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肝移植后脑桥中央髓鞘溶解症5例
引用本文:张舒凤,崔莉莉,闫四梅,李正军,吴士文,马维娅.肝移植后脑桥中央髓鞘溶解症5例[J].疑难病杂志,2009,8(10):587-590.
作者姓名:张舒凤  崔莉莉  闫四梅  李正军  吴士文  马维娅
作者单位:武警总医院神经内科,北京,100039
摘    要:目的总结原位肝移植术后脑桥中央髓鞘溶解症(CPM)的诊治体会。方法回顾性分析5例原位肝移植术后并发CPM的临床资料。结果4例为肝炎后肝硬化失代偿期患者,1例为重型肝炎患者。术前均有持续低血钠,原位肝移植术后48 h内血钠纠正至正常范围或高于正常。CPM症状出现时间在术后1~2周,主要临床表现有精神异常、不同程度的意识障碍、言语不清及肢体活动障碍,1例伴有抽搐。5例患者均经头颅MRI检查证实为脑桥中央髓鞘溶解症,其中2例合并脑桥外髓鞘溶解(EPM)。经积极对症支持治疗,5例患者无1例死亡,4例患者神经系统症状明显改善,1例患者恢复较差,随访5个月仍处于昏迷状态。结论肝移植后发生CPM并非罕见,它可能是多种因素共同作用的结果,头颅MRI是主要确诊依据,脑电图可协助判断预后,脑脊液无特异性改变。CPM虽预后较差,但并非不可逆。

关 键 词:肝移植  脑桥中央髓鞘溶解症  低钠血症

Central pontine myelinolysis after liver transplantation:5 cases report
Institution:ZHANG Shu-feng, CUI Li-li, YAN Si- mei,et al.(Department of Neurology, General Hospital Of Chinese People' s Armed Police Forces, Beijing 100039, China.)
Abstract:Objective To smnmarize the characteristic of central pontine myelinolysis (CPM) after orthotopic liver transplantation (OLT). Methods The clinical data of five cases with CPM after orthotopic liver transplantation were collected and analyzed retrospectively. Results Four of 5 cases were posthepatilic cirrhosis, and 1 case was severity hepatitis. All cases were continuously hyponatremia before operation, and serum sodium concentration rised rapidly to normal level or higher 48 h after OLT. Symptoms started at 1 to 2 weeks after operation, which included psychiatric symptoms, conscious disturbance, dysarthria, limb disability and convulsion in one case. Five cases were diagnosed as CPM confirmed by magnetic resonance image ( MRI), and 2 cases combined with EPM. After positive symptomatic management and support, 4 cases improved significantly and one was in coma constantly after 5 months follow up. Conclusion CPM is not a rare complication after liver transplantation and it owed to widly array of etiologies. The MRI was the main evidence to diagonisis CPM,EEG may aid in the prognosis appraisal. The CSF had no specific change in CPM. Symptoms of CPM are not inreversible though its bad prognosis.
Keywords:Liver transplantation  Central pontine myelinolysis  Hyponatremia
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