Huntington’s Disease Definition
Huntington’s disease, or Huntington’s chorea, is a progressive, inherited condition that causes brain cells to lose function and die, leading to involuntary movements, cognitive decline, and emotional changes (Cleveland Clinic, 2023).
There are two types of HD:
- Adult onset: Symptoms typically appear after age 30
- Juvenile onset: A rare form presenting in children and teenagers
Etiology
Huntington’s disease is caused by a mutation in the HTT gene that leads to abnormal huntingtin proteins that damage neurons. Inheritance is in an autosomal dominant pattern. A child of an affected parent has a 50% chance of inheriting the mutation (National Institute of Neurological Disorders and Stroke, 2024).
Rarely, Huntington’s disease may occur without a family history.
Huntington’s Disease Diagnostic Criteria
A definitive diagnosis of Huntington's disease is made by genetic testing. Individuals who have the gene will develop the disease. MRI and CT scans may be performed to show images of the brain. These tests may show areas of the brain affected and rule out other causes of symptoms.
A preliminary diagnosis of Huntington's disease is made by review of symptoms, family history, and physical, neurological, and psychiatric exams.
Specialists evaluations may include:
- Neuropsychology
- Speech pathology
- Genetic counseling
Common Signs and Symptoms of Huntington’s Disease
Signs and symptoms of Huntington’s include:
- Chorea or involuntary movements
- Unsteady gait
- Increasingly slow and rigid movements
- Forgetfulness
- Impaired judgment
- Difficulty concentrating
- Obsessive-compulsive symptoms
- Personality changes
- Slurred speech
- Mood swings and depression
- Difficulty swallowing
- Weight loss
Red Flags
Notify the healthcare provider immediately for:
- Severe weight loss due to dysphagia and involuntary movements
- Increased choking or coughing during meals
- Sudden behavioral changes or worsening cognitive symptoms
Potential Complications
Complications may include:
- Pneumonia from aspiration
- Malnutrition due to feeding difficulties
- Falls resulting in fractures or head injuries
- Dementia progression
Treatments
Pharmacological
There is currently no treatment to slow or stop the progression of Huntington's disease. Current treatment focuses on easing the symptoms associated with Huntington's disease, such as (Huntington’s Chorea Nursing Management, 2025):
- Tetrabenazine (Xenazine®) and deutetrabenazine (Austedo®) for chorea
- Antidepressants and antipsychotics for mood and behavioral changes
Non-Pharmacological
Treatment requires an interdisciplinary approach and may consist of a:
- Neurologist
- Psychiatrist
- Psychologist
- Speech therapist
- Occupational therapist
- Physical therapist
There are investigative therapies, including gene editing and molecular therapies, currently being researched.
Huntington’s Disease Nursing Management Care Plan
Assessment and Interventions
To assess for injuries from falls:
- Assist with ambulation and transfers.
- Provide physical and occupational therapy.
- Modify the environment to support safety and independence.
To assess nutritional deficits:
- Consult speech therapy.
- Provide high-calorie, nutrient-dense meals in small, manageable portions.
- Assist the person with sitting up during meals.
- Monitor for coughing and choking during meals.
- Follow speech therapist recommendations.
- Have suction available at the bedside.
- Provide respiratory support such as suctioning and oxygenation as needed.
To assess emotional and psychiatric disturbances:
- Actively listen to feelings and concerns.
- Assess for potential suicide risk and suicidal ideation.
- Administer medications as ordered.
- Refer to a psychiatrist and social worker as needed.
Psychosocial and Cultural Considerations
Psychosocial Support
Support includes:
- Provide emotional support for coping with progressive disability.
- Connect the person and families to support groups.
Cultural Considerations
Consider the person’s culture by:
- Respecting cultural beliefs influencing decisions about genetic testing
Home Management
Self-Care
Management includes:
- Encourage physical activity to reduce stiffness.
- Maintain hydration and monitor for signs of dehydration.
Safety Measures
Ensure the person’s safety by:
- Adapting the home environment to prevent falls, including installing railings and removing hazards
- Using adaptive utensils to aid feeding
FAQs
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Content Release Date
6/11/2024
Content Expiration
12/31/2029
Course Contributor
The content for this course was created by Kim Matthews, RN. Ms. Matthews obtained a nursing degree from Western Kentucky University in 1998. Ms. Matthews possesses over 20 years of nursing experience with over 17 of those in the Skilled Nursing industry. Ms. Matthews has extensive experience in MDS, restorative nursing programs, and nursing management. Ms. Matthews is currently a Post-Acute Care Content writer and subject matter expert for MDS.
References
- Cleveland Clinic. (2023). Huntington’s disease: Overview, symptoms, and care. https://my.clevelandclinic.org/health/diseases/14369-huntingtons-disease
- Huntington’s Chorea Nursing Management. (2025). Nursing reference guide. https://www.rnpedia.com/nursing-notes/medical-surgical-nursing-notes/huntingtons-chorea-nursing-management/#google_vignette
- National Institute of Neurological Disorders and Stroke. (2024). Huntington's disease: Updated information and care. https://www.ninds.nih.gov/health-information/disorders/huntingtons-disease?search-term=Huntington%27s
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