Reference — Neurology
ALS & Huntington’s Disease Reference
Two progressive, ultimately fatal neurodegenerative diseases with opposite signatures: ALS destroys the motor neurons while leaving the mind intact, and Huntington’s — inherited and predictable — brings chorea alongside cognitive and psychiatric decline. Each reshapes nursing care around a different core threat.
Educational use only. Disease-modifying options are limited and specialist-directed; care is largely supportive and palliative. Genetic testing and counseling for Huntington’s carry significant implications and belong with trained counselors. This material supports nursing education and exam review. It is not medical advice and is not a substitute for clinical judgment, institutional policy, or medical direction. Always follow facility protocols and current provider orders.
Side by Side
| Feature | ALS (Lou Gehrig’s) | Huntington’s disease |
|---|---|---|
| What it is | Progressive degeneration of upper AND lower motor neurons | Inherited (autosomal dominant) degeneration of the basal ganglia |
| Inheritance / onset | Mostly sporadic; typically middle-older adulthood | Autosomal dominant — 50% risk to each child; onset usually 30–50 |
| Hallmark | Progressive muscle weakness, atrophy, fasciculations, spasticity; eventually swallowing and breathing | Chorea (involuntary jerky movements) + progressive cognitive decline + psychiatric changes |
| Cognition | Usually PRESERVED — the mind stays sharp as the body fails | Progressive dementia and personality/psychiatric change |
| Top nursing focus | Airway, aspiration, respiratory support, communication as speech fails | Safety (falls, injury from chorea), nutrition/aspiration, behavioral and family support |
| Course | Relentlessly progressive; respiratory failure is the usual cause of death | Progressive over ~10–20 years; fatal, often from complications |
Nursing Priorities
ALS: the airway is everything as the disease advances — monitor respiratory function, support secretion clearance, prevent aspiration, and plan ahead for ventilation and feeding decisions. Establish a communication method early, since speech is lost while the mind remains sharp. Mobility, skin, and contracture care, plus deeply honest goals-of-care and palliative conversations.
Huntington’s: safety from injury (chorea causes falls and trauma; pad the environment), nutrition and aspiration (chorea and dysphagia burn calories and risk choking — high-calorie modified-texture diet), and managing the behavioral and psychiatric symptoms (depression, irritability, impulsivity, high suicide risk) with structure and support. Family teaching includes genetic counseling given the 50% inheritance.
NCLEX Pearls
- ✦ALS destroys motor neurons but COGNITION STAYS INTACT — the trapped-in-a-failing-body picture; airway and communication are the priorities.
- ✦ALS death is usually from respiratory failure — plan ventilation and feeding decisions and palliative care early.
- ✦Huntington's is autosomal dominant — 50% risk to each child; offer genetic counseling.
- ✦Huntington's triad: chorea + cognitive decline + psychiatric changes; watch for high suicide risk.
- ✦Both are progressive and ultimately fatal — care is supportive and palliative; protect nutrition and prevent aspiration in both.
Related Resources
Standards & sources
Fact-checked Jun 21, 2026This page is written to align with American Heart Association / American Stroke Association (AHA/ASA) · American Association of Neuroscience Nurses (AANN). It is an educational summary, not a citation of any single document — always verify specific doses, values, and protocols against current guidelines and your facility policy. How we source content →