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

## TL;DR

This paper reports four cases of a rare neurological disorder called PERM, emphasizing the importance of early diagnosis and treatment for better recovery.

## Contribution

The study provides new clinical and immunological insights into PERM through a case series in China, where awareness is limited.

## Key findings

- Most PERM patients showed significant improvement with immunosuppressive therapy.
- Three out of four cases required ICU admission, indicating severe disease progression.
- Early recognition and intervention are critical for better outcomes in PERM.

## 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.