Application of NMOSD IPND-2015 Criteria in AQP4-IgG Positive, MOG-IgG Positive and Seronegative Inflammatory Demyelinating Diseases
Samira Apostolos-Pereira1, João Vitor Mahler Ferreira Oliveira2, Guilherme Diogo Silva1, Vinicius Schoeps3, Ana Beatriz Ayroza Galvao Ribeiro Gomes4, Natalia Mendes3, Aline Matos5, Marina Solti2, Pedro Henrique Torretta1, Milena Pitombeira6, Mateus Boaventura de Olivei3, Renata Paolilo1, Carolina Rimkus1, Tarso Adoni1, Anne-Katrin Proebstel4, Douglas Sato7, Dagoberto Callegaro1
1Hospital das Clinicas, Faculdade de Medicina da Universidade de São Paulo, 2Faculdade de Medicina, Universidade de Sao Paulo, 3Hospital Das Clinicas Da Faculdade de Medicina Da Universidade de Sao Paulo, 4University Hospital Basel, 5Instituto de Medicina Tropical de São Paulo - FMUSP, 6Hospital Geral de Fortaleza, 7Pontifical Catholic University of Rio Grande do Sul
Objective:
To evaluate the frequency of cases fulfilling IPND-2015 in a group of AQP4-IgG, MOG-IgG and seronegative patients with inflammatory demyelinating central nervous system disorders
Background:
The International Panel for Neuromyelitis Optica Diagnosis (IPND-2015) criteria improved early recognition of neuromyelitis optica spectrum disorder (NMOSD). The publication of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) criteria in 2023 underscores the need for validation of these criteria in different populations not only to verify diagnostic yield but also to identify overlaps and areas for improvement.
Design/Methods:
We performed a cross-sectional study conducted at the Neuroimmunology Clinic of the University of São Paulo. We randomly selected from our records 40 AQP4+, 40 MOG+, and 40 seronegative patients with demyelinating events suspected for NMOSD. All seronegative patients had been tested for both MOG-IgG and AQP4-IgG serum antibodies using cell-based assays. Patients with multiple sclerosis were excluded.
Results:
All 40 AQP4-IgG+ cases fulfilled IPND-2015, in contrast to 12.5% of MOG-IgG+ and 42.5% of seronegative patients. Demographics were similar across the three groups, except for female proportion (87.5%, 65%, 62.5%) ; AQP4-IgG+, MOG-IgG+ and seronegative patients respectively. Bilateral optic neuritis (35%, 57.5%, 30%), all-time myelitis (85%, 50%, 72.5%), longitudinally extensive transverse myelitis (LETM) (70%, 17.5%, 47.5%), and area postrema syndromes (17.5%, 0%, 20%) had significantly different frequencies between AQP4-IgG, MOG-IgG+ and seronegative patients. The frequency of relapsing course (87.5%, 70%, 55%) was higher in AQP4-IgG+ and MOG-IgG+ when compared to seronegative patients.
Conclusions:
IPND-2015 criteria were fulfilled in some MOG-IgG+ and almost half of seronegative patients. This finding highlights the need for IPND-2015 criteria refinement regarding the classification of seronegative patients and overlap of both MOGAD-2023 and IPND-2015. Of note, MOGAD cases are now a distinct entity, so use IPND-2015 for these cases may be misleading. Future reviews of IPND-2015 NMOSD diagnostic criteria will require special attention to include seronegative cases.