MOGAD-Like Encephalitic Syndromes Without MOG Antibodies in Children

BACKGROUND AND OBJECTIVES: 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. METHODS: 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. RESULTS: = 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. DISCUSSION: 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.

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Journal
Neurology Neuroimmunology & Neuroinflammation
Published
2026-08-24
DOI
https://doi.org/10.1212/nxi.0000000000200635
Primary Topic
Autoimmune Neurological Disorders and Treatments
Type
article
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article

MOGAD-Like Encephalitic Syndromes Without MOG Antibodies in Children

Eugenia Martínez‐Hernández, Elianet Fonseca, Francesc Graus, Lídia Sabater et al.
Neurology Neuroimmunology & Neuroinflammation
Autoimmune Neurological Disorders and Treatments
article

MOGAD-Like Encephalitic Syndromes Without MOG Antibodies in Children

Eugenia Martínez‐Hernández, Elianet Fonseca, Francesc Graus, Lídia Sabater, Gemma Olivé-Cirera, Ludovica Falcioni, María Sepúlveda, Armando Reques, Raquel Ruiz‐García, Elísabet Poyatos-Canton, Mar Guasp, Josep Dalmau, Ana Beatriz Serafim, Marianna Spatola, Albert Saiz, Romana Höftberger, Thaís Armangué, Laura Naranjo, Yolanda Blanco, Anna Fetta, Gianni Ippoliti, Maria Gual, for the Spanish Pediatric Autoimmune Encephalitis study group
article en

Abstract

BACKGROUND AND OBJECTIVES: 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. METHODS: 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. RESULTS: = 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. DISCUSSION: 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.

Neurology Neuroimmunology & NeuroinflammationVol. 13(5)
Hospital Sant Joan de Déu Barcelona (ES), University of Sassari (IT), Hospital de Sant Pau (ES), Centro de Investigación Biomédica en Red de Enfermedades Respiratorias (ES), Centre for Biomedical Network Research on Rare Diseases (ES), Centro de Investigación Biomédica en Red (ES), Hospital Clínic de Barcelona (ES), Hospital de Sabadell (ES), Institute of Research and Innovation Parc Tauli (ES), ERN GUARD-Heart (NL), Consorci Institut D'Investigacions Biomediques August Pi I Sunyer (ES), Azienda Ospedaliera G. Brotzu (IT), Medical University of Vienna (AT), University of Pennsylvania (US), University of Bologna (IT)
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