ABSTRACT
BACKGROUND: Neuropsychiatric symptoms are highly prevalent in Huntington's disease (HD). However, little is known of the prevalence and course of obsessive-compulsive behaviors (OCBs) and perseverative behaviors (PBs) during the progression of the disease. OBJECTIVE: This review provides a summary of the literature on OCBs and PBs in HD gene expansion carriers (HDGECs). METHODS: Pubmed database was searched for articles on OCBs and PBs in HD up to 2017. We used search terms, all synonyms for HD, and various terms for OCBs and PBs. RESULTS: We found 5 case series and 11 original articles that describe a prevalence range of 5 to 52% for OCBs and up to 75% for PBs depending on disease stage and measurement scale used. Premanifest HDGECs report more OCBs compared to controls, and manifest HDGECs report a higher rate of OCBs compared to premanifest HDGECs. OCBs and PBs are associated with a longer disease duration and disease severity in manifest HDGECs, but decrease in the most advanced stages. When HDGECs come closer to estimated motor onset, the companion ratings on OCBs appear to be higher than the self-ratings of HDGECs. CONCLUSIONS: Both OCBs and PBs are characteristic neuropsychiatric features of HD. Perseveration is probably best distinguished from OCBs as it occurs without the individual's full awareness or insight into their presence (and the behavior may not be distressing). Although these behaviors are seldom distinguished, we conclude that differentiating OCBs from PBs in HD is beneficial for the management and treatment of these symptoms in HDGECs.
Subject(s)
Cognition Disorders/psychology , Huntington Disease/psychology , Obsessive-Compulsive Disorder/genetics , Adult , Aged , Behavior , Cognition Disorders/diagnosis , Cognition Disorders/genetics , Disease Progression , Female , Heterozygote , Humans , Huntington Disease/diagnosis , Huntington Disease/genetics , Male , Middle Aged , Obsessive-Compulsive Disorder/diagnosis , Obsessive-Compulsive Disorder/epidemiology , Obsessive-Compulsive Disorder/psychology , PrevalenceABSTRACT
Huntington's disease (HD) patients and families deserve expert treatment and care throughout their lives, but uniformity in functional diagnosis and treatment was lacking. In the aim of reaching this uniformity on day-to-day treatment and care offered by multidisciplinary outreach teams from Dutch long term care facilities for ambulatory HD patients, a consensus trajectory was started to harmonise our care programme with international standards and within the country. The consensus statements, given as supplementary material, should lead to expert treatment and care for HD families throughout the Netherlands and this manuscript should contribute and revitalise a global discussion on standards of treatment and care.
