Brewer G J, Terry C A, Aisen A M, Hill G M
Arch Neurol. 1987 May;44(5):490-3. doi: 10.1001/archneur.1987.00520170020016.
We describe a patient with Wilson's disease who presented with neurologic disease, was treated with D-penicillamine, and suffered sudden neurologic deterioration coincident with therapy. Replicate brain magnetic resonance imaging examinations after six weeks and 11 months of penicillamine therapy documented the development of new brain lesions during this period, while liver biopsy specimen data disclosed that excellent hepatic decoppering had occurred. To develop information on the relative rarity or frequency of neurologic worsening with the initiation of penicillamine therapy, we conducted a retrospective survey of 25 additional patients with Wilson's disease who met the criteria of presenting with neurologic disease and having been treated with penicillamine. The replies indicate that, at least from the patient's viewpoint, this syndrome occurs frequently. We suggest that the cause of this distressing syndrome, and ways to mitigate or circumvent it, must be discovered.
我们描述了一名患有威尔逊氏病的患者，该患者出现神经系统疾病，接受了青霉胺治疗，且在治疗期间突然出现神经系统恶化。在青霉胺治疗6周和11个月后进行的重复脑磁共振成像检查记录了在此期间新脑病变的出现，而肝活检标本数据显示肝脏已实现良好的排铜。为了获取关于开始使用青霉胺治疗后神经系统恶化的相对罕见性或频率的信息，我们对另外25名符合出现神经系统疾病并接受青霉胺治疗标准的威尔逊氏病患者进行了回顾性调查。回复表明，至少从患者的角度来看，这种综合征很常见。我们建议必须找出这种令人苦恼的综合征的病因以及减轻或规避它的方法。
