Huntington's chorea

Huntington's chorea is a neurodegenerative disorder that affects muscle coordination and leads to cognitive decline and psychiatric problems. It is the most common genetic cause of abnormal involuntary writhing movements called chorea, which is characterized by brief, irregular contractions that are not repetitive or rhythmic, but appear to flow from one muscle to the next.

Symptoms

Huntington's chorea symptoms can vary between individuals and among affected members of the same family. The earliest symptoms are often subtle problems with mood or mental abilities. A general lack of coordination and an unsteady gait often follow. As the disease advances, uncoordinated, jerky body movements become more apparent. Physical abilities gradually worsen until coordinated movement becomes difficult and the person is unable to talk. Mental abilities generally decline into dementia.

Causes

Huntington's chorea is caused by an inherited defect in a single gene. Huntington's disease is an autosomal dominant disorder, which means that a person needs only one copy of the defective gene to develop the disorder.

Diagnosis

The diagnosis of Huntington's chorea is based on a physical examination, a review of family medical history, and neurological and psychological tests. Genetic testing can provide a definitive diagnosis of Huntington's chorea.

Treatment

There is no cure for Huntington's chorea, but treatment can help manage symptoms. Medications are available to help manage the movement problems and psychiatric symptoms. Physical therapy can also help manage movement problems.

Prognosis

The length of time from disease onset to death is often about 10 to 30 years. Juvenile Huntington's disease usually results in death within 10 years after symptoms develop.

See also

• Neurodegenerative disorders
• Chorea
• Dementia
• Genetic disorders
• Autosomal dominant

Huntington's chorea Resources
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