Huntington's disease


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Huntington's disease,

hereditary, acute disturbance of the central nervous system usually beginning in middle age and characterized by involuntary muscular movements and progressive intellectual deterioration; formerly called Huntington's chorea. The disease is sometimes confused with choreachorea
or St. Vitus's dance,
acute disturbance of the central nervous system characterized by involuntary muscular movements of the face and extremities. The disease, known also as Sydenham's chorea (not to be confused with Huntington's disease, a hereditary disease of
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 or St. Vitus's dance, which is not hereditary. A faulty gene produces a defective protein attacks neurons in the basal ganglia, clusters of nerve tissue deep within the brain that govern coordination.

The onset is insidious and inexorably progressive; no treatment is known. Psychiatric disturbances range from personality changes involving apathy and irritability to bipolar or schizophreniform illness. Motor manifestations include flicking movements of the extremities, a lilting gait, and motor impersistence (inability to sustain a motor act such as tongue protrusion).

In 1993 the gene responsible for the disease was located; within that gene a small segment of code is, for some reason, copied over and over. Genetic counseling is extremely important, since 50% of the offspring of an affected parent inherit the gene, which inevitably leads to the disease.

References in periodicals archive ?
Our research has taken advantage of cutting edge genomics approaches using a simple model organism to identify a novel area for potential therapeutic intervention for Huntington's disease," said Flaviano Giorgini, lead author of the study.
Roughly 30,000 people in the United States have Huntington's disease, a condition marked by a loss of coordination, slurred speech, swallowing difficulties, and other problems.
The profile of a person with Huntington's disease is quite different from that of the average person in a nursing home," said Sharon St.
They repeated the calls to the UK Government to intervene to help ensure that those with Huntington's disease can access affordable insurance.
This is potentially an exciting breakthrough in Huntington's disease therapeutic research, giving hope to this community and future generations, without whose support and commitment, we would not be able to conduct clinical trials.
For more information about Huntington's disease please visit www.
According to the latest epidemiological study conducted in 2015 at the institute, there are 42 patients with Huntington's disease and 22 asymptomatic carriers of the Huntington gene mutation in Cyprus, all of whom from 19 families.
Non-choreic movement disorders as initial manifestations of Huntington's disease.
A snapshot of the global therapeutic scenario for Huntington's Disease.
Asa Petersen, Associate Professor of Neuroscience at Lund University, asserted that they're the first to show that it is possible to prevent the depression symptoms of Huntington's disease by deactivating the diseased protein in nerve cell populations in the hypothalamus in the brain.
The Huntington's Disease Association (HDA) won the prestigious GlaxoSmithKline IMPACT Award for its outstanding contribution to improving the lives of people with the hereditary disease across the UK.
KineMed, Isis and CHDI collaborate to advance therapeutic development for Huntington's disease and develop novel biomarkers for pre-clinical and clinical use in drug development and therapeutic monitoring

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