Huntington's disease

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Related to Huntington's disease: Parkinson's disease

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 ?
This phase of the study examined the effect of RP103 in Huntington's disease subjects treated earlier, beginning at Month 0 (RP103/RP103) compared to subjects with a delayed start to treatment, beginning at Month 18 (placebo/RP103).
Now that we have been able to show in animal experiments that depression and anxiety occur very early in Huntington's disease, we want to identify more specifically which nerve cells in the hypothalamus are critical in the development of these symptoms.
A pharmacodynamic biomarker could be an important contribution to our clinical development efforts, and we look forward to working with KineMed and CHDI to evaluate KineMed's biomarker platform in our Huntington's disease program," said Frank Bennett, Ph.
While the trial had found a reduction in Huntington's disease symptoms after treatment with CoQ 10, the current research uncovered a 17% reduction in 8OHdG levels in Huntington's disease patients as well as a nonsignificant reduction in subjects who did not have the disease.
The new breakthrough involves taking skin cells from patients with Huntington's disease.
Huntington's disease is a degenerative brain disorder that typically strikes adults in mid-life.
Children of affected persons each have a 50/50 chance of inheriting a defective version of the huntingtin gene that causes Huntington's disease," says Dr.
The experience of Stem Cell Sciences in standardizing conditions for growing stem cells should help accelerate the evaluation of the various mouse ES cells for Huntington's disease target validation and drug discovery programs.
Janet Greeson, Chief Executive Officer of Samaritan, commented: "We believe our CNS drugs hold a lot of promise to cognitively improve Alzheimer's patients and protect their brain from further damage; so we are extremely hopeful our drugs will demonstrate and hold the same promise for Huntington's disease.
Huntington's disease (HD) is a late-onset inherited progressive neurodegenerative disorder characterised by motor impairment, chorea, dementia and psychiatric disturbances associated with selective neuronal death in the striatum and cortex.
A GIRL has died of Huntington's disease aged just nine.
The company has also generated pre-clinical efficacy data with its ReN005 stem cell therapy for Huntington's disease, a rare, genetic and fatal neurodegenerative disorder which affects around 1 in 100,000 people.

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