Young, Anne B. Department of Neurology, University of Michigan, Ann Arbor, Michigan.
Last reviewed:August 2019
Show previous versions
- Related Primary Literature
- Additional Reading
A hereditary disorder of the basal ganglia of the brain causing progressive motor incoordination, abnormal involuntary movements (chorea), and intellectual decline. Huntington's disease is a rare disorder that affects approximately five to ten of every 100,000 persons. The disease, which progresses gradually over 15 to 20 years, is invariably fatal. Inherited as an autosomal dominant mendelian trait, it inevitably develops in those who carry the gene if they live long enough. Men and women are affected equally. The average age at onset is between 35 and 40 years, but the disease can begin as early as 2 years or as late as 80 years (see illustration). The mutation rate is very low, and no confirmed new mutations have been reported. Pathologically, the nerve cells in the caudate nucleus and putamen degenerate; these regions are the most seriously damaged, although other regions of the brain are affected to a minor extent. See also: Brain; Degenerative neural diseases; Gene; Genetics; Human genetics; Mutation; Nerve; Nervous system disorders
The content above is only an excerpt.
for your institution. Subscribe
To learn more about subscribing to AccessScience, or to request a no-risk trial of this award-winning scientific reference for your institution, fill in your information and a member of our Sales Team will contact you as soon as possible.
to your librarian. Recommend
Let your librarian know about the award-winning gateway to the most trustworthy and accurate scientific information.
AccessScience provides the most accurate and trustworthy scientific information available.
Recognized as an award-winning gateway to scientific knowledge, AccessScience is an amazing online resource that contains high-quality reference material written specifically for students. Contributors include more than 9000 highly qualified scientists and 43 Nobel Prize winners.
MORE THAN 8700 articles covering all major scientific disciplines and encompassing the McGraw-Hill Encyclopedia of Science & Technology and McGraw-Hill Yearbook of Science & Technology
115,000-PLUS definitions from the McGraw-Hill Dictionary of Scientific and Technical Terms
3000 biographies of notable scientific figures
MORE THAN 19,000 downloadable images and animations illustrating key topics
ENGAGING VIDEOS highlighting the life and work of award-winning scientists
SUGGESTIONS FOR FURTHER STUDY and additional readings to guide students to deeper understanding and research
LINKS TO CITABLE LITERATURE help students expand their knowledge using primary sources of information