Neuropsychiatric Aspects of Huntington’s Disease

Abstract

Huntington’s Disease is a progressive neurodegenerative disorder inherited in an autosomal dominant fashion with distinct phenotypesas chorea and dystonia, incoordination, cognitive disorders, and behavioural problems. In addition to cognitive and motor symptoms,neuropsychiatric symptoms are among the core symptoms of the disease. Neuropsychiatric symptoms are quite common in Huntington’sDisease and the prevalence of neuropsychiatric disorders in different stages of the disease is estimated to be 33-76%. Although the prevelanceof neuropsychiatric symptoms varies among the stages of the disease, it is also known that the onset of these symptoms may be years beforethe onset of motor symptoms. Common neuropsychiatric symptoms and disorders in Huntington’s Disease include depression, anxiety, suicide,irritability, apathy, obsessive-compulsive symptoms, perseverations, psychosis, sleep disorders, and sexual dysfunctions. Neuropsychiatricsymptoms constitute one of the most important factors for the burden on families, and are considered to be the most important predictors ofdecrease in daily functionality and institutionalizations in care centers and hospitalizations. Considering both its frequency and outcomes, it isvery important for patients, their relatives and caregivers to recognize and intervene in neuropsychiatric symptoms of Huntington’s Disease.Although there are no studies with a high level of evidence on the treatment of neuropsychiatric symptoms of the disease, studies with lowerlevels of evidence, case reports and treatment guidelines based on expert opinions are found in the literature in recent years. Another issue tobe considered in this area is the evaluation of individuals at genetic risk, genetic counseling and setting a safe protocol during these evaluations.In this article, neuropsychiatric disorders common in Huntington’s Disease, the management of these disorders and the conditions to beconsidered in the evaluation of individuals at genetic risk are reviewed in the light of current literature.

Keywords

• huntington's disease
• neuropsychiatric disorder
• treatment
• genetic risk