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中枢与周围神经联合脱髓鞘:一例类似脑脊髓神经根神经病的病例报告

Combined central and peripheral demyelination: a case report resembling encephalomyeloradiculoneuropathy.

作者信息

Zhou Xuan, Peng Ali, Li Chuan, Li Lin, Yao Dan, Hao Yunfeng, Zhao Chao, Yan Qi, Li Ying, Liu Juntong, Liu Shuyu, Zhu Wenping, Du Ying, Zhang Wei

机构信息

Department of Neurology, Tangdu Hospital, Air Force Medical University, Xi'an, Shaanxi Province, China.

Xi'an Medical University, Xi'an, Shaanxi Province, China.

出版信息

Front Neurol. 2024 Jan 16;14:1288546. doi: 10.3389/fneur.2023.1288546. eCollection 2023.

Abstract

Combined central and peripheral demyelination (CCPD) is an extremely rare disease characterized by inflammatory demyelination in both the central and peripheral nervous systems. Herein, we reported case of a 14-year-old teenager who initially presented with the symptoms of acute myelitis (AM). Subsequently, the patient developed symptoms consistent with Guillain-Barré syndrome (GBS), which was supported by nerve conduction studies (NCV) and cerebrospinal fluid (CSF) analysis. Throughout the course of the disease, the patient experienced abdominal pain and abnormal liver function. After a comprehensive evaluation, we determined that the abnormal liver function was a result of hepatitis E virus (HEV) infection, which may have acted as a trigger for GBS. The patient was treated with corticosteroids, intravenous immunoglobulin and Rituximab, resulting in symptom relief and clinical improvement after therapy and follow-up. This case highlights the potential responsiveness and reversibility of CCPD. Given the heterogeneous nature of CCPD, there is currently no standardized diagnostic criteria or clear consensus on its treatment. Therefore, we recommend a thorough assessment of all possibilities and the development of consolidated management guidelines based on available data for this disorder.

摘要

中枢与周围联合性脱髓鞘病变(CCPD)是一种极为罕见的疾病,其特征为中枢神经系统和周围神经系统均出现炎性脱髓鞘。在此,我们报告了一例14岁青少年病例,该患者最初表现为急性脊髓炎(AM)症状。随后,患者出现了与吉兰-巴雷综合征(GBS)相符的症状,神经传导研究(NCV)和脑脊液(CSF)分析证实了这一点。在疾病过程中,患者经历了腹痛和肝功能异常。经过全面评估,我们确定肝功能异常是戊型肝炎病毒(HEV)感染所致,这可能是GBS的触发因素。患者接受了皮质类固醇、静脉注射免疫球蛋白和利妥昔单抗治疗,治疗及随访后症状缓解,临床情况改善。该病例突出了CCPD的潜在反应性和可逆性。鉴于CCPD的异质性,目前尚无标准化的诊断标准,对其治疗也没有明确的共识。因此,我们建议对所有可能性进行全面评估,并根据该疾病的现有数据制定综合管理指南。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7c8d/10825037/ad15c9ab698b/fneur-14-1288546-g001.jpg

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