Combined central and peripheral demyelination: a case report resembling encephalomyeloradiculoneuropathy

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

doi:10.3389/fneur.2023.1288546

Combined central and peripheral demyelination: a case report resembling encephalomyeloradiculoneuropathy

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

Authors

Xuan Zhou¹, Ali Peng¹, Chuan Li¹, Lin Li¹, Dan Yao¹, Yunfeng Hao¹, Chao Zhao¹, Qi Yan¹, Ying Li¹, Juntong Liu^{1

2}, Shuyu Liu^{1

2}, Wenping Zhu^{1

2}, Ying Du¹, Wei Zhang¹

Affiliations

¹ Department of Neurology, Tangdu Hospital, Air Force Medical University, Xi'an, Shaanxi Province, China.
² Xi'an Medical University, Xi'an, Shaanxi Province, China.

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.

Keywords: Guillain-Barré syndrome; HEV infection; acute myelitis; combined central and peripheral demyelination; treatment.

Publication types

Case Reports

Grants and funding

The author(s) declare financial support was received for the research, authorship, and/or publication of this article. This work was supported by research grants from the National Natural Science Foundation of China (81971003, 82171406, and 82271457), Shaanxi Province Health Research and Innovation Team for Cognitive Dysfunction Disease (2023TD-06), Tangdu Hospital Innovation Development Foundation (2018QYTS010, 2019QYTS002), Tangdu Hospital Clinical Research Project (2021LCYJ040), and Tangdu hospital foundation for social recruitment talent (2021SHRC011).