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Induction and Clinical Scoring of Chronic-Relapsing Experimental Autoimmune Encephalomyelitis
Published on: July 4, 2007
Progressive encephalomyelitis with rigidity: A Taiwanese case and review of literature
Anna Chang1, Kuan-Yu Lin1, Kai-Ju Chuang1
1Department of Neurology, Shin Kong Wu Ho-Su Memorial Hospital, No. 95, Wenchang Road, Shilin District, Taipei, Taiwan.
Introduction:
Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a rare disorder. However, the outcome is still variable with different serological and tumor associations, and the elements to good response with less relapse is yet to be elucidated.
Method:
We present a case and obtain a literature review of patients with PERM and make comparisons based on different serological groups. We also analyze patients with idiopathic PERM that had detailed medical records.
Results:
81 patients were collected and analyzed. The largest group were glycine receptor-antibody (GlyR-Ab)-positive (70%), and the seropositive-GlyR-Ab-negative group had better response to immunotherapy. Malignancy can occur up to 2 years from the presentation of PERM. Among the 18 cases with detailed records, the patients who had good outcome initiate immunotherapy within 2 months from presentation. 9 of the 12 patients who experienced no relapse had non-steroid immunotherapy. The maximal interval time of relapse was 24 months.
Conclusion:
We recommend tumor surveillance up to 2 years in patients with PERM and early administration of immunotherapies and maintain with non-steroid immunotherapy with or without oral corticosteroid for a minimum of 2 years to reduce the risk of relapse in GlyR-Ab-positive patients.
Insights
Early immunotherapy and long-term non-steroid treatment are recommended for progressive encephalomyelitis with rigidity and myoclonus (PERM), especially in glycine receptor-antibody positive patients. Tumor surveillance is advised for up to two years to minimize relapse risk.
Area of Science:
- Neurology
- Immunology
- Oncology
Background:
- Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a rare neurological disorder with variable outcomes.
- Understanding factors influencing treatment response and relapse is crucial for managing PERM.
- Associations with specific antibodies and malignancy require further elucidation.
Observation:
- A literature review analyzed 81 patients with PERM, with 70% testing positive for glycine receptor antibodies (GlyR-Ab).
- Seropositive-GlyR-Ab-negative PERM patients showed better immunotherapy response.
- Malignancy was associated with PERM, occurring up to two years prior to presentation.
Findings:
- Early immunotherapy (within 2 months) correlated with better outcomes in 18 detailed cases.
- Non-steroid immunotherapy was associated with no relapse in 9 of 12 patients.
- Relapses occurred up to 24 months after initial presentation.
Implications:
- Recommend tumor surveillance for up to two years in PERM patients.
- Advocate for early immunotherapy administration and a minimum of two years of non-steroid immunotherapy (with or without oral corticosteroids) to reduce relapse risk in GlyR-Ab-positive PERM.
- Highlight the importance of timely and sustained immunomodulatory treatment strategies.
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