Validation of the 2023 International Diagnostic Criteria for MOGAD in a Selected Cohort of Adults and Children

Abstract

Background and ObjectivesTo test the performance of the 2023 myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) criteria in adults and children with inflammatory demyelinating conditions who were tested for MOG antibodies (Abs). MethodsThis was a retrospective study of patients tested for MOG-Abs from 2018 to 2022 in 2 specialist hospitals. The inclusion criteria comprised ≥1 attendance in an adult or pediatric demyelinating disease clinic and complete clinical and MRI records. The final clinical diagnosis of MOGAD, made by the treating neurologist, was taken as the benchmark against which the new criteria were tested. The international MOGAD diagnostic criteria were applied retrospectively; they stipulate at least 1 clinical or MRI supporting feature for MOGAD diagnosis in positive fixed MOG cellbased assay without a titer. The performance MOG-Ab testing alone for MOGAD diagnosis was also assessed and compared with that of MOGAD criteria using the McNemar test. ResultsOf the 1,879 patients tested for MOG-Abs, 539 (135 pediatric and 404 adults) met the inclusion criteria. A clinical diagnosis of MOGAD was made in 86/539 (16%) patients (37 adults, 49 children), with a median follow-up of 3.6 years. The MOGAD diagnostic criteria had sensitivity of 96.5% (adults 91.9%, children 100%), specificity of 98.9% (adults 98.8%, children 98.9%), positive predictive value of 94.3% (adults 89.4%, children 98%), negative predictive value of 99.3% (adults 99.2%, children 100%), and accuracy of 98.5% (adults 98.3%, children 99.2%). When compared with MOG-Ab testing alone, a difference was seen only in adults: a significantly higher specificity (98.9% vs 95.6%, p = 0.0005) and nonstatistically significant lower sensitivity (91.9% vs 100%, p = 0.08). DiscussionThe international MOGAD diagnostic criteria exhibit high performance in selected patients with inflammatory demyelinating diseases (who had a high pretest probability of having

Unveiling the Performance of Proposed MOGAD Diagnostic Criteria

“…Despite the aforementioned limitations inherent to its retrospective nature, this study 3 strongly supports the adoption of the proposed MOGAD diagnostic criteria. 2 Future studies should aim to address generalizability in a more diverse clinical setting (i.e., outside expert centers) with prospective standardized data collection and possibly more sensitive MOG-Abs assays.…”

“…7 Finally, in the context of Varley's study retrospective design, most of the individuals tested for MOG-Abs did not have enough clinical data available to further assess the diagnostic criteria, possibly affecting accuracy estimates. 3 Despite the aforementioned limitations inherent to its retrospective nature, this study 3 strongly supports the adoption of the proposed MOGAD diagnostic criteria. 2 Future studies should aim to address generalizability in a more diverse clinical setting (i.e., outside expert centers) with prospective standardized data collection and possibly more sensitive MOG-Abs assays.…”

“…In this issue of Neurology ®, Varley et al 3 publish a study in which they evaluated the performance of the proposed diagnostic criteria in a large retrospective cohort of adults and children who underwent MOG-Ab testing using fixed cell-based assays at a centralized laboratory and attended one of 3 neuroimmunology clinics in the United Kingdom. 3 The authors found that the criteria had high specificity and sensitivity when compared with their clinical diagnosis at the last follow-up as the reference standard. As expected, MOG-Ab testing alone was slightly less specific (96.3% vs 98.6%, p = 0.0005) and more sensitive (100% vs 96.5%, p = 0.08) than the proposed diagnostic criteria.…”

“…7 Finally, in the context of Varley's study retrospective design, most of the individuals tested for MOG-Abs did not have enough clinical data available to further assess the diagnostic criteria, possibly affecting accuracy estimates. 3…”