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本文对11例Wilson’s病患者的晚期神经精神障碍、青霉胺总摄入量、尸检神经病理发现和脑组织铜浓度〔mcg/g(干脑组织)〕四者的相互关系进行了研究.11例用青霉胺治疗最长达17年;结果5例症状完全缓解,6例显著改善.大部份死于肝脏病变及其并发症,仅1例因抑郁症自杀.尸检发现所有患者脑中都存在异常胶质细胞,8例壳核有空腔形成.作者综合资料认为:1.神经精神障碍和神经病理改变及青霉胺摄入量或脑铜含量基本无相关.如病例S_17和A_2,震颤和构音困难很快被青霉胺控制,临床表现正常;但尸检壳核病变显著(前部大量微小空腔形成).而W_3有明显神经精神障碍、严重扑翼样震颤,病理改变却仅有纹状体纤维变性及少量异常胶质细胞.观察其中9例,有8例用青霉胺治疗后症状明显改善,而脑铜含量仍高(如D_6、T_7、G_1和M_5分别为451、
In this paper, 11 patients with Wilson’s disease in patients with advanced neuropsychiatric disorders, penicillamine total intake, autopsy neuropathological findings and brain tissue copper concentration [mcg / g (dry brain)] four interrelationship was studied.11 Example treatment with penicillin up to 17 years; results in 5 cases of complete remission of symptoms, 6 cases improved significantly, most died of liver disease and complications, only 1 case of depression due to suicide.All post-mortem findings in all patients There are abnormal glial cells, 8 cases of the nucleus have cavity formation. The author believes that: 1. Neuropsychiatric disorders and neuropathological changes and penicillamine intake or brain copper content was basically no correlation. Case S_17 and A_2 , Tremor and dysarthria were soon penicillamine control, the clinical manifestations of normal; but autopsy nuclear putative lesions (front a large number of tiny cavities) .W_3 obvious neuropsychiatric disorders, severe flapping-wing tremor, pathological changes But only the number of striatum fibrosis and a small amount of abnormal glial cells observed in 9 cases, 8 cases with penicillin treatment significantly improved the symptoms, while the content of brain copper is still high (such as D_6, T_7, G_1 and M_5 were 451,