Treatment of Behavioral Disinhibition in Huntington's Disease With Valproic Acid

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Excerpt

To the Editors
Huntington's disease (HD) is an autosomal dominant disease with a population prevalence of about 10 per 100,000. Huntington's disease is caused by a single gene mutation, a cytosine-adenine-guanine trinucleotide repeat expansion in the huntingtin gene on chromosome 4. This leads to striatal atrophy, manifesting clinically as choreiform movements, cognitive impairment, and psychiatric changes.1 The latter is inevitable in HD, often antedating the movement disorder, and may be difficult to treat.2,3 We recently cared for a woman with HD whose severe psychiatric problems were best explained as behavioral disinhibition; her response to valproic acid (VPA) was gratifying.
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