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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Dentatorubral-pallidoluysian atrophy


Other Names for this Disease

  • Ataxia, chorea, seizures, and dementia
  • Dentatorubropallidoluysian atrophy
  • DRPLA
  • Haw River syndrome
  • Myoclonic epilepsy with choreoathetosis
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Treatment

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How might dentatorubral-pallidoluysian atrophy (DRPLA) be treated?

There is no cure for DRPLA; treatment is generally symptomatic and supportive.[1] Management of signs and symptoms may include:[2] 

  • Treatment of seizures with anti-epileptic drugs
  • Treatment of psychiatric problems with appropriate psychotropic medications
  • Adaptation of environment and care to the level of dementia
  • Adaptation of educational programs for affected children.
Last updated: 9/5/2012

References
  1. 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.
  2. Shoji Tsuji. DRPLA. GeneReviews. June 1, 2010; http://www.ncbi.nlm.nih.gov/books/NBK1491/. Accessed 9/5/2012.


Management Guidelines

  • GeneReviews provides current, expert-authored, peer-reviewed, full-text articles describing the application of genetic testing to the diagnosis, management, and genetic counseling of patients with specific inherited conditions. Click on the link to view the article on this topic.

Clinical Trials & Research for this Disease

  • The Research Portfolio Online Reporting Tool (RePORT) provides access to reports, data, and analyses of research activities at the National Institutes of Health (NIH), including information on NIH expenditures and the results of NIH-supported research. Although these projects may not conduct studies on humans, you may want to contact the investigators to learn more. To search for studies, enter the disease name in the "Text Search" box. Then click "Submit Query".
Other Names for this Disease
  • Ataxia, chorea, seizures, and dementia
  • Dentatorubropallidoluysian atrophy
  • DRPLA
  • 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.