Associations of paediatric demyelinating and encephalitic syndromes with myelin oligodendrocyte glycoprotein antibodies: a multicentre observational study

Autor: Hilario Gómez-Martín, Rocío Calvo, Cesar Ruiz, Cristina Auger, Maria D M Mendibe, Berta Pujol-Soler, Josep Dalmau, Alberto Alcantud, Albert Saiz, Gabriela Orellana, Xavier Montalban, Teresa Bermejo, Ana Camacho Salas, Lourdes Aquino Fariña, Desire Gonzalez-Barrios, Lorena Monge-Galindo, Raquel Ruiz-García, Ana Felipe-Rucián, Miguel Tomás-Vila, Maria Vázquez-López, Gema Arriola-Pereda, Diana Alvarez Demanuel, Natalia Juliá-Palacios, Elena Miravet, Ainhoa García-Ribes, Marta Muñoz-Batista, Gemma Aznar-Laín, Elena Maqueda-Castellote, Maria Jesús Martínez-González, Ignacio Málaga, Mar Tintoré, Eva Caballero, Eulàlia Turon-Viñas, Maite Benavides-Medina, Eduardo López-Laso, Verónica Delgadillo-Chilavert, Sara Jimena-Garcia, María Jiménez-Legido, Adeline Vanderver, Maria Concepción Miranda-Herrero, Cristina Villar-Vera, Maria Sepúlveda, Amagoia Elosegi-Castellanos, Verónica Cantarín-Extremera, Montserrat Garcia-Puig, Helena Ariño, Vanesa Esteban Canto, Maria D Mora-Ramírez, Maria I Rodriguez-Lucenilla, Sergio Aguilera-Albesa, Luis Querol, Tania Nunes-Cabrera, Joaquín A. Fernández-Ramos, Beatriz Muñoz-Cabello, Verónica González-Álvarez, Francesc Graus, Alfredo Ramírez, Simone Mattozi, Marta Martínez González, Eugenia Martinez-Hernandez, Laura Toledo Bravo de Laguna, Lucía Martín-Viota, Mireia Alvarez Molinero, Georgina Arrambide, Víctor Soto-Insuga, Gemma Olivé-Cirera, Raquel Blanco-Lago, Itxaso Martí-Carrera, David Conejo-Moreno, Luisa Arrabal, Thaís Armangue, Luis González-Gutiérrez-Solana, Juan Navarro-Morón
Rok vydání: 2020
Předmět:
Male
0301 basic medicine
REVISIONS
CHILDREN
Pediatrics
DISEASE
Cohort Studies
0302 clinical medicine
immune system diseases
Medicine
CRITERIA
Prospective Studies
Child
Prospective cohort study
DISSEMINATED ENCEPHALOMYELITIS
First episode
NEUROMYELITIS-OPTICA
biology
Neuromyelitis Optica
Immunoglobulins
Intravenous
hemic and immune systems
Syndrome
MULTIPLE-SCLEROSIS
Magnetic Resonance Imaging
Child
Preschool
Acute disseminated encephalomyelitis
Encephalitis
Female
Rituximab
medicine.drug
medicine.medical_specialty
DIAGNOSIS
Myelin oligodendrocyte glycoprotein
03 medical and health sciences
Internal medicine
Humans
MOG
Optic neuritis
Autoantibodies
Autoimmune encephalitis
RECEPTOR
business.industry
medicine.disease
nervous system diseases
030104 developmental biology
nervous system
Spain
Immunoglobulin G
biology.protein
Myelin-Oligodendrocyte Glycoprotein
Neurology (clinical)
business
030217 neurology & neurosurgery
Demyelinating Diseases
Zdroj: LANCET NEUROLOGY
r-IIS La Fe. Repositorio Institucional de Producción Científica del Instituto de Investigación Sanitaria La Fe
instname
r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
r-FSJD: Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
Fundació Sant Joan de Déu
ISSN: 1474-4422
Popis: BACKGROUND: Investigations of myelin oligodendrocyte glycoprotein (MOG) antibodies are usually focused on demyelinating syndromes, but the entire spectrum of MOG antibody-associated syndromes in children is unknown. In this study, we aimed to determine the frequency and distribution of paediatric demyelinating and encephalitic syndromes with MOG antibodies, their response to treatment, and the phenotypes associated with poor prognosis. METHODS: In this prospective observational study, children with demyelinating syndromes and with encephalitis other than acute disseminated encephalomyelitis (ADEM) recruited from 40 secondary and tertiary centres in Spain were investigated for MOG antibodies. All MOG antibody-positive cases were included in our study, which assessed syndromes, treatment and response to treatment (ie, number of relapses), outcomes (measured with the modified Rankin scale [mRS]), and phenotypes associated with poor prognosis. We used Fisher's exact and Wilcoxon rank sum tests to analyse clinical features, and survival Cox regression to analyse time to antibody negativity. FINDINGS: Between June 1, 2013, and Dec 31, 2018, 239 children with demyelinating syndromes (cohort A) and 296 with encephalitis other than ADEM (cohort B) were recruited. 116 patients had MOG antibodies, including 94 (39%) from cohort A and 22 (7%) from cohort B; 57 (49%) were female, with a median age of 6·2 years (IQR 3·7-10·0). Presenting syndromes in these 116 patients included ADEM (46 [68%]), encephalitis other than ADEM (22 [19%]), optic neuritis (20 [17%]), myelitis (13 [11%]), neuromyelitis optica spectrum disorders (six [5%]), and other disorders (nine [8%]). Among the patients with autoimmune encephalitis in cohort B (n=64), MOG antibodies were more common than all neuronal antibodies combined (22 [34%] vs 21 [33%]). After a median follow-up of 42 months (IQR 22-67), 33 (28%) of the 116 patients had relapses, including 17 (17%) of 100 diagnosed at first episode. Steroids, intravenous immunoglobulin, or plasma exchange were used in 100 (86%) patients at diagnosis, and 32 (97%) of 33 at relapses. Rituximab was mainly used at relapses (11 [33%]). 99 (85%) of 116 patients had substantial recovery (mRS 2; one died). Phenotypes of poor prognosis included ADEM-like relapses progressing to leukodystrophy-like features, and extensive cortical encephalitis evolving to atrophy. Time to antibody negativity was longer in patients with relapses (HR 0·18, 95% CI 0·05-0·59). INTERPRETATION: The spectrum of paediatric MOG antibody-associated syndromes is wider than previously reported and includes demyelinating syndromes and encephalitis. Recognition of these disorders has important clinical and prognostic implications. FUNDING: Mutua Madrileña Foundation; ISCIII-Subdirección General de Evaluación y Fomento de la Investigación Sanitaria; Fondo Europeo de Desarrollo Regional; Pediatrics Spanish Society; Departament de Salut, Generalitat de Catalunya; Marato TV3 Foundation; Red Española de Esclerosis Múltiple; La Caixa Foundation; and Fundació CELLEX.
Databáze: OpenAIRE
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