MOGAD-Like Encephalitic Syndromes Without MOG Antibodies in Children.

Authors

Gemma Olivé-Cirera, Elianet Gisell Fonseca, Mar Guasp, Ludovica Falcioni, Anna Fetta, Raquel Ruiz García, Elisabet Poyatos, Ana Beatriz Serafim, Maria Gual, Gianni Ippoliti, Armando Reques, Laura Naranjo Rondán, Marianna Spatola, Yolanda Blanco, Eugenia Martinez-Hernandez, Francesc Graus, Maria Sepulveda, Romana Höftberger, Lidia Sabater, Albert Saiz, Josep Dalmau, Thais Armangue, Spanish Pediatric Autoimmune Encephalitis study group

Published in

Neurology(R) neuroimmunology & neuroinflammation. Volume 13. Issue 5. Pages e200635. Epub Aug 24, 2026.

Abstract

Although acute disseminated encephalomyelity (ADEM) can be myelin oligodendrocyte glycoprotein-IgG (MOG-IgG) positive or negative, it is unclear whether other MOG-antibody-associated disease (MOGAD)-like encephalitis syndromes occur without MOG-IgG. We aimed to define the frequency, clinico-radiologic features, outcomes, and antibody associations of such cases in children.
Prospective cohort study of children (<18 years) with encephalitis meeting clinico-radiologic MOGAD criteria, regardless of MOG-IgG status. Serum from all patients and CSF when available were tested by live cell-based assays (CBA-IIF, CBA-FACS) at 2 laboratories. Brain MRIs were centrally reviewed for MOGAD-like patterns (ADEM, cortical encephalitis, isolated/predominant central gray matter). Additional testing included CBAs for MOG-IgA, MOG-IgM, PLP1-IgG, glial fibrillary acidic protein-IgG, and AQP4-IgG, along with rat brain immunohistochemistry.
Among 160 patients with MOGAD-like encephalitis, 120 were MOG-IgG positive and 40 negative (20 ADEM, 11 cortical encephalitis, 9 isolated/predominant central gray matter encephalitis). Clinical-radiologic features were broadly comparable between groups, including lesion distribution, frequency of associated longitudinally extensive transverse myelitis, lesion resolution, and functional outcomes. Relapses were more frequent in MOG-IgG-positive patients (22, 18% vs 1, 3%; p = 0.017). Immunohistochemistry revealed myelin immunostaining in 14 (12%) MOG-IgG-positive cases (CBA confirmed cross-reactivity with rodent MOG-epitopes) and 10 (25%) MOG-IgG-negative patients (no cross-reactivity with rodent MOG-epitopes); this included a fulminant case with autopsy showing perivenous demyelination and C4d complement deposition, suggesting autoantibodies against an unknown myelin antigen. MOG-IgA, MOG-IgM, or PLP1-IgG occurred in 36/111 (32%) MOG-IgG-positive and 7/37 (19%) MOG-IgG-negative cases, without clinical differences between groups.
Children can develop the full spectrum of MOGAD-like encephalitis without MOG-IgG. Clinical features and immunotherapy response are similar to MOGAD, but relapses are uncommon.

PMID:
42636412
Bibliographic data and abstract were imported from PubMed on 25 Aug 2026.

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