Work

Clinical features of progressive encephalomyelitis with rigidity and myoclonus: Case Report

Year: 2025

Type: article

Abstract: 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.

Source: Frontiers in Immunology

DOI: 10.3389/fimmu.2025.1616207

Authors: Lixia Qin, Qihua Chen

Institutions: Central South University, Second Xiangya Hospital of Central South University, Xiangya Hospital Central South University

Language: English

Topic: Autoimmune Neurological Disorders and Treatments

Subfield: Neurology

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: gold

Estimated APC paid: $3,801