Oosterloo Mayke, Craufurd David, Nijsten Hanneke, van Duijn Erik
Department of Neurology, Maastricht University Medical Center, Maastricht, The Netherlands.
Division of Evolution and Genomic Sciences, School of Biological Sciences, Faculty of Biology, Medicine and Health, University of Manchester, Manchester Academic Health Science Centre, Manchester, UK.
J Huntingtons Dis. 2019;8(1):1-7. doi: 10.3233/JHD-180335.
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.
This review provides a summary of the literature on OCBs and PBs in HD gene expansion carriers (HDGECs).
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.
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.
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.
神经精神症状在亨廷顿舞蹈症（HD）中极为普遍。然而，对于该疾病进展过程中强迫行为（OCBs）和重复行为（PBs）的患病率及病程却知之甚少。
本综述总结了有关HD基因扩展携带者（HDGECs）中OCBs和PBs的文献。
在Pubmed数据库中检索截至2017年关于HD中OCBs和PBs的文章。我们使用了搜索词、HD的所有同义词以及OCBs和PBs的各种术语。
我们发现5个病例系列和11篇原创文章描述了OCBs的患病率范围为5%至52%，PBs的患病率高达75%（取决于疾病阶段和所使用的测量量表）。与对照组相比，症状前HDGECs报告的OCBs更多；与症状前HDGECs相比，症状性HDGECs报告的OCBs发生率更高。在症状性HDGECs中，OCBs和PBs与疾病持续时间延长和疾病严重程度相关，但在疾病最晚期会减少。当HDGECs接近预计运动发病时，对OCBs的同伴评分似乎高于HDGECs的自我评分。
OCBs和PBs都是HD的典型神经精神特征。重复行为可能最好与OCBs区分开来，因为它是在个体没有完全意识到或洞察到其存在的情况下发生的（并且这种行为可能不会令人痛苦）。尽管这些行为很少被区分，但我们得出结论，在HD中区分OCBs和PBs有利于HDGECs中这些症状的管理和治疗。
