Guarino M, Stracciari A, D'Alessandro R, Pazzaglia P
Department of Neurology, S. Orsola-Malpighi Hospital, Bologna, Italy.
Acta Neurol Scand. 1995 Nov;92(5):405-8. doi: 10.1111/j.1600-0404.1995.tb00155.x.
Orthotopic liver transplantation (OLT) represents the sole etiological treatment for Wilson's disease (WD), but its efficacy in resolving the neurological symptoms is still a matter of debate. We present the case of a young male affected with WD with hepatic and neurological involvement, who underwent OLT for subacute liver failure. Clinical, neuropsychological and neuroradiological assessment were performed before and after OLT. OLT had no effect on neurological symptoms, suggesting that the basal ganglia damage may be irreversible. Nineteen months after OLT, the patient went on to develop new permanent extrapyramidal symptoms. We postulate a sequela of an early post-operative central pontine and extrapontine myelinolysis.
原位肝移植（OLT）是威尔逊病（WD）唯一的病因治疗方法，但其在缓解神经症状方面的疗效仍存在争议。我们报告了一例患有WD且有肝脏和神经受累的年轻男性病例，该患者因亚急性肝衰竭接受了OLT。在OLT前后进行了临床、神经心理学和神经放射学评估。OLT对神经症状没有影响，这表明基底神经节损伤可能是不可逆的。OLT后19个月，患者继而出现了新的永久性锥体外系症状。我们推测这是术后早期发生的中央桥脑和脑桥外髓鞘溶解的后遗症。
