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Published on: April 14, 2014
Pediatric neuromyelitis optica spectrum disorders in Portugal: A multicentre retrospective study
Cecília Martins1, João Moura2, Sónia Figueiroa3
1Department of Pediatrics, Centro Hospitalar do Médio Ave, V. N. Famalicão, Portugal; Department of Pediatric Neurology, Centro Materno Infantil do Norte/Centro Hospitalar Universitário do Porto, Porto, Portugal.
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.
Objective And Methods:
Description of pediatric NMOSD cases identified in a national multicentric NMOSD Portuguese registry.
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 anti-MOG-positive) were on maintenance immunosuppressive therapy at the time of data collection.
Conclusion:
NMOSD may present in pediatric age. It is essential to establish the diagnosis and promptly start therapy to improve the prognosis.
