急性播散性脑脊髓炎合并多发性神经病的临床研究  

Clinical study of acute disseminated encephalomyelitis with polyneuropathy

作　　者：臧卫周[1] 杨红 张杰文[1] ZANG Weizhou;YANG Hong;ZHANG Jiewen(Henan Provincial People’s Hospital,Zhengzhou 450003,China;Shangqiu First People’s Hospital,Shangqiu 476000,China)

机构地区：[1]河南省人民医院,河南郑州450003 [2]商丘市第一人民医院,河南商丘476000

出　　处：《中国实用神经疾病杂志》2021年第5期414-419,共6页Chinese Journal of Practical Nervous Diseases

基　　金：2015年河南省科技攻关计划项目(编号:1521023101400)。

摘　　要：目的分析急性播散性脑脊髓炎合并多发性神经病的临床特点,以提高临床医生对本病的认识和诊疗水平。方法对2017-12—2019-04就诊于河南省人民医院的8例急性播散性脑脊髓炎合并多发性神经病患者的临床表现、影像学、神经电生理、脑脊液特点及全身免疫相关指标进行回顾性分析。结果本组8例患者为急性或亚急性起病,起病前多有前驱感染史。首先表现为中枢神经系统受累,其中7例影像学证实脑和(或)脊髓白质受累,1例脑电图证实中枢神经系统受损;迟缓性瘫痪、腱反射减弱或消失等周围神经受损的证据也随后出现,而后肌电图证实多发性神经根受损或神经源性损害。脑脊液检查细胞轻度升高或正常,蛋白升高或正常,只有1例见脑脊液细胞-蛋白分离。治疗上急性期激素冲击或免疫球蛋白治疗,症状和体征明显改善,缓解期辅以免疫抑制剂,随访无复发。结论中枢神经系统和周围神经系统同时或相继出现脱髓鞘性改变,可能是一种不同于单纯中枢神经系统脱髓鞘和周围神经系统脱髓鞘的新疾病体,也可能是一种新的叠加综合征。Objective To analyze the clinical characteristics of acute disseminated encephalomyelitis with polyneuropathy,so as to improve the clinician’s understanding,diagnosis and treatment level of the disease.Methods The clinical manifestations,radiological data,neurophysiological finding,cerebrospinal fluid features and systemic immunoassay of 8 patients with acute disseminated encephalomyelitis complicated with polyneuropathy,admitted to our hospital from December 2017 to April 2019,were retrospectively analyzed.Results The 8 patients were acute or subacute onset,most patients had a history of infection.The first manifestation is the central nervous system involvement symptoms,which has been confirmed the brain and(or)spinal cord white matter involvement in seven cases,and one patients was confirmed central nervous system damage by electroencephalogram(EEG).Delayed paralysis,loss of tendon reflex and other symptoms of peripheral nerve lesion subsequently appeared,then multiple nerve root injury and neurogenic damage were confirmed by electromyography(EMG).The cerebrospinal fluid(CSF)cells were slightly elevated or normal and the protein increased or normal,only one case has dissociation of protein from cell in cerebrospinal fluid.In acute stage,steroid pulsed immunotherapy and/or high dose of IV immunoglobulin replacement therapy resulted in clear and often dramatic clinical improvements,and the immunosuppressive agents were used in the remission stage.No recurrence was found in the follow-up.Conclusion The demyelination concurrently or sequentially occuring in the central and peripheral nervous system,which may be a new disease entity and is different from the independent central nervous system or peripheral nervous system demyelination.It may also be a new of the superposition syndrome.

关 键 词：急性播散性脑脊髓炎 多发性神经病 吉兰-巴雷综合征 脱髓鞘疾病 肌电图特征 

分 类 号：R744.53[医药卫生—神经病学与精神病学]