Cardoso Francisco
UFMG, Belo Horizonte, Brazil.
Int Rev Neurobiol. 2017;134:1397-1408. doi: 10.1016/bs.irn.2017.05.004. Epub 2017 Jun 1.
Huntington disease (HD) is characterized by the triad of motor abnormalities, cognitive dysfunction, and behavioral changes. The aim of this chapter is to describe the frequency, clinical features, and management of behavioral and cognitive dysfunction in HD. Depression, suicidal ideation, apathy, irritability, aggressiveness, obsessions, and compulsions are the most common behavioral abnormalities in HD. All HD patients develop cognitive decline. Recent data suggest that these nonmotor changes are found in premanifest HD gene carriers. Finally, we review the therapeutic options available for behavioral and cognitive abnormalities in HD. The management of these conditions is based on experts' agreement since there is a lack of empirical data.
亨廷顿舞蹈症(HD)的特征为运动异常、认知功能障碍和行为改变三联征。本章旨在描述HD中行为和认知功能障碍的发生率、临床特征及管理。抑郁、自杀观念、冷漠、易怒、攻击性、强迫观念和强迫行为是HD中最常见的行为异常。所有HD患者都会出现认知衰退。最近的数据表明,这些非运动性改变在HD基因携带者的症状前阶段就已出现。最后,我们回顾了HD中行为和认知异常的可用治疗选择。由于缺乏实证数据,这些病症的管理基于专家共识。