Juvenile Huntington disease
Other Names for this Disease
- Huntington disease, juvenile onset
- Juvenile onset HD
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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 mutation called a trinucleotide repeat in the HTT gene. Most often, children with juvenile HD inherit the mutation repeat from their fathers, although on occasion they inherit it from their mothers. Juvenile Huntington disease has a rapid disease progression once symptoms present. There currently is no cure. Treatment is supportive and focused on increasing quality of life.Juvenile Huntington disease (HD) is a less common, early-onset form of
Last updated: 3/11/2016
- Warby SC, Graham RK &Hayden MR. Huntington Disease. GeneReviews. December 11, 2014; http://www.ncbi.nlm.nih.gov/books/NBK1305/.
- Huntington disease. Genetics Home Reference. June 2013; http://ghr.nlm.nih.gov/condition/huntington-disease.
- Juvenile Onset HD. Huntington's Disease Society of America. http://hdsa.org/living-with-hd/juvenile-onset-hd/. Accessed 3/11/2016.
- Genetics Home Reference (GHR) contains information on Juvenile Huntington disease. This website is maintained by the National Library of Medicine.
- The Merck Manuals Online Medical Library provides information on this condition for patients and caregivers.
- The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.
- Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
- The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
- 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.