Huntington Hastalığı’nın Nöropsikiyatrik Yönü

Abstract

Huntington Hastalığı; kore ve distoni, koordinasyon bozukluğu, bilişsel performansta bozulmalar ve davranışsal sorunlar gibi farklı
fenotipler ile ortaya çıkabilen, genetik olarak otozomal dominant geçiş özelliğine sahip, ilerleyici tipte bir nörodejeneratif hastalıktır. Bilişsel
ve motor belirtilerin yanı sıra nöropsikiyatrik belirtiler de hastalığın çekirdek belirtileri arasında yer almaktadır. Huntington Hastalığı’nda
nöropsikiyatrik belirtiler oldukça sık görülmekte ve hastalığın farklı dönemlerinde psikiyatrik bozuklukların görülme prevelansı %33-76 olarak
tahmin edilmektedir. Nöropsikiyatrik belirtilerin görülme sıklığı hastalığın evrelerine göre farklılık gösterse de başlangıcının motor belirtiler
başlamadan yıllar önce olabileceği de bilinmektedir. Huntington Hastalığı’nda sık görülen nöropsikiyatrik belirti ve bozukluklar depresyon,
anksiyete, intihar, irritabilite, apati, obsesif-kompulsif belirtiler, perseverasyonlar, psikoz, uyku bozuklukları ve cinsel işlev bozuklukları olarak
sayılabilir. Nöropsikiyatrik belirtiler aileler üzerindeki yükün en önemli nedenlerinden birini oluşturmakta, günlük işlevsellikteki azalma ile
bakım kurumlarına yerleştirilme ve hastaneye yatışların en önemli öngörücüsü olarak değerlendirilmektedir. Hem sıklığı hem de sonuçları
göz önüne alındığında Huntington Hastalığı’ndaki nöropsikiyatrik belirtilerin tanınması ve bu belirtilere müdahale edilmesi hastalar, hasta
yakınları ve bakımverenleri için oldukça önemlidir. Hastalıkta görülen nöropsikiyatrik belirtilerin tedavisi ile ilgili yüksek kanıt düzeyine
sahip araştırmalar olmasa da daha düşük kanıt düzeyine sahip çalışmalar, vaka bildirimleri ve uzman görüşlerine dayalı tedavi kılavuzları
son yıllarda yazında kendine yer bulmuştur. Bu alanda dikkat edilmesi gereken başka bir konu da risk altındaki bireylerin değerlendirilmesi,
genetik danışmanlık ve bu değerlendirmeler sırasında güvenli bir protokolün oluşturulmasıdır. Bu yazıda Huntington Hastalığı’nda sık görülen
nöropsikiyatrik bozukluklar, bu bozuklukların tedavisi ve risk altındaki bireyleri değerlendirmede dikkat edilmesi gereken durumlar güncel
yazın ışığında derlenmiştir.

Keywords

• huntington hastalığı
• nöropsikiyatrik bozukluk
• tedavi
• genetik risk

Neuropsychiatric Aspects of Huntington’s Disease

Abstract

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.

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