Huntington’s disease is a rare, inherited disease that causes the progressive breakdown (degeneration) of nerve cells in the brain. Huntington’s disease has a broad impact on a person’s functional abilities and usually results in movement, thinking (cognitive) and psychiatric disorders.
Symptoms usually begin between 30 and 50 years of age but can start at any age.
Huntington’s disease is typically inherited. A child of an affected person usually has a 50% chance of inheriting the disease. Diagnosis is by genetic testing, which can be carried out at any time, regardless of whether or not symptoms are present. This fact raises several ethical debates, e.g. the age at which an individual is considered mature enough to choose to test. If the condition develops before age 20, it is called juvenile Huntington’s disease. When Huntington’s develops early, symptoms are somewhat different, and the disease may progress faster.
Medications are available to help manage the symptoms of Huntington’s disease. But treatments cannot prevent the physical, mental and behavioural decline associated with the condition.
- Stumbling and clumsiness
- Involuntary jerking or fidgety movements of the limbs and body
- Mood swings and personality changes
- Problems swallowing, speaking and breathing
- Difficulty moving
Treatment and Support:
Full-time nursing care is needed in the later stages of the condition. It is usually fatal 15 to 20 years after symptoms start. There is currently no cure for Huntington’s disease or any way to stop it getting worse, but treatment and support can help reduce some of the problems it causes, such as:
- Medication for depression, mood swings and involuntary movements
- Occupational therapy
- Speech and language therapy for feeding and communication problems
- Physiotherapy to help with movements and balance
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Huntington’s disease is named after George Huntington, who described it among residents of East Hampton, Long Island in 1872. It is a hereditary neurodegenerative disease. In 1993, a collaborative group of investigators discovered the gene that causes Huntington’s disease.