CASE REPORT article
Front. Immunol.
Sec. Autoimmune and Autoinflammatory Disorders : Autoimmune Disorders
Volume 16 - 2025 | doi: 10.3389/fimmu.2025.1616207
Clinical Features of Progressive Encephalomyelitis with Rigidity and Myoclonus: Case Series
Provisionally accepted
- Department of Neurology, Second Xiangya Hospital, Central South University, Changsha, China
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Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a rare neurological disorder characterized by rigidity, painful spasms, hyperekplexia, brainstem involvement, and autonomic dysfunction. In China, limited awareness and delayed antibody testing often hinder early diagnosis. Here, we report four cases of PERM, three of which required intensive care unit (ICU) admission. The clinical and immunological features of these patients were systematically summarized. This case series highlights the characteristic clinical and immunological profiles of PERM and underscores the importance of early recognition and timely intervention, as most patients achieve substantial recovery with immunosuppressive therapy.
Keywords: progressive encephalomyelitis with rigidity and myoclonus, stiff person syndrome, glycine receptor, GAD65, Brainstem dysfunction
Received: 23 Apr 2025; Accepted: 17 Oct 2025.
Copyright: © 2025 Chen and Qin. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence: Qihua Chen, chenqihua1984@163.com
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