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Chorea (disease)

Chorea (also known as St. Vitus dance) is an abnormal voluntary movement disorder, one of a group of neurological disorders called dyskinesias, which are caused by overactivity of the neurotransmitter dopamine in the areas of the brain that control movement. Chorea is characterized by brief, irregular contractions that are not repetitive or rhythmic, but appear to flow from one muscle to the next. Chorea often occurs with athetosis, which adds twisting and writhing movements. Chorea is a primary feature of Huntington's disease, a progressive, hereditary movement disorder that appears in adults, but it may also occur in a variety of other conditions. more...

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Sydenham's chorea occurs in a small percentage (20 percent) of children and adolescents as a complication of rheumatic fever. Chorea can also be induced by drugs (levodopa, anti-convulsants, and anti-psychotics) metabolic and endocrine disorders and vascular incidents.

When chorea is serious, slight movements will become thrashing motions, this form of severe chorea is referred to as "ballism". Walking may become peculiar, and include odd postures and leg movements. Unlike ataxia and dystonia, which affect the quality of voluntary movements or parkinsonism, which is a hindrance of voluntary movements, the movements of chorea and ballism occur on their own, without conscious effort.

The term chorea is derived from a Greek word khoreia (a kind of dance, see chorea), as the quick movements of the feet or hands are vaguely comparable to dancing or piano playing.

Treatment

There is no standard course of treatment for chorea. Treatment depends on the type of chorea and the associated disease. Treatment for Huntington's disease is supportive, while treatment for Syndenham's chorea usually involves antibiotic drugs to treat the infection, followed by drug therapy to prevent recurrence. Adjusting medication dosages can treat drug-induced chorea. Metabolic and endocrine-related choreas are treated according to the cause(s) of symptoms.

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Unsaturated fatty acids for Huntington's disease - Literature Review & Commentary - Brief Article
From Townsend Letter for Doctors and Patients, 4/1/03 by Alan R. Gaby

Seventeen patients with Huntington's disease were randomly assigned to receive, in double-blind fashion, a supplement containing unsaturated fatty acids or a placebo. The dosage of unsaturated fatty acids was eight 1-g capsules per day; each capsule contained 70 mg of gamma-linolenic acid (GLA), 35 mg of eicosapentaenoic acid (EPA), 20 mg of docosahexaenoic acid (DHA), 50 mg of alpha-lipoic acid, and 30mg of vitamin E, with linoleic acid as a carrier. The placebo contained hydrogenated coconut oil, alpha-lipoic acid, and vitamin E. The mean duration of treatment was 19 months for active treatment and 20 months for placebo. On the Rockland-Simpson Dyskinesia Rating Scale, 7 patients receiving active treatment improved and 2 became worse, whereas 1 patient receiving placebo improved, 1 was unchanged, and 6 became worse (p = 0.01 for the difference in the response between groups). A similar trend (p = 0.08) was seen using the Unified Huntington's Disease Rating Scale. No significant side effects were seen.

Comment: Huntington's disease is a hereditary, progressive degenerative brain disorder that eventually results in death. Because no effective conventional treatment is available, the results of this new study are encouraging. In another recent double-blind study (Neuroreport 2002;13:123-6), supplementation with the ethyl-ester of EPA (1 g twice a day for 6 months) resulted in significant improvement in motor function in patients with Huntington's disease. In addition, each of two patients in the active-treatment group who underwent MRI brain scans before and after treatment showed a reversal of cerebral atrophy. These two studies of unsaturated fatty acids offer new hope in the treatment of this devastating disease.

Vaddadi KS, et al. A randomised, placebo-controlled, double blind study of treatment of Huntington's disease with unsaturated fatty acids. Clin Neurosci Neuropathol 2002;13:29-33.

COPYRIGHT 2003 The Townsend Letter Group
COPYRIGHT 2003 Gale Group

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