To determine the clinical applicability of 2015 neuromyelitis optica spectrum disorder (NMOSD) criteria for Myelin Oligodendrocyte Glycoprotein-IgG (MOG-IgG+) Patients.

The 2015 international diagnostic criteria for NMOSD were developed to define patients with a similar clinical phenotype and included aquaporin-4-IgG seropositive and seronegative subsets. Further, recent drug trials have suggested these groups have differing responses to immunotherapy.

Myelin oligodendrocyte glycoprotein antibody (MOG-IgG), a biomarker associated with central demyelinating diseases, sometimes referred to as “seronegative NMOSD”. However, our understanding of MOG-IgG associated diseases has grown and we now recognize distinct clinical and radiological features from aquaporin-4-IgG NMOSD.

In this study we evaluate how often MOG-IgG+ patients meet the 2015 NMOSD AQP4-IgG seronegative diagnostic criteria.

Retrospective analysis of the clinical and radiologic features of Mayo Clinic MOG-IgG patients was undertaken. MOG-IgG1 was detected by a clinically validated live flow cytometric assay.

We identified 186 MOG-IgG+ patients (adults 128 [50% female], children 58 [64% female]).

Twenty six adult patients (20%) met 2015 NMOSD criteria including ≥2 attacks of: optic neuritis and LETM (19), optic neuritis, cerebral and brainstem syndrome (3), LETM, cerebral and brainstem syndrome (1), cerebral syndrome and optic neuritis (1), acute brainstem syndrome and LETM (1), area postrema (AP) syndrome with AP lesion (1).

Among children, 13 (22%) met the 2015 NMOSD criteria including ≥2 attacks of: optic neuritis and LETM (10), acute brainstem syndromes and LETM (2), optic neuritis and cerebral syndrome (1).

No MOG-IgG+ adults or children had narcolepsy.   

Red flags suggesting MOG-IgG+ included; headache, ADEM, post-infection/vaccination, LETM with predominant grey matter and conus involvement.

The majority of MOG-IgG+ patients did not fulfill 2015 NMOSD seronegative criteria highlighting a distinct clinical-radiological phenotype. Future central demyelinating disease criteria should use biomarkers to distinguish between these clinically, radiologically and immunologically distinct conditions.