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Genetic and Rare Diseases Information Center (GARD)

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Juvenile Huntington disease


Other Names for this Disease

  • Huntington disease, juvenile onset
  • JHD
  • Juvenile onset HD
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Overview

Juvenile Huntington disease (HD) is a less common, early-onset form of Huntington disease that begins in childhood or adolescence. It is also a progressive disorder that causes the breakdown of brain cells in certain areas of the brain. This results in uncontrolled movements, loss of intellectual abilities, and emotional disturbances. Juvenile HD is defined by the onset of symptoms before age 20 years and accounts for 5-10% of HD cases. It is inherited in an autosomal dominant pattern and is caused by a large number of CAG trinucleotide repeats in the HTT gene. A larger number of repeats is usually associated with an earlier onset of signs and symptoms. Most often, children with juvenile HD inherit the expanded CAG trinucleotide repeat from their fathers, although on occasion they inherit it from their mothers.[1][2]
Last updated: 6/27/2011

References

  1. Warby SC, Graham RK &Hayden MR. Huntington Disease. GeneReviews. April 2010; http://www.ncbi.nlm.nih.gov/books/NBK1305/. Accessed 2/4/2014.
  2. Huntington disease . Genetics Home Reference. OCtober 2008; http://ghr.nlm.nih.gov/condition/huntington-disease . Accessed 6/27/2011.
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Basic Information

In Depth 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 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. 
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Juvenile Huntington disease. Click on the link to view a sample search on this topic.

Insurance Issues

Other Names for this Disease
  • Huntington disease, juvenile onset
  • JHD
  • Juvenile onset HD
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.