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 ?
The report provides a snapshot of the global therapeutic landscape of Huntington Disease
DNA haplotype analysis of Huntington disease reveals clues to the origins and mechanisms of CAG expansion and reasons for geographic variations of prevalence.
At the end of the placebo-controlled phase of the trial, the rate of atrophy in the brain's cortex and striatum among pre-manifest Huntington disease carriers was slower among those who received creatine than among the placebo group, who experienced progression.
This is very promising and should give hope to those with Huntington disease.
CAG/CTG repeat expansions at the HDL2 locus are a common cause of Huntington disease in black South Africans.
Sex-dependent mechanisms for expansions and contractions of the CAG repeat on affected Huntington disease chromosomes.
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.
Huntington disease in black African populations (letter).
We have donated $1,000 to the Huntington Disease Society of America in her honor.
Orphan Drug Designation Granted for Dimebon for Treatment of Huntington Disease

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