Medical treatment of behavioral manifestations of Huntington disease

Abstract

Almost all patients with Huntington disease (HD) show some form of behavioral changes during the disease progression. These behavioral manifestations may already be present before cognitive or motor symptoms become apparent, and vary from subtle anxiety to severe psychosis and suicide attempts. These behavioral manifestations are related to both psychologic factors, like the burden of having a severe disease, and biologic factors that are related to the disease itself. In this chapter, both nonpharmacologic and pharmacologic interventions are discussed. Common treatment guidelines give guidance to the treatment of symptoms like depression, anxiety, and psychosis, but for more disease-specific symptoms like apathy, irritability, and obsessive-compulsive behaviors, off-label use of psychotropic medication seems to be inevitable since evidence for all interventions in HD is limited. Type and dosage of medication often depend on comorbidity, side-effects, and the use of other drugs, and because of the neurodegenerative nature of the disease, it is necessary to monitor patients clinically at a regular interval and adjust drugs accordingly as the disease progresses.

Keywords: anxiety; apathy; behavior; depression; irritability; obsessive-compulsive behaviors; psychiatric; psychosis; psychotropics.