Huntington's disease

(redirected from Huntington disease)
Also found in: Dictionary, Thesaurus, Medical, Wikipedia.

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
..... Click the link for more information.
 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 ?
The study enrolled Stage 1 patients showing early disease symptoms with a Unified Huntington Disease Rating Scale (UHDRS), Total Motor Score (TMS) [greater than or equal to] 5, Total Functional Capacity (TFC) > 10 and a CAG repeat > 38.
The trial included 109 men and women with a family history of Huntington disease, some of whom were aware of carrying a mutated form of the gene that causes the disease.
Because the new therapy uses a molecule already being used in clinical trials for other diseases, it could be used in a clinical trial for Huntington disease within the next one to two years.
The current study raised uncertainty about this potential therapeutic approach to the treatment of Huntington disease.
Genetic linkage between Huntington disease and the D4S10 locus in South African families: Further evidence against non-allelic heterogeneity.
SAHA has also been shown to prevent movement disorders in a mouse version of Huntington disease, where the buildup of abnormal proteins in brain cells jams the acetylation--deacetylation regulatory system.
Davies, professor of gerontology in the USC Davis School of Gerontology and professor of biological sciences in the USC College of Letters, Arts and Sciences has found that a form of the gene RCAN1, known as RCAN1-1L, is dramatically decreased in human brains affected by Huntington disease.
Preliminary evidence suggests PBT2 250mg reduced atrophy of brain tissue in areas affected in Huntington disease, seen in a pilot imaging sub-study.
Orphan Drug Designation Granted for Dimebon for Treatment of Huntington Disease
a non-profit organization pursuing the discovery and development of drugs to prevent or slow the progression of Huntington disease (HD), announced today that they have entered into an agreement under which CHDI will assess Intellect's OXIGON(TM) as a potential therapy for Huntington disease.
Stem Cell Innovations is developing a potentially powerful technology that may offer a route to screening for novel treatment alternatives for patients with Huntington disease," said Dr.

Full browser ?