This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Huntington's disease (HD) is an autosomal dominant inherited neurodegenerative disorder that is characterized by progressive motor dysfunction, emotional disturbances, dementia, and weight loss. HD occurs worldwide in all races and ethnic groups [1]. Its prevalence is 5-10 cases per 100,000, and there is a new mutation rate as high as 1-3% [2, 3]. There are about 30,000 affected individuals in the United States while another 150,000 Americans have a genetic risk for developing the disease. The average age of onset is about 37 years of age, however the range is from infancy into the 80's. There is increasing reason to believe that pathologic alterations occur in the brain for years before symptoms manifest. Affected individuals are rapidly disabled by early functional decline and require care and supervision for another 15-25 years before succumbing to the effects of severe physical and mental deterioration. There is no therapy proven to delay onset or slow progression, and current medical care focuses on symptom management and optimizing function [4-6]. The primary objective of this clinical trial is to extend findings from the dose-finding study [(CREST-UP1, PHRC Protocol#2004-p-000925)] to evaluate the long-term safety, tolerability and clinical impact of 30 grams per day of creatine in subjects with HD. To serve as a basis for subsequent trials designed to specifically address creatine's ability to slow or halt the progression
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