肝移植术后脑桥外和脑桥中央髓鞘溶解症——附8例报告

摘要

Objective To investigate the pathogenesis, clinical characteristics and prognosis of central pontine and extrapontine myelinolysis following liver transplantation.Methods 8 cases diagnosed as central pontine and extrapontine myelinolysis following liver transplantation were retrospectively analyzed.Results Aphasia, dysphagia, epilepsy and coma were observed in early period of post liver transplantation.Among the 8 cases, 2 cases were restored to consciousness with mental disorder, 4 cases were still in coma until discharged ,and 2 cases died of MODS.Conclusion The main pathogenesis of central pontine and extrapontine myelinolysis following liver transplantation is severe change of plasma osmotic pressure with diversity of clinical characteristics and poor prognosis.Combination treatment with plasmapheresis and intravenous immunoglobulin may improve neurologic outcome.%目的 探讨肝移植术后脑桥外和脑桥髓鞘溶解症的发病机制、临床表现和预后.方法 回顾性分析8例肝移植术后发生脑桥外和脑桥髓鞘溶解症患者临床诊治经过和预后.结果 本组患者肝移植术后3～11d发生失语、吞咽困难、癫痫、昏迷,头部MRI提示脑干、基底节、脑叶脱髓鞘改变,经治疗后2例神志清楚但存在智力障碍,4例仍昏迷,2例因并发多器官功能衰竭死亡.结论 围手术期血浆渗透压的显著波动是移植术后脑桥外和脑桥髓鞘溶解症的主要病因,临床表现为术后早期发生失语、吞咽困难、癫痫、昏迷等神经系统症状,头部MRI提示脑干、基底节、脑叶脱髓鞘改变,治疗上以脏器功能支持为主,血浆置换联合免疫球蛋白治疗可以改善预后.