ALS — Amyotrophic Lateral Sclerosis

ALS destroys motor neurons while leaving the mind fully intact — the client watches their own body shut down. Knowing which functions are spared versus lost changes every nursing priority.

Amyotrophic lateral sclerosis is a progressive, fatal degeneration of both upper and lower motor neurons in the brain and spinal cord. Upper motor neuron signs include spasticity, hyperreflexia, and a positive Babinski. Lower motor neuron signs include muscle weakness, atrophy, fasciculations (visible muscle twitching), and flaccidity. The disease typically begins with asymmetric limb weakness or difficulty speaking/swallowing (bulbar onset). Progression is relentless — mean survival is 3–5 years from diagnosis, with respiratory failure as the primary cause of death. Critically, ALS spares cognition, sensation, vision, hearing, and bowel/bladder function (autonomic neurons are largely unaffected). The client is cognitively aware throughout. Riluzole is the only oral medication shown to modestly extend survival (by approximately 2–3 months) and works by reducing glutamate excitotoxicity. Nursing priorities center on airway management, aspiration prevention, nutritional support (often requiring PEG tube as dysphagia worsens), communication strategies as speech declines, and early advance directive discussions while the client can still communicate preferences. Serial pulmonary function tests — especially forced vital capacity (FVC) — guide decisions about noninvasive ventilation (BiPAP) and eventual tracheostomy.

Key Distinctions

Don't confuse ALS with myasthenia gravis: MG features fluctuating weakness that improves with rest and anticholinesterase drugs; ALS weakness is progressive, irreversible, and never improves. Don't confuse ALS with Guillain-Barré: GBS is ascending, often reversible, and typically post-infectious; ALS has mixed upper and lower motor neuron signs with no recovery. Students often assume ALS affects cognition — it does not. The mind stays intact, making psychosocial support and communication planning essential.

Clinical Pearl

ALS locks the body but not the brain. If the client can't speak, they can still think, hear, and decide — protect their autonomy with communication tools early.