Dening T R, Berrios G E
Department of Psychiatry, University of Cambridge, England.
Arch Gen Psychiatry. 1989 Dec;46(12):1126-34. doi: 10.1001/archpsyc.1989.01810120068011.
A series of 195 cases of Wilson's disease were assessed retrospectively on a range of variables, including psychiatric, neurologic, and hepatic symptoms, and biochemical data as recorded at first admission to a specialist clinic. Ninety-nine patients (51%) were rated as displaying some evidence of psychopathologic features, and 39 (20%) had seen a psychiatrist before the diagnosis of Wilson's disease. The most common psychiatric features were abnormal behavior and personality change, although depression and cognitive impairment were also rated frequently. Schizophrenialike psychoses were rare, apparently occurring at no more than chance frequency. Psychiatric symptoms were related to neurologic rather than hepatic symptoms, and certain symptoms (incongruous behavior, irritability, and personality change) had a particularly significant relationship with bulbar and dystonic disorders but not with tremor. Psychiatric manifestations are important in Wilson's disease, and many of the psychopathologic features seem to have an organic basis.
对195例肝豆状核变性患者进行了回顾性评估，涉及一系列变量，包括精神、神经和肝脏症状，以及首次入住专科诊所时记录的生化数据。99名患者（51%）被评定有一些精神病理特征的证据，39名（20%）在肝豆状核变性诊断之前看过精神科医生。最常见的精神特征是行为异常和人格改变，不过抑郁和认知障碍也经常被评定。类精神分裂症性精神病很少见，显然其发生率不超过偶然概率。精神症状与神经症状而非肝脏症状相关，某些症状（行为不协调、易怒和人格改变）与延髓和张力障碍有特别显著的关系，但与震颤无关。精神表现在肝豆状核变性中很重要，许多精神病理特征似乎有器质性基础。
