Psychiatric management of Huntington's disease in extended care settings.

Abstract

Huntington’s disease is a neurodegenerative disorder characterized by midlife onset, involuntary movements, cognitive decline, and behavioral disturbances. In 1872 George Huntington described a familial form of chorea and named it hereditary chorea. His description, which appeared in the Philadelphia Medical and Surgical Reporter, included all the features considered diagnostic for the disease that now bears his name (1,2). Huntington’s disease is caused by a mutated autosomal dominant gene located on the short arm of chromosome 4. Persons who have a parent with Huntington’s disease have a 50 percent risk of developing the disease themselves. Genetic testing is almost 100 percent sensitive and specific for detecting the abnormal gene. The classic computed tomography finding of flattening of the lateral aspects of the lateral ventricles seen on transverse sections represents atrophic changes in the caudate nuclei and may assist the practitioner in diagnosis. However, the cornerstone of any diagnosis of Huntington’s disease is the patient’s family history (3). A patient with Huntington’s disease may present with neurological or psychiatric symptoms, or both. The movement disorder may begin with simple twitching or jerking or with clumsiness or coordination problems. As the disease progresses, the patient exhibits generalized chorea. The gait may become bizarre, including stuttering or dancing movements compounded by ataxia. In addition, frank dementia becomes apparent as the disease advances. Psychiatric signs and symptoms spanning a wide range of disturbances appear among 35 to 73 percent of patients with Huntington’s disease; 30 percent of patients meet DSM criteria for major depression (4,5). Suicide is a common cause of death, occurring at four to six times the rate in the general population. Huntington’s disease usually proves fatal between ten and 20 years after onset because of pneumonia and respiratory compromise with progressing dysphagia, which frequently lead to recurrent aspiration (3,5).