Neuromyelitis Optica Spectrum Disorder and Other Non–Multiple Sclerosis Central Nervous System Inflammatory Diseases

PURPOSE OF REVIEW This article reviews the clinical features, diagnostic approach, treatment, and prognosis of central nervous system inflammatory diseases that mimic multiple sclerosis (MS), including those defined by recently discovered autoantibody biomarkers.

RECENT FINDINGS The discovery of autoantibody biomarkers of inflammatory demyelinating diseases of the central nervous system (aquaporin-4 IgG and myelin oligodendrocyte glycoprotein IgG) and the recognition that, despite some overlap, their clinical phenotypes are distinct from MS have revolutionized this field of neurology. These autoantibody biomarkers assist in diagnosis and have improved our understanding of the underlying disease pathogenesis. This has allowed targeted treatments to be translated into clinical trials, three of which are now under way in aquaporin-4 IgG–seropositive neuromyelitis optica (NMO) spectrum disorder.

SUMMARY Knowledge of the clinical attributes, MRI findings, CSF parameters, and accompanying autoantibody biomarkers can help neurologists distinguish MS from its inflammatory mimics. These antibody biomarkers provide critical diagnostic and prognostic information and guide treatment decisions. Better recognition of the clinical, radiologic, and laboratory features of other inflammatory MS mimics that lack autoantibody biomarkers has allowed us to diagnose these disorders faster and initiate disease-specific treatments more expeditiously.

Address correspondence to Dr Eoin P. Flanagan, Mayo Clinic, Department of Neurology, 200 First St SW, Rochester, MN 55905, [email protected].

RELATIONSHIP DISCLOSURE: Dr Flanagan receives research/grant support from MedImmune/Viela Bio.

UNLABELED USE OF PRODUCTS/INVESTIGATIONAL USE DISCLOSURE: Dr Flanagan discusses the unlabeled/investigational use of azathioprine, cetirizine, corticosteroids, eculizumab, inebilizumab, IV immunoglobulin, methotrexate, mycophenolate mofetil, plasma exchange, rituximab, SA237, sivelestat, and tocilizumab for the treatment of neuromyelitis optica spectrum disorder and other non–multiple sclerosis central nervous system inflammatory diseases.