New diagnostic criteria for MOGAD show high performance in children and adults

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Author: Joaquin A Pena MD

Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) has emerged as a distinct entity, separate from multiple sclerosis and aquaporin-4 antibody (AQP4-Ab) positive neuromyelitis optica spectrum disorder (NMOSD). The 2023 international diagnostic criteria for MOGAD, which include specific clinical and radiological features and MOG-Ab testing, have been validated in a recent study published in Neurology. These criteria play a crucial role in the accurate diagnosis of MOGAD.

Studies have indicated that the criteria effectively identify most children and adults with MOGAD, achieving the highest accuracy during disease attacks (96%) compared to remissions (89%). Researchers from the Queen Square MS Centre and Great Ormond Street Hospital in London conducted a study where they retrospectively analyzed 539 patients (135 children and 404 adults) with suspected inflammatory demyelination who underwent MOG-Ab testing between 2018 and 2022. The study aimed to evaluate the performance of the newly proposed MOGAD criteria against the clinical diagnoses made by treating neurologists. The large cohort and retrospective data offer a strong foundation for the study's conclusions.

The results were striking. The MOGAD criteria showed high sensitivity (96.5%), specificity (98.9%), and accuracy (98.5%) across all patients. Notably, performance was even better in children, with 100% sensitivity and 99.2% accuracy. In adults, although sensitivity was slightly lower (91.9%), specificity remained high at 98.9%. Interestingly, the criteria outperformed MOG-Ab testing alone, particularly in adults. The addition of supporting clinical and MRI features, such as specific symptoms and characteristic brain and spinal cord lesions, improved specificity from 95.6% to 98.9% (p=0.0005) in adults, highlighting the importance of these criteria beyond antibody status.

The study also provided valuable insights into the clinical and radiological features of MOGAD. Children exhibited more supporting features than adults, especially in myelitis and optic neuritis presentations. Longitudinal optic nerve involvement was the most common feature in children with optic neuritis (81.3%), and bilateral simultaneous involvement was more frequent in adults (50%).

These findings have significant implications for clinical practice. The high performance of the criteria, especially in children, supports their use in diagnosing MOGAD. The improved specificity in adults emphasizes the importance of considering antibody status and supporting features, providing reassurance and confidence in the diagnostic process. Adopting these criteria can significantly improve patient outcomes by enabling earlier and more accurate diagnosis and appropriate treatment.

However, the study has limitations. Its retrospective nature and the inclusion of patients from specialized neuroimmunology clinics may introduce selection bias. Additionally, relying on MOG-Ab positivity as a core criterion raises questions about seronegative cases with typical MOGAD features. These limitations should be considered when interpreting the study's results and applying the requirements in clinical practice. It's important to note that these limitations do not negate the significance of the study's findings but rather underscore the need for further research.

In conclusion, this study provides compelling evidence in support of the 2023 MOGAD diagnostic criteria. It emphasizes their effectiveness in differentiating MOGAD from other demyelinating disorders, especially in children. As we progress, prospective studies and further refinement of these criteria will enhance our understanding and management of MOGAD. This gives us hope for the future of MOGAD research and its potential to improve patient outcomes significantly.

References:

Varley JA, Champsas D, Prossor T, et al. Validation of the 2023 international diagnostic criteria for MOGAD in a selected cohort of adults and children. Neurology 2024;103(1):e209321. PMID 38870448

Banwel B, Bennett J, Marignier R, et al. Diagnosis of myelin oligodendrocyte glycoprotein antibody-associated disease: International MOGAD Panel proposed criteria. Lancet Neurol 2023; 22:268-82.

Fonseca E, Olivé-Cirera G, Martinez-Hernandez E, et al. Investigating the 2023 MOGAD criteria in children and adults with MOG-antibody positivity within and outside attacks. Neurology 2024;103(6):e209682. PMID 39190859

Rechtman A, Freidman-Korn T, Zveik O, Shweiki L, Hoichman G, Vaknin-Dembinsky A. Assessing the applicability of the 2023 international MOGAD panel criteria in real-world clinical settings. J Neurol 2024;271(8):5102-8. PMID 38809270

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