Email a Friend
Print ArticleBrain & Nervous System Health Center
Huntington's Disease
Important
It is possible that the main title of the report Huntington's Diseaseis not the name you expected.
Synonyms
- Chronic Progressive Chorea
- Degenerative Chorea
- HD
- Hereditary Chorea
- Hereditary Chronic Progressive Chorea
- Huntington's Chorea
- Very Early Onset Huntington's Disease
- Woody Guthrie's Disease
- VEOHD
Disorder Subdivisions
- None
General Discussion
Huntington's disease is a genetic, progressive, neurodegenerative disorder characterized by the gradual development of involuntary muscle movements affecting the hands, feet, face, and trunk and progressive deterioration of cognitive processes and memory (dementia). Neurologic movement abnormalities may include uncontrolled, irregular, rapid, jerky movements (chorea) and athetosis, a condition characterized by relatively slow, writhing involuntary movements. Dementia is typically associated with progressive disorientation and confusion, personality disintegration, impairment of memory control, restlessness, agitation, and other symptoms and findings. In individuals with the disorder, disease duration may range from approximately 10 years up to 25 years or more. Life-threatening complications may result from pneumonia or other infections, injuries related to falls, or other associated developments.
Huntington's disease is transmitted as an autosomal dominant trait. The disease results from changes (mutations) of a gene known as "huntingtin" located on the short arm (p) of chromosome 4 (4p16.3). In those with the disorder, the huntingtin gene contains errors in the coded "building blocks" (nucleotide bases) that make up the gene's instructions. The gene contains abnormally long repeats of coded instructions consisting of the basic chemicals cytosine, adenine, and guanine (CAG trinucleotide repeat expansion). The length of the expanded repeats may affect the age at symptom onset. The specific symptoms and physical features associated with Huntington's disease result from degeneration of nerve cells (neurons) within certain areas of the brain (e.g., basal ganglia, cerebral cortex).
Resources
Huntington's Disease Society of America
505 Eighth Avenue
Suite 902
New York
NY
10018
USA
Tel: (212)242-1968
Fax: (212)239-3430
800: (800)345-4372
info@hdsa.org
http://www.hdsa.org
WE MOVE (Worldwide Education and Awareness for Movement Disorders)
204 West 84th Street
New York
NY
10024
USA
Tel: (212)875-8312
Fax: (212)875-8389
wemove@wemove.org
http://www.wemove.org
March of Dimes Birth Defects Foundation
1275 Mamaroneck Avenue
White Plains
NY
10605
Tel: (914)428-7100
Fax: (914)997-4763
800: (888)663-4637
Askus@marchofdimes.com
http://www.marchofdimes.com
Hereditary Disease Foundation
3960 Broadway
6th Floor
New York
NY
10032
Tel: (212)928-2121
Fax: (212)928-2172
cures@hdfoundation.org
http://www.hdfoundation.org
Huntington Society of Canada
151 Frederick St, Suite 400
Kitchner
Ontario
Intl
N2H 2M2
Canada
Tel: 5197497063
Fax: 5197498965
800: 8009987398
info@hsc-ca.org
http://www.huntington society.org
National Institute of Neurological Disorders and Stroke (NINDS)
31 Center Drive
8A07
Bethesda
MD
20892-2540
Tel: (301)496-5751
Fax: (301)402-2186
800: (800)352-9424
braininfo@ninds.nih.gov
http://www.ninds.nih.gov/
MUMS (Mothers United for Moral Support, Inc) National Parent-to-Parent Network
150 Custer Court
Green Bay
WI
54301-1243
USA
Tel: (920)336-5333
Fax: (920)339-0995
800: (877)336-5333
mums@netnet.net
http://www.netnet.net/mums/
Sjældne Diagnoser / Rare Disorders Denmark
Frederiksholms Kanal 2, 3rd Floor
Copenhagen K
1220
Denmark
Tel: 45 33 14 00 10
Fax: 45 33 14 55 09
mail@sjaeldnediagnoser
http://www.raredisorders.dk
Alzheimer's Foundation of America
322 8th Ave
6th Floor
New York
NY
10001
USA
Tel: (866)232-8484
Fax: (646)638-1546
800: (866)232-8484
info@alzfdn.org
http://www.alzfdn.org
New Horizons Un-Limited, Inc.
811 East Wisconsin Ave
Suite 937
Milwaukee
WI
53202
USA
Tel: (414)299-0124
Fax: (414)347-1977
horizons@new-horizons.org
http://www.new-horizons.org
UCSF Memory and Aging Center
350 Parnassus Avenue
Suite 706
San Francisco
CA
94117
Tel: (415)476-6800
Fax: (415)476-4800
http://memory.ucsf.edu
Advocacy for Neuroacanthocytosis Patients
32 Launceston Place
London
W8 5RN
UK
Tel: +44 (0)20 7937-2938
Fax: +44 (0)207 495 4245
glenn@naadvocacy.org
http://www.naadvocacy.org
For a Complete Report:
This is an abstract of a report from the National Organization for Rare Disorders, Inc. ® (NORD). A copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html
The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.
It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report
This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.
For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org
Last Updated: 8/15/2008
Copyright 1984, 1985, 1986, 1987, 1988, 1989, 1990, 1991, 1992, 1993, 1994, 1996, 1997, 1998, 1999, 2000, 2001, 2002, 2004, 2006, 2007 National Organization for Rare Disorders, Inc.
WebMD Medical Reference from the National Organization of Rare Disorders
Many people experience dizzy spells, but how much is too much? Learn when dizzy equals danger.
