Janati A
J Nerv Ment Dis. 1985 Oct;173(10):632-5. doi: 10.1097/00005053-198510000-00010.
In this paper the first case of Kluver-Bucy syndrome (KBS) in Huntington's chorea is reported. The patient, a 46-year-old man with advanced Huntington's disease, displayed prosopagnosia, oral tendencies, emotional changes, hypersexual behavior, and hyperphagia associated with severe dementia. Haloperidol in moderate doses controlled both the KBS and the chorea, suggesting a possible role for the dopaminergic system in the pathogenesis of KBS in Huntington's disease. The presence of profound dementia in our patient supports the previous assertion that human cases of KBS are invariably associated with severe cognitive dysfunction. Since KBS was established as an entity, a great deal of attention has been directed to its neuroanatomical basis. However, due to the multidetermined nature of human behavior, the role of physiological, psychological, and environmental factors should also be taken into consideration with regard to the pathogenesis of this syndrome.
本文报道了亨廷顿舞蹈病中首例克吕弗-布西综合征（KBS）。该患者为一名46岁患有晚期亨廷顿病的男性，表现出人面失认症、口部趋向、情绪变化、性行为亢进及食欲亢进，并伴有严重痴呆。中等剂量的氟哌啶醇控制了KBS和舞蹈病症状，提示多巴胺能系统在亨廷顿病KBS发病机制中可能发挥作用。我们患者中存在的严重痴呆支持了之前的论断，即人类KBS病例总是与严重认知功能障碍相关。自从KBS被确认为一种病症以来，大量关注都指向了其神经解剖学基础。然而，由于人类行为的多因素决定性质，在该综合征的发病机制方面，生理、心理和环境因素的作用也应予以考虑。
