The United States Food and Drug Administration (FDA) has approved tetrabenazine (Xenazine®) for the treatment of chorea in people with Huntington disease (HD). This is the first drug approved for the treatment of any symptom of HD. The FDA based this decision on the results of a randomized, placebo-controlled double-blind, multicenter study and other supporting studies.

About Huntington Disease
Huntington disease affects between 4 and 7 out of every 100,000 people. The classic signs of HD include the development of chorea or involuntary, rapid, irregular, jerky movements that may affect the face, arms, legs, or trunk. Other symptoms may include a gradual loss of thought processing and acquired abilities to think and reason (dementia). The person with HD may have trouble with memory, abstract thinking, and judgment; improper perceptions of time, place, or identity (disorientation); increased agitation; and personality changes. Although symptoms typically become evident during the fourth or fifth decades of life, the age at onset may vary and ranges from early childhood to late adulthood (even up to age 70 or 80).

HD is transmitted in an autosomal-dominant manner. That is, only one mutated copy of the gene IT15 is necessary for a person to have HD. Every child of a person with HD has a 50% chance of inheriting the mutated gene.

For more information on HD, please visit the WE MOVE web site at wemove.org/hd. While you are visiting the web site, check out the HD discussion forum by clicking on the discussion tab at the top of the home page. Once you’ve registered, you can share your thoughts and ask questions about living with HD.

About Tetrabenazine
People with HD have overactive dopamine systems in their brains. Tetrabenazine reduces the amount of dopamine in the brain and subsequently reduces the abnormal movements or chorea in people with HD. The FDA granted orphan drug status to Xenazine.

Serious side effects reported with use of tetrabenzine include depression and suicidal thoughts and actions. Tetrabenazine should not be used in patients who are actively suicidal or in patients with untreated depression. Concerns about the risk of suicide are heightened in all people with HD.

To view the FDA’s announcement, go to http://www.fda.gov/bbs/topics/NEWS/2008/NEW01874.html

This entry was posted on Thursday, August 21st, 2008 at 7:08 pm and is filed under Dementia. You can follow any responses to this entry through the RSS 2.0 feed. Responses are currently closed, but you can trackback from your own site.