Portal de investigação
Pediatric neuromyelitis optica spectrum disorders in Portugal: A multicentre retrospective study
Data de publicação:
Data Ahead of Print:
Autores da FMUP
Participantes de fora da FMUP
- Martins, C
- Moura, J
- Figueiroa, S
- Garrido, C
- Martins, J
- Samoes, R
- Sousa, R
- Palavra, F
- Ferreira, J
- da Silva, AM
- Sa, MJ
- Santos, E
Unidades de investigação
Abstract
Introduction: Neuromyelitis optica spectrum disorders (NMOSD) are more prevalent in adulthood, with few cases reported in pediatric age (< 18 years). In this group, anti-aquaporin 4 (AQP4) antibodies are less frequent, while antibodies against myelin oligodendrocyte glycoprotein (MOG) are more commonly detectable than in adults.& nbsp;Objective and methods: Description of pediatric NMOSD cases identified in a national multicentric NMOSD Portuguese registry.& nbsp;Results: Twenty (11.1%) NMOSD cases were diagnosed in pediatric age. Twelve (60%) were female, with a median age of onset of 12.5 (6.8-16.5) years. The presenting feature was transverse myelitis in 10 (50%), 4 of which with simultaneous optic neuritis and 2 with concomitant brainstem syndrome. Nine patients (45%) had pleocytosis in the CSF. Six (30.0%) exhibited anti-AQP4 antibodies, 13 (65.0%) anti-MOG antibodies, and one was seronegative for both. Four anti-AQP4 antibodies-positive patients had >= 1 relapse. Most anti-MOG-positive cases were monophasic (53.8%). In the acute phase, all patients received IV methylprednisolone, nine received IVIg and four plasma exchange. One anti-AQP4-positive patient died. Ten patients (5 anti-AQP4-positive/5 antiMOG-positive) were on maintenance immunosuppressive therapy at the time of data collection.& nbsp;Conclusion: NMOSD may present in pediatric age. It is essential to establish the diagnosis and promptly start therapy to improve the prognosis.
Dados da publicação
- ISSN/ISSNe:
- 2211-0356, 2211-0348
- Tipo:
- Article
- Páginas:
- 180-
- Link para outro recurso:
- www.scopus.com
Multiple Sclerosis and Related Disorders Elsevier
Citações Recebidas na Web of Science: 2
Citações Recebidas na Scopus: 5
Documentos
- Não há documentos
Filiações
Keywords
- Neuromyelitis optica; Pediatric; Neuroimmunology; Anti-aquaporin 4 autoantibodies; Anti-myelin oligodendrocyte related protein; autoantibodies; Central nervous system
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Citar a publicação
Martins C,Moura J,Figueiroa S,Garrido C,Martins J,Samoes R,Guimaraes J,Melo C,Sousa R,Palavra F,Ferreira J,da AM,Sa MJ,Santos E. Pediatric neuromyelitis optica spectrum disorders in Portugal: A multicentre retrospective study. Mult. Scler. Relat. Disord. 2022. 59. p. 180-180. IF:4,000. (2).
