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.

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References in periodicals archive ?
Carter et al.; "Reversal of Cellular Phenotypes in Neural Cells Derived from Huntington's Disease Monkey-Induced Pluripotent Stem Cells"; Stem Cell Reports, 2014; DOI: 10.1016/j.stemcr.2014.07.011
* Coverage of the Huntington's Disease pipeline on the basis of route of administration and molecule type.
But in the striatum, the region of the brain that is devastated by Huntington's disease, this capability is "switched off" in adulthood.
Editor's Note: Huntington's disease is a neurodegenerative disorder caused by a genetic error that produces abnormal proteins in the brain's cells.
Huntington's disease gradually takes away the ability to control movements, think normally, and cam for oneself.
Behavioral and psychiatric symptoms associated with Huntington's disease. Adv Neurol.
CHDI and its collaborators have developed a unique series of 20 ES cell lines from genetically modified mouse models that mimic aspects of Huntington's disease. These cell lines and mouse models represent an important tool for medical researchers to use in a wide variety of research programmes in Huntington's disease, a genetic neurological disorder affecting 7 in 100,000 people.
In December 2007, all 12 members of the Food and Drug 'Administration's Peripheral and Central Nervous System Drugs Advisory Committee agreed that available data from a randomized, placebo-controlled study of 84 ambulatory patients with Huntington's disease supported the drug's approval.
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."
The high frequency of juvenile Huntington's disease in South Africa.
The FDA issued an "approvable" letter for tetrabenazine for the treatment of chorea associated with Huntington's disease, according to a statement issued by Prestwick.
In addition to its stroke and Huntington's disease programmes, ReNeuron is developing stem cell therapies for Parkinson's disease, Type 1 diabetes and diseases of the retina.

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