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Other Names for this Disease
- Ataxia, chorea, seizures, and dementia
- Dentatorubropallidoluysian atrophy
- Haw River syndrome
- Myoclonic epilepsy with choreoathetosis
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Dentatorubral-pallidoluysian atrophy (DRPLA) is a progressive brain disorder that causes involuntary movements; mental and emotional problems; and a decline in thinking ability. The average age of onset of DRPLA is 30 years, but the condition can appear anytime from infancy to mid-adulthood. Specific signs and symptoms may differ among affected individuals and sometimes affects children and adults differently. DRPLA is caused by a mutation in the ATN1 gene and is inherited in an autosomal dominant manner. Treatment is symptomatic and supportive.
- Dentatorubral-pallidoluysian atrophy. Genetics Home Reference (GHR). http://ghr.nlm.nih.gov/condition=dentatorubralpallidoluysianatrophy. Accessed May 10, 2010.
- Brice A. Dentatorubral pallidoluysian atrophy. Orphanet. http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=101. Accessed May 10, 2010.
On this page
- Stanford University's HOPES Web site offers a detailed description of DRPLA with illustrations. Click on HOPES to view the information page.
- Genetics Home Reference (GHR) contains information on Dentatorubral-pallidoluysian atrophy. Click on the link to go to GHR and review the information.
- Medscape Reference provides information on this topic. Click on the link to view this information. You may need to register to view the medical textbook, but registration is free.
- The National Organization for Rare Disorders (NORD) is a federation of more than 130 nonprofit voluntary health organizations serving people with rare disorders. Click on the link to view information on this topic.
- Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge. Click on the link to read information on this topic.
- PubMed is a searchable database of medical literature and lists journal articles that discuss Dentatorubral-pallidoluysian atrophy. Click on the link to view a sample search on this topic.
- The The Online Mendelian Inheritance in Man (OMIM) database contains genetics resources that discuss Dentatorubral-pallidoluysian atrophy. Click on the link to go to OMIM and review these resources.