Other Names for this Disease
- Ataxia, chorea, seizures, and dementia
- Dentatorubropallidoluysian atrophy
- Haw River syndrome
- Myoclonic epilepsy with choreoathetosis
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.
mutation in the ATN1 gene and is inherited in an autosomal dominant manner. Treatment is symptomatic and supportive.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
Last updated: 9/5/2012
- Dentatorubral-pallidoluysian atrophy. Genetics Home Reference (GHR). 2008; http://ghr.nlm.nih.gov/condition=dentatorubralpallidoluysianatrophy. Accessed 5/10/2010.
- Brice A. Dentatorubral pallidoluysian atrophy. Orphanet. 2004; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=101. Accessed 5/10/2010.
- 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. This website is maintained by the National Library of Medicine.
- 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.
- 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.
- Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine.
- Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
- 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.