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Physiological AdaptationMotor neuron death, mind intact

Amyotrophic Lateral SclerosisNCLEX cheat sheet

Amyotrophic lateral sclerosis (Lou Gehrig's disease) is a progressive, fatal degeneration of upper and lower motor neurons, leading to muscle weakness, atrophy, and eventual paralysis.

19 key facts 5 practice questions Reviewed Oct 2026
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Mechanism

  • Upper + lower motor neuron loss
  • Progressive, fatal, no cure
  • Cognition/senses stay intact

Signs

  • Progressive muscle weakness/atrophy
  • Fasciculations, spasticity, cramps
  • Dysphagia, dysarthria late

Do

  • Riluzole slows progression
  • Aspiration precautions, PEG tube
  • Communication aids, support

Report

  • Resp failure = #1 death cause
  • Discuss advance directives early
Key takeaway

Progressive muscle weakness, atrophy, fasciculations (twitching), and spasticity occur as motor neurons die.

Before you read

Test yourself first: 5 questions

Question 1 of 5

A nurse reviews the clinical picture of a client with amyotrophic lateral sclerosis (ALS). Which combination of findings is consistent with this disorder?

What is amyotrophic lateral sclerosis?

Sensation, cognition, and eye movement are typically preserved, so the patient remains mentally aware throughout. There is no cure; death usually results from respiratory failure. Care is supportive and focused on maintaining function, safety, and dignity.

Amyotrophic Lateral Sclerosis illustrated

Amyotrophic Lateral Sclerosis — clinical diagram
Amyotrophic Lateral Sclerosis — clinical diagram. Illustration: Paul Wicks / Wikimedia Commons via Wikimedia Commons, CC BY-SA 4.0.

Key points to remember

  • Progressive muscle weakness, atrophy, fasciculations (twitching), and spasticity occur as motor neurons die.
  • Mental status, intelligence, and sensation remain intact, while voluntary muscle control is lost.
  • Dysphagia and dysarthria develop as bulbar muscles weaken, increasing aspiration risk.
  • Respiratory muscle weakness progresses to respiratory failure, the usual cause of death.
  • The disease is relentlessly progressive with no cure; riluzole may modestly slow progression.

Signs & symptoms

  • Progressive muscle weakness/atrophy
  • Fasciculations, spasticity, cramps
  • Dysphagia, dysarthria late

Nursing priorities

  1. Monitor respiratory function closely and support ventilation as the disease advances.
  2. Assess swallowing and implement aspiration precautions; anticipate enteral feeding as dysphagia worsens.
  3. Provide a communication method (boards, devices) as speech declines, since cognition stays intact.
  4. Prevent complications of immobility with repositioning, skin care, and range-of-motion exercises.
  5. Discuss and document advance directives and end-of-life wishes while the patient can communicate.

Patient teaching

  • The mind stays sharp even as the body weakens, so maintaining communication tools is important.
  • Plan early for advance directives, ventilation decisions, and feeding-tube preferences.
  • Use assistive and communication devices, energy conservation, and support services to maintain quality of life.

Red flags: report now

  • Increasing respiratory distress or declining vital capacity signals respiratory failure, the leading cause of death.
  • Choking, coughing, or inability to manage secretions indicates high aspiration risk, hold oral intake.
  • Aspiration pneumonia from dysphagia is a frequent and dangerous complication.

References

Clinical references for this topic

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