Rare immune-mediated diseases of the CNS are acquired disorders that are presumed to be triggered by environmental risk factors in individuals who carry a genetic risk variant burden. Geographical and regional variation may exist in prevalence and incidence of these disorders, e.g. NMOSD incidence and prevalence is higher in Asia than in Europe. Rare immune-mediated diseases of the central nervous system often pose diagnostic, prognostic and therapeutic challenges. Incidence and prevalence of these disorders may vary on a global and regional level, affecting the local resource allocation and care pathways. Accurate diagnosis may be a complex and long process, although recent advances in diagnosis with antibody testing and neuro-imaging techniques have refined the diagnostic process and accuracy. Despite recognition of risk-factors (age, ethnicity, gender, pregnancy) in some rare CNS immune-mediated diseases, individual prognosis remains challenging. Also, the true disease burden from rare immune-mediated diseases is ill-defined. Finally, treatment decisions are only rarely supported by results of randomized clinical trials and are based on cohort-studies, registries, cases-series and expert-opinion. Research from single-center or multi-center cohorts may provide new data that can aid in making an early and accurate diagnosis and prognosis. Analysis of risk factors may aid the treatment decisions. Treatment patterns and assessment of long-term outcomes may help to determine appropriate timing, sequencing and stopping of immunosuppressive treatments. The diagnostic and therapeutic approach to these diseases in resource-limited areas and potential ways to improve the care pathway, are in scope of this research topic.

In this Research Topic we welcome articles on rare immune-mediated disease of the CNS, including Susac syndrome, CNS vasculitis, neurosarcoidosis, neuro-Behçcet, autoimmune encephalitis, tumefactive demyelination, MOGAD (MOG antibody associated disease) and NMOSD (neuromyelitis optica spectrum disorders).

Areas of interest include, but are not limited to the following:

• Natural history studies

• Prevalence and incidence studies

• Quality of life

• Studies of risk-factors (environmental, genetic)

• Studies on diagnostics (neuroimaging, immunoassays)

• Studies on prognosis and prognostic factors

• Studies on treatment

We welcome translational and clinical articles including: Original Research, Systematic Review, Reviews, Case Reports and Perspectives.

The institution of BW has received honoraria for acting as a member of Scientific Advisory Boards for Almirall, Biogen, Celgene/BMS, Merck Serono, Novartis, Roche, Sanofi-Genzyme and speaker honoraria and travel support from Biogen, Merck Serono, Novartis, Roche, Sanofi-Genzyme, TEVA; research and patient support grants from Roche, Biogen, Merck-Serono, Novartis, Sanofi-Genzyme. BW is supported by Horlait Dapsens Medical Foundation 2020 and FWO-TBM grant T001121N. I.K. received travel expenses for attending meetings from Pfizer and CSL Behring. I.K. received speaker honoraria from Daiichi Sankyo and Pfizer.

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