A Review of the Etiology and Pathogenesis of Posterior Reversible Encephalopathy Syndrome (PRES) in Patients with Multiple Sclerosis (MS) and Neuromyelitis Optica Spectrum Disorder (NMOSD)

Abstract

Background: Posterior Reversible Encephalopathy Syndrome (PRES) is a neurological disorder with multifaceted pathophysiology, typically presenting with vasogenic edema on brain imaging. Although the occurrence of PRES in patients with MS and NMOSD is rare, identifying the etiological factors and pathogenesis mechanisms in these patients is crucial for optimal diagnosis and management. Methods: This study was conducted through a literature search of PubMed, Scopus, Web of Science, and Google Scholar databases using relevant keywords. Findings: Seventeen relevant case reports were identified and reviewed. In NMOSD patients, the pathogenesis of PRES is primarily associated with blood-brain barrier (BBB) dysfunction caused by the attack of anti-AQP4 IgG antibodies on astrocytes. Furthermore, common treatments for this disease, such as rituximab (which induces endothelial injury), corticosteroids, and plasmapheresis (which induces blood pressure fluctuations), have been identified as significant predisposing risk factors for PRES. In MS patients, perivascular inflammation and impaired neurovascular coupling at the blood-brain barrier provide a suitable basis for the development of PRES. Medications such as fingolimod (via an endothelial injury mechanism) and interferon-beta (through the induction of hypertension or disruption of the BBB), as well as the use of high-dose corticosteroids, are among the triggering factors for PRES in these patients. Conclusion: Although PRES is a rare complication in MS and NMOSD patients, the nature of these diseases, which is associated with disruption of the blood-brain barrier, along with the use of specific treatments, places these patients at risk of developing PRES. © 2025, Isfahan University of Medical Sciences(IUMS). All rights reserved.