Huntington's disease

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Related to Huntington's: Huntington's chorea, Parkinson's

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 ?
4 -- 5 -- color) This Ecua-Bess orchid, left, is planted in the Huntington's Tropical Forest Rotunda and may be viewed by visitors to the exhibition ``A Natural Obsession,'' which also features a variety of hand-illustrated books, including ``The Orchidaceae of Mexico and Guatemala,'' above.
Huntington's central concern is that 35 years of heavy migration has established a large Hispanic population in the United States that is substantially different from earlier immigrant groups.
Somehow, despite Huntington's pessimism, we Chicagoans are surviving this terrific cultural crisis with good grace, a good sense of humor, and a hopefulness, optimism, and vitality that is completely lacking in his American dream interpretation, obscured as it is by prejudice and fear.
Egypt as a part of Islam or Germany as part of the West), "core state" (a country, such as China, that constitutes the main representative of a civilization), "lone country" (Japan, which coincides with a whole civilization), "cleft country" (one so unfortunate, as in the case of the Sudan, to be split into two or more civilizations), and "torn country" (one such as Turkey whose leadership has--unwisely from Huntington's point of view--tried to uproot it from its own civilization and transplant it in alien soil).
In the March Neuron, Junying Yuan of Harvard Medical School in Boston and her colleagues report that certain features of the mutant proteins made in Huntington's disease, so-called polyglutamine repeats, help activate one of the many caspases found in mammalian cells.
Using the single largest gift in the Huntington Library's history, Henry Huntington's garage has been transformed into the new MaryLou and George Boone Gallery now housing the ``Art of Bloomsbury'' exhibit.
The same kind of simplistic view underlies Huntington's view of relations between states.
Another research group has also begun treating Huntington's patients with fetal cells, but this team uses porcine tissue.
We've never seen crowds like we have today,'' said Catherine Babcock, the Huntington's communications director.
The gene behind the neurological disorder Huntington's disease continues to surprise investigators.
Among the more notable scholarly contributions have been the energetic dissents to Huntington's thesis published in Foreign Affairs in September/October 1993 (including, surprisingly, comments by Fouad Ajami and Jeane J.
Using mutant proteins from people with Huntington's disease as bait, investigators have reeled in a novel brain protein that may play a role in this neurodegenerative disorder.

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