科研之友
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摘要
AimTo investigate the incidence and severity of anti-N-methyl-d-aspartate (anti-NMDA) receptor encephalitis in children from New Zealand. MethodA retrospective case series was undertaken of all children (18y) diagnosed with anti-NMDA receptor encephalitis from January 2008 to October 2015. ResultsSixteen patients were identified with anti-NMDA receptor antibodies in the cerebrospinal fluid, three of whom had an associated teratoma. Fifteen children had Mori and/or Pacific Island ancestry. The incidence of anti-NMDA receptor encephalitis in Mori children was 3.4 per million children per year (95% confidence interval [CI] 1.4-7.0) and the incidence in Pacific children was 10.0 per million children per year (95% CI 4.3-19.8) compared with 0.2 per million children per year (95% CI 0.0-1.0) in children without Mori or Pacific Island ancestry. Sixty-seven per cent of children had a good outcome (modified Rankin Score 2) at 2 years' follow-up. This compares unfavourably with other cohorts despite a shorter median time to first-line immunotherapy (13d; range 4-89) and a higher proportion of children being treated with second-line therapy (50%). InterpretationMori and Pacific Island children have a higher incidence of anti-NMDA receptor encephalitis and possibly a more severe phenotype. These data suggest a genetic predisposition to anti-NMDA receptor encephalitis in these populations. What this paper adds Mori and Pacific Island children are at increased risk of anti-N-methyl-d-aspartate (NMDA) receptor encephalitis (3.4 and 10.0 per million children per year respectively). One third of Mori and Pacific Island children with anti-NMDA receptor encephalitis have a poor outcome. This article is commented on by Njafang and Missa on page 673 of this issue.
