Dystonia Association South Africa

(Incorporating Essential Tremor, Tourette's Syndrome

 and other Movement Disorders)

Reg No 004-729 NPO

P O Box 4351, Randburg, 2125 or 3rd Floor Standard Bank Building, Oak Avenue, Randburg 2194

Tel: +27 11 326 2112 Fax: +27 11 326 3041

e-mail dystonia@dystonia.org.za , Web www.dystonia.org.za
Office hours - 08.30 to 14.00 Monday to Friday
Urgent enquiries 082 357 6586
 

YEAR 2006/2007

WE ARE SPONSORED BY THE NATIONAL LOTTERY DISTRIBUTION TRUST FUND
 ADVERTISING SPONSORED BY ETv

 

Huntingtons
Tourettes Syndrome
Essential Tremor

HUNTINGTON'S DISEASE

 WHAT IS IT?
A dominant genetic disorder in which a protein is produced abnormally, leading to the breakdown in the parts of the brain that control movement. A genetic disorder of the nervous system, characterised by involuntary movement and progressive mental deterioration. A genetic disease of the central nervous system, that produces progressive dementia and involuntary movements. It usually starts between ages 30 and 50 and slowly progresses to death. Since it is a dominant disease, a child of a parent with Huntington’s disease has a 50-50 chance of inheriting it.

An adult onset disease characterised by progress mental and physical deterioration; it is caused by an inherited dominant gene mutation. A late but variable age onset lethal human disease of nerve degeneration. Inherited as an autosomal dominant phenotype.

Shows imprinting individuals inheriting HD from their father shows significantly earlier onset than those inheriting a maternal allele.

Shows genetic anticipation, in that the severity increase and age of onset decreases in affected individuals of later generations in a pedigree. The HD gene, encoding a protein called huntingtin, has been cloned. A hereditary, progressive, neuro-degenerative disorder primarily characterized by the development of emotional, behavioural, and psychiatric abnormalities; gradual deterioration of the thought processing and acquired intellectual ability-(dementia) and movement  abnormalities; including involuntary, rapid irregular jerky movement (chorea) of the face, arms, legs or trunk. An inherited sex-linked nervous disease that develops in adulthood and leads to a state of the deteriorated mentality. A fatal neurological disease that begins after age 35 and leads to loss of motor control and intellectual deterioration.

It is an hereditary disease of the brain characterised by the onset in adult life of unusual body movements and mental deterioration. the disease is chronically progressive.

Huntington’s chorea: hereditary disease is chronically progressive.

Huntington’s disease or Huntington’s chorea is an inherited disorder characterized by abnormal body movement called chorea and loss of memory. There also is evidence that doctors as far back as the middle ages knew of this devastating disease. The incidence is 5 to 8 per 100,000.

 

 

 

 

 

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