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Physiological AdaptationAutosomal dominant → chorea + dementia

Huntington DiseaseNCLEX cheat sheet

Huntington disease is an inherited, autosomal-dominant, progressive degenerative disorder of the basal ganglia and cerebral cortex causing involuntary movements (chorea), progressive dementia, and emotional disturbance.

20 key facts 3 practice questions Reviewed Oct 2026
The 1-minute sheetCinnaRN

Mech

  • Autosomal dominant, CAG repeat
  • Basal ganglia degeneration
  • Onset age 30-50

Signs

  • Chorea: jerky involuntary moves
  • Progressive dementia
  • Mood swings, depression

Do

  • Aspiration precautions
  • High-calorie soft diet
  • Safety: pad, prevent injury

Report

  • No cure → supportive care
  • Suicide risk, genetic counseling
Red flag to remember

Expressed suicidal ideation or a plan — initiate suicide precautions and notify the provider immediately.

Before you read

Test yourself first: 3 questions

Question 1 of 3

The nurse is assessing a newborn for Hirschsprung disease. Which finding in the first 48 hours of life is most suggestive of this disorder?

What is huntington disease?

Symptoms usually begin between ages 30 and 50, after most patients have already had children, and the disease is relentlessly progressive over 10 to 20 years. Each child of an affected parent has a 50 percent chance of inheriting the gene. There is no cure; treatment is symptomatic and supportive.

Key points to remember

  • Autosomal-dominant inheritance: each offspring of an affected parent has a 50 percent chance of inheriting the defective gene, and genetic testing can confirm the diagnosis.
  • Classic triad: choreiform (jerky, writhing, uncontrollable) movements, progressive cognitive decline/dementia, and psychiatric/behavioral changes including depression and irritability.
  • Chorea and dysphagia worsen over time, leading to high risk for falls, aspiration, malnutrition, and weight loss.
  • No cure exists; tetrabenazine and deutetrabenazine reduce chorea, and antipsychotics/antidepressants manage behavioral symptoms.
  • Suicide risk is elevated due to depression, loss of independence, and a known fatal prognosis.

Signs & symptoms

  • Chorea: jerky involuntary moves
  • Progressive dementia
  • Mood swings, depression

Nursing priorities

  1. Prevent aspiration: assess swallowing, provide thickened liquids and soft foods, position upright for meals, and have suction available.
  2. Maximize caloric intake with high-calorie, high-protein foods because constant movement greatly increases metabolic demand.
  3. Maintain safety: pad the environment, use proper positioning and seating, and implement fall and injury precautions.
  4. Assess mood and screen for suicidal ideation; provide emotional support and psychiatric referral.
  5. Offer genetic counseling and support resources for the patient and family, and facilitate advance care planning.

Patient teaching

  • Offer genetic counseling to the patient and at-risk family members; testing can be done before symptoms appear.
  • Eat frequent high-calorie, high-protein meals and use soft or thickened foods to reduce choking and meet energy needs.
  • Use safety measures such as padded surfaces, supportive seating, and fall precautions as movements worsen.
  • Recognize and report signs of depression or thoughts of self-harm.
  • Complete advance directives and discuss long-term care planning while able to participate.

Red flags: report now

  • Expressed suicidal ideation or a plan — initiate suicide precautions and notify the provider immediately.
  • Coughing, choking, or new fever with meals indicating aspiration — stop oral intake and reassess swallowing.

References

Clinical references for this topic

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