Non-infectious meningitis and CNS demyelinating diseases: A conceptual review

Etemadifar M., Fereidan-Esfahani M., Sedaghat N., Kargaran P., Mansouri A., Abhari A., ...More

Revue Neurologique, vol.179, no.6, pp.533-547, 2023 (SCI-Expanded) identifier identifier

  • Publication Type: Article / Review
  • Volume: 179 Issue: 6
  • Publication Date: 2023
  • Doi Number: 10.1016/j.neurol.2022.10.006
  • Journal Name: Revue Neurologique
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus, PASCAL, BIOSIS, CAB Abstracts, Psycinfo
  • Page Numbers: pp.533-547
  • Keywords: Anti-N-Methyl-D-Aspartate Receptor Encephalitis, Aseptic Meningitis, CNS Demyelinating Autoimmune Diseases, Meningoencephalitis, Neuromyelitis Optica
  • Istanbul Medipol University Affiliated: No


Many cases of aseptic meningitis or meningoencephalitis, unresponsive to antimicrobial treatments, have been reported recently in patients with established/new-onset central nervous system (CNS) inflammatory demyelinating diseases (CNSIDDs). Given the higher probability of infectious etiologies, CNSIDDs are rarely considered among the differentials in meningitis or meningoencephalitis cases. We gathered and tabulated cases of non-infectious, steroid-responsive meningitis or meningoencephalitis associated with neuromyelitis optica spectrum disorder (NMOSD) and myelin oligodendrocyte glycoprotein-associated disease (MOGAD). This conceptual review highlights the need to bolster routine infectious workups with immunological workups in cases of meningoencephalitis or meningitis where potential autoimmune etiologies can be suspected. Although differentiating CNSIDDs with meningeal involvement from infectious meningitis may not substantially affect acute treatment strategies, long-term management and follow-up of the two are entirely different. We also discuss future research directions and hypotheses on how CNSIDDs may be associated with meningitis-like presentations, e.g. overlapping glial fibrillary acidic protein astrocytopathy or autoimmune encephalitis, alterations in regulatory T-helper cells function, and undetected viral agents.