OBJECTIVE Huntington disease (HD) is an autosomal dominant neurodegenerative disease involving motor disturbances, cognitive decline and psychiatric symptoms. Psychotic symptoms occur in a significant proportion of patients. We sought to characterise the clinical outcomes of this group of patients. METHODS Data were drawn from the Cooperative Huntington Observational Research Trial, a prospective, multi-centre observational study. 1082 patients with HD were recruited. Measures of cognition, function, behavioural disturbance and motor function were completed annually over 5 years. RESULTS Overall, 190 patients (17.6%) displayed psychotic symptoms. These patients demonstrated worse cognition, function and behavioural disturbances than patients without psychosis over time. Patients with psychosis also demonstrated lower levels of chorea than patients without psychosis, despite adjusting for concurrent antipsychotic and tetrabenazine use. CONCLUSIONS Psychosis in HD is associated with poorer outcomes in cognition, function and behavioural symptoms. Patients with psychotic symptoms may also have less chorea. Altogether, the findings suggest patients with psychosis have a distinct clinical course.

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亨廷顿病的精神病和纵向预后：COHORT研究。

目的亨廷顿病（HD）是一种常染色体显性遗传神经退行性疾病，涉及运动障碍，认知能力下降和精神病症状。很大一部分患者会出现精神病性症状。我们试图表征这组患者的临床结局。方法数据来自亨廷顿合作观察研究试验，这是一项前瞻性，多中心观察研究。招募了1082例HD患者。认知，功能，行为障碍和运动功能的测量每年超过5年完成。结果总体上，有190例患者（17.6％）出现精神病症状。与没有精神病的患者相比，这些患者随着时间的推移表现出较差的认知，功能和行为障碍。尽管调整了同时使用抗精神病药和丁苯那嗪的治疗方法，但精神病患者的舞蹈病水平也低于无精神病患者。结论HD的精神病与认知，功能和行为症状的预后较差有关。有精神病症状的患者可能也有较少的舞蹈症。总的来说，这些发现表明精神病患者具有独特的临床过程。