Progressive encephalomyelitis with rigidity: a Taiwanese case and review of literature
<p><strong>Introduction</strong></p> 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...
| Autors principals: | , , , , , , , , , |
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| Format: | Journal article |
| Idioma: | English |
| Publicat: |
Elsevier
2021
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| _version_ | 1826317369274793984 |
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| author | Chang, A Lin, K-Y Chuang, K-J Waters, P Irani, SR Mgbachi, V Yeh, H-L Lien, L-M Chiu, H-C Chen, W-H |
| author_facet | Chang, A Lin, K-Y Chuang, K-J Waters, P Irani, SR Mgbachi, V Yeh, H-L Lien, L-M Chiu, H-C Chen, W-H |
| author_sort | Chang, A |
| collection | OXFORD |
| description | <p><strong>Introduction</strong></p>
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.
<p><strong>Method</strong></p>
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.
<p><strong>Results</strong></p>
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.
<p><strong>Conclusion</strong></p>
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. |
| first_indexed | 2025-02-19T04:38:57Z |
| format | Journal article |
| id | oxford-uuid:10907de9-f6e3-4a8c-a4a5-053898eb5b94 |
| institution | University of Oxford |
| language | English |
| last_indexed | 2025-02-19T04:38:57Z |
| publishDate | 2021 |
| publisher | Elsevier |
| record_format | dspace |
| spelling | oxford-uuid:10907de9-f6e3-4a8c-a4a5-053898eb5b942025-02-14T11:17:29ZProgressive encephalomyelitis with rigidity: a Taiwanese case and review of literatureJournal articlehttp://purl.org/coar/resource_type/c_dcae04bcuuid:10907de9-f6e3-4a8c-a4a5-053898eb5b94EnglishSymplectic ElementsElsevier2021Chang, ALin, K-YChuang, K-JWaters, PIrani, SRMgbachi, VYeh, H-LLien, L-MChiu, H-CChen, W-H<p><strong>Introduction</strong></p> 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. <p><strong>Method</strong></p> 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. <p><strong>Results</strong></p> 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. <p><strong>Conclusion</strong></p> 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. |
| spellingShingle | Chang, A Lin, K-Y Chuang, K-J Waters, P Irani, SR Mgbachi, V Yeh, H-L Lien, L-M Chiu, H-C Chen, W-H Progressive encephalomyelitis with rigidity: a Taiwanese case and review of literature |
| title | Progressive encephalomyelitis with rigidity: a Taiwanese case and review of literature |
| title_full | Progressive encephalomyelitis with rigidity: a Taiwanese case and review of literature |
| title_fullStr | Progressive encephalomyelitis with rigidity: a Taiwanese case and review of literature |
| title_full_unstemmed | Progressive encephalomyelitis with rigidity: a Taiwanese case and review of literature |
| title_short | Progressive encephalomyelitis with rigidity: a Taiwanese case and review of literature |
| title_sort | progressive encephalomyelitis with rigidity a taiwanese case and review of literature |
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