Progressive encephalomyelitis with rigidity: A Taiwanese case and review of literature.

Autor: Chang A; Department of Neurology, Shin Kong Wu Ho-Su Memorial Hospital, No. 95, Wenchang Road, Shilin District, Taipei, Taiwan., Lin KY; Department of Neurology, Shin Kong Wu Ho-Su Memorial Hospital, No. 95, Wenchang Road, Shilin District, Taipei, Taiwan., Chuang KJ; Department of Neurology, Shin Kong Wu Ho-Su Memorial Hospital, No. 95, Wenchang Road, Shilin District, Taipei, Taiwan., Waters P; Oxford Autoimmune Neurology Diagnostic Laboratory, Nuffield Department of Clinical Neurosciences, University of Oxford, Level 6, West Wing, John Radcliffe Hospital, Oxford OX3 9DU, UK., Irani S; Oxford Autoimmune Neurology Diagnostic Laboratory, Nuffield Department of Clinical Neurosciences, University of Oxford, Level 6, West Wing, John Radcliffe Hospital, Oxford OX3 9DU, UK., Mgbachi V; Oxford Autoimmune Neurology Diagnostic Laboratory, Nuffield Department of Clinical Neurosciences, University of Oxford, Level 6, West Wing, John Radcliffe Hospital, Oxford OX3 9DU, UK., Yeh HL; Department of Neurology, Shin Kong Wu Ho-Su Memorial Hospital, No. 95, Wenchang Road, Shilin District, Taipei, Taiwan., Lien LM; Department of Neurology, Shin Kong Wu Ho-Su Memorial Hospital, No. 95, Wenchang Road, Shilin District, Taipei, Taiwan., Chiu HC; Department of Neurology, Shin Kong Wu Ho-Su Memorial Hospital, No. 95, Wenchang Road, Shilin District, Taipei, Taiwan., Chen WH; Department of Neurology, Shin Kong Wu Ho-Su Memorial Hospital, No. 95, Wenchang Road, Shilin District, Taipei, Taiwan. Electronic address: bian_na23@hotmail.com.
Jazyk: angličtina
Zdroj: Clinical neurology and neurosurgery [Clin Neurol Neurosurg] 2021 Sep; Vol. 208, pp. 106807. Date of Electronic Publication: 2021 Jul 13.
DOI: 10.1016/j.clineuro.2021.106807
Abstrakt: 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.
(Copyright © 2021 Elsevier B.V. All rights reserved.)
Databáze: MEDLINE
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