Abstract
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.
Highlights
BackgroundChanges in behavior are a common feature of Huntington’s disease (HD) and can cause distress to the patient as well as caretakers
Agitation is an array of symptoms and behaviors that are commonly seen in patients with psychiatric or neurological disorders
A recent case report detailed the use of electroconvulsive therapy (ECT) to treat agitation in patients with HD, whose agitation was refractory to pharmacotherapy [25]
Summary
Changes in behavior are a common feature of Huntington’s disease (HD) and can cause distress to the patient as well as caretakers. There was a significant improvement in two weeks with respect to choreiform movements and psychosis with the above doses [13] Another advanced symptomatic patient with agitation showed an improvement of agitation with aripiprazole (15 mg/day), with good control of motor symptoms without adverse effects [14]. This article highlighted the potential for the use of other long-acting injectable antipsychotic medications as a possible intervention for HD patients who cannot take oral medications or have compliance issues Another case report showed that intramuscular (IM) zuclopenthixol was more effective than paliperidone in the management of aggression [18]. A recent case report detailed the use of ECT to treat agitation in patients with HD, whose agitation was refractory to pharmacotherapy [25] They noted that after three sessions, the patient’s behavior was improved with significantly decreased verbal and physical aggression [25]. Clinicians should be mindful of the possibility of this side effect
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