Clinical Features of Children with MOG-IgG Who Fulfill Criteria of Multiple Sclerosis and Overlapping Disorders
Elianet Fonseca1, Gemma Olivé-Cirera2, Li-Wen Chen3, Fernando Paredes-Carmona4, Noemí Nuñez Enamorado5, Sabas Boyero Duran6, Maria Del Mar Mendibe Bilbao6, Nuria Visa-Reñé4, Maria Vazquez-Lopez7, Ana Felipe-Rucián8, Gemma Romeu9, Eugenia Martinez-Hernandez10, Yolanda Blanco Morgado11, Maria Sepulveda11, Albert Saiz10, Josep Dalmau12, Thais Armangue13
1Neuroimmunology Program, Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS) – Caixa Research Institute, Hospital Clínic de Barcelona, Barcelona, Spain. Pediatric Neuroimmunology Unit, Neurology Department, Sant Joan de Déu Children’s Hospital, ERN-RITA reference center, University of Barcelona, Barcelona, Spain, 2Neuroimmunology Program, Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS) – Caixa Research Institute, Hospital Clínic de Barcelona, Barcelona, Spain. Pediatric Neuroimmunology Unit, Neurology Department, Sant Joan de Déu Children’s Hospital, ERN-RITA reference center, University of Barcelona, Barcelona, Spain. Pediatric Neurology Department, Hospital Parc Taulí de Sabadell, Spain, 3Department of Pediatrics, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan, Taiwan, 4Pediatric Neurology Section, Hospital Arnau de Vilanova, Lleida, Spain, 5Pediatric Neurology Department, Hospital 12 de Octubre, Madrid, Spain, 6Neurology Department, Hospital Universitario Cruces, Barakaldo, Bizkaia, Spain. Biobizkaia Health Research Institute., 7Pediatric Neurology Department, Hospital Gregorio Marañon, Madrid, Spain, 8Pediatric Neurology Section, Hospital Universitario Vall d'Hebron, Barcelona, Spain, 9Ophtalmology Unit, Sant Joan de Déu Children’s Hospital, University of Barcelona, Barcelona, Spain, 10Neuroimmunology Program, Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS) – CaixaResearch Institute, Hospital Clínic de Barcelona, Barcelona, Spain, 11Neuroimmunology Program, Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS) – Caixa Research Institute, Hospital Clínic de Barcelona, Barcelona, Spain, 12Neuroimmunology Program, Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS) – Caixa Research Institute, Hospital Clínic de Barcelona, Barcelona, Spain. Department of Neurology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA, 13Neuroimmunology Program, Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS) – Caixa Research Institute, Hospital Clínic de Barcelona, Barcelona, Spain. Pediatric Neuroimmunology Unit, Neurology Department, Sant Joan de Déu Children’s Hospital, ERN-RITA reference center, University of Barcelona, Barcelona, Spain.
Objective:
To assess the clinical features, disease-modifying treatment response, and outcomes of children with MOG-IgG who fulfilled the 2017 McDonald criteria for MS
Background:
The 2023 MOGAD criteria have shown high accuracy in pediatric and adult patient populations, but for those who simultaneously meet criteria for MOGAD and multiple sclerosis (MS) the differential diagnosis remains challenging. The clinical significance and outcomes of pediatric patients in this clinical scenario are unclear.
Design/Methods:
This prospective observational nationwide study included children (<18 years old) with a suspected acute demyelinating syndrome (ADS) whose serum or CSF was positive for MOG-IgG using a live cell-based assay, met the McDonald 2017 criteria, and had ≥ 1 year of clinical follow-up. We assessed clinical and radiological features at onset, during relapses, and treatment response.
Results:
Among a cohort of 554 children with confirmed ADS (196 with MOG-IgG), 8 patients (median age 11 years, IQR 9-14) harbored MOG-IgG antibodies and met criteria for MS: 2 had clinical-radiological features of MS and are no longer discussed here, and 6 of overlapping MOGAD-MS. Of these six, five presented with typical MOGAD manifestations (3 had bilateral optic neuritis, 1 cortical encephalitis, 1 brainstem-cerebellar syndrome), and four of them had specific CSF oligoclonal bands (CSF-OCB). The remaining patient had short myelitis; well-delimited brain MRI lesions, MOG-IgG restricted to CSF, negative CSF-OCB, and developed an early relapse of bilateral optic neuritis. All 6 patients had persistent silent radiological activity with lesional location and morphology suggestive of MS-like features, leading to initiation of disease modifying treatments (DMT). Although MS treatments were safe and effective, 5/6 (83%) patients eventually required high efficacy DMT.
Conclusions:
About 4% of patients with MOG-IgG met MS criteria. The clinical-radiological spectrum ranged from typical MS to overlapping features between both entities. Patients usually required high efficacy DMT.
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