Lingam S, Wilson J, Nazer H, Mowat A P
Neuropediatrics. 1987 Feb;18(1):11-2. doi: 10.1055/s-2008-1052427.
The therapeutic responses of seven children with Wilson's disease who presented with neurological disease were evaluated. Neurological abnormalities comprised intellectual deterioration in 7, conduct disorder in five, dystonia in three, choreoathetosis in three, seizures in one and hemiparesis in one. Lethargy and weight loss were present for several months in 6 children. Four children had clinically demonstrable liver disease which was fatal in two. Electroencephalography performed in two children was normal. Computed tomography (CT) of the brain in three children showed cerebral atrophy in all and areas of low attenuation in the basal ganglia which resolved on treatment in one. All patients were treated with penicillamine but, in four, triethylene tetramine (TETA) was substituted because of adverse effects. Neurological abnormalities in these patients were reversible.
对七名患有威尔逊氏病且伴有神经疾病的儿童的治疗反应进行了评估。神经异常包括7名儿童智力衰退、5名儿童品行障碍、3名儿童肌张力障碍、3名儿童舞蹈手足徐动症、1名儿童癫痫发作和1名儿童偏瘫。6名儿童出现了数月的嗜睡和体重减轻。4名儿童有临床可证实的肝病，其中2例死亡。对两名儿童进行的脑电图检查正常。对三名儿童进行的脑部计算机断层扫描（CT）显示，所有人都有脑萎缩，基底神经节有低密度区，其中一名经治疗后消退。所有患者均接受青霉胺治疗，但有4名患者因不良反应改用三亚乙基四胺（TETA）。这些患者的神经异常是可逆的。
