Management of Agitation in Huntington’s Disease: A Review of the Literature

Huntington’s disease (HD) is a rare neurodegenerative disease of the central nervous system characterized by choreatic movements, behavioral disturbances, and neuropsychiatric sequelae. The disease is inherited in an autosomal dominant fashion by an increased number of CAG repeats on the short arm of chromosome 4p16.3 in the Huntingtin gene. Huntington’s disease demonstrates the genetic principle of anticipation, where the larger the number of CAG repeats the earlier the signs and symptoms of the disease appear in subsequent generations. The symptoms often consist of behavioral disturbances and learning disturbances. The disease is suspected based on signs and symptoms and confirmed by genetic testing. There is no cure for the disease, and there is a high rate of neuropsychiatric symptoms including depression, and aggressive behavior. A significant risk of suicide in this population exists given the severity and unrelenting nature of the disease. Most patients will have multiple hospitalizations during the course of the illness. A consultant psychiatrist may be asked to evaluate and make recommendations for the treatment of acute agitation in HD patients. This can be a challenging task given the limited number of studies and the complex nature of agitation in the hospital setting. The aim of this review is to look at the currently available data for the treatment of acute agitation in patients with Huntington’s disease.