%
Unexplained sensory symptoms — 30%
Weight loss — 56%
Sleep disorders — common

These symptoms are frequently undertreated. Identifying and communicating them to the medical team improves patient comfort and quality of life.

Therapy Implications

  • Align goals with the patient's wishes and goals of care. Therapy in ALS is supportive and adaptive — not curative. Establish what matters most to the patient early. Request a palliative care consult if symptom management outweighs functional benefits or pain control is inadequate.

  • Do not overfatigue the patient. Exercise is submaximal. The program is too aggressive if the patient has pain or is still fatigued 30 minutes after the session such that ADLs are limited. Exercise emphasis is on preventing decline in muscle endurance for as long as possible:

    • Provide PROM or AAROM depending on the patient's abilities — avoid resistive exercises

    • Avoid eccentric exercises; do not exercise muscles that lack antigravity strength

    • Ensure the patient can still talk during exercise — if they can't, it's too intense

    • Allow adequate recovery time after exercises

    • Upper limb weakness typically begins in the dominant hand with thenar muscle involvement first (first interosseous and finger extensors before flexors)

  • Energy conservation is central. Prioritize high-value activities; use adaptive equipment to reduce demand (universal cuff, built-up handles, wrist support, mobile arm supports, environmental control units).

  • AAC (Augmentative and Alternative Communication) assessment is a critical OT/SLP role. Begin before speech is significantly impaired — options range from low-tech letter boards to high-tech eye-gaze systems. Preserving communication preserves autonomy.

  • Splinting and hand management: Consider nighttime hand splinting to prevent skin breakdown, maintain palmar arch, reduce pain, and improve joint integrity. Anti-claw splints can improve grasp. Short opponens splints may improve pinch.

  • Cognitive and behavioral deficits (present in 40–50% of patients) require adaptation of therapy approaches: use visual cues and calendars, simplify instructions, involve family in strategies, and educate staff on behavioral management.

  • Psychosocial and safety: Suicidal ideation affects approximately 34% of patients. Depression affects up to 92%. Screen sensitively and involve social work and psychology. Quality of life is generally rated higher by patients than caregivers would expect — the patient's own assessment should anchor goals.

  • Caregiver and family education is a primary therapy role — include DME needs, body mechanics, safe transfer techniques, positioning and ROM strategies, communication strategies, and precautions (skin integrity, do not overfatigue). Education should be hands-on and include written handouts.

  • Defer vigorous activity during acute illness or respiratory compromise. Short, low-demand sessions focused on function and quality of life are most appropriate in the ICU setting.

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References

Hamby, J. R. (2024). The nervous system, part 2: Neurodegenerative diseases and other conditions. In H. Smith-Gabai & S. E. Holm (Eds.), Occupational Therapy in Acute Care (3rd ed., pp. 449–496). AOTA Press.

National Institute of Neurological Disorders and Stroke. (2021, November 15). Amyotrophic Lateral Sclerosis (ALS) Fact Sheet. https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Amyotrophic-Lateral-Sclerosis-ALS-Fact-Sheet