Folstein S E, Folstein M F
Psychiatr Dev. 1983 Summer;1(2):193-205.
The psychiatric manifestations of Huntington's Disease (HD) include dementia, irritability and apathy, a major affective syndrome, and hallucinosis. The theoretical and practical utility of chorea as a focus of research interest in HD is questioned, whereas the data reviewed suggest that assessments of cognition, functional capacity and motor impairment are better correlated neuropathologically, and are better indicators of disease severity and progress than chorea. The high incidence of major affective disorders on modified DSM III criteria among HD patients (41 per cent) may be explained either as a manifestation of genetic heterogeneity within the HD phenotype or on the basis of genetic linkage between HD and manic depressive illness (MDI). This is supported by the high coincidence of HD and MDI (20 out of 23) among secondary cases of HD ascertained through probands having both disorders, indicating a strong familial clustering of the association. This implies that a young adult at risk for HD who has had episodes of severe depression has considerably more than 50 per cent likelihood of progressing to manifest HD. Although auditory hallucinations appear occasionally in patients with HD, most do not meet current criteria for schizophrenia.
亨廷顿舞蹈症(HD)的精神症状包括痴呆、易怒和冷漠、一种主要的情感综合征以及幻觉症。舞蹈症作为HD研究兴趣焦点的理论和实际效用受到质疑,而所回顾的数据表明,认知、功能能力和运动障碍的评估在神经病理学上具有更好的相关性,并且比舞蹈症更能准确反映疾病的严重程度和进展情况。根据修订后的《精神疾病诊断与统计手册》第三版标准,HD患者中主要情感障碍的高发病率(41%),既可以解释为HD表型内遗传异质性的一种表现,也可以基于HD与躁郁症(MDI)之间的遗传联系来解释。通过患有这两种疾病的先证者确定的HD继发病例中,HD与MDI的高重合率(23例中有20例)支持了这一点,表明这种关联存在强烈的家族聚集性。这意味着,有HD风险的年轻成年人若曾有过严重抑郁发作,那么其发展为HD的可能性超过50%。虽然HD患者偶尔会出现幻听,但大多数不符合目前精神分裂症的诊断标准。