Müller J, Landgraf F, Trabert W
Universitätsnervenklinik Psychiatrie-Psychotherapie, Homburg/Saar.
Nervenarzt. 1998 Mar;69(3):264-8.
Wilson's disease is a rare, autosomal recessive disorder of copper metabolism due to low serum ceruloplasm, resulting in increased copper deposition, especially in the liver and basal ganglia in the brain. The pseudosclerotic type of Wilson's disease, also known as the Westphal-Strümpell form, is distinguished by positional tremor, ataxia and dysarthria as the main symptoms. We use the example of a 23-year-old patient whose neurological symptoms were preceded by a long history of a schizophrenic-like disorder. Clinical symptoms are presented. MRI, SPECT and PET images are illustrated. Therapy and outcome are discussed.
威尔逊病是一种罕见的常染色体隐性铜代谢紊乱疾病,因血清铜蓝蛋白水平低,导致铜沉积增加,尤其是在肝脏和大脑的基底神经节。威尔逊病的假硬化型,也称为韦斯特法尔-施特吕姆佩尔型,其特征是以姿势性震颤、共济失调和构音障碍为主要症状。我们以一名23岁患者为例,其神经症状之前有很长一段类似精神分裂症的病史。展示了临床症状。展示了MRI、SPECT和PET图像。讨论了治疗方法和结果。