Huntington Hastalığı’nın Nöropsikiyatrik Yönü

Huntington’s Disease is a progressive neurodegenerative disorder inherited in an autosomal dominant fashion with distinct phenotypes as 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’s Disease and the prevalence of neuropsychiatric disorders in different stages of the disease is estimated to be 33-76%. Although the prevelance of neuropsychiatric symptoms varies among the stages of the disease, it is also known that the onset of these symptoms may be years before the 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. Neuropsychiatric symptoms constitute one of the most important factors for the burden on families, and are considered to be the most important predictors of decrease in daily functionality and institutionalizations in care centers and hospitalizations. Considering both its frequency and outcomes, it is very 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 lower levels of evidence, case reports and treatment guidelines based on expert opinions are found in the literature in recent years. Another issue to be 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 be considered in the evaluation of individuals at genetic risk are reviewed in the light of current literature.