Treatment and outcome of children and adolescents with N-methyl-D-aspartate receptor encephalitis.
J Neurol. 2015-05-19; 262(8): 1859-1866
DOI: 10.1007/s00415-015-7781-9
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The objective of this study is to describe the treatment and outcome of children
and adolescents with N-methyl-D-aspartate receptor (NMDA-R) encephalitis. A
retrospective study of children and adolescents with NMDA-R encephalitis was
performed by the French Paraneoplastic Neurological Syndrome Reference Center
between January 1, 2007 and December 31, 2012. The modified Rankin scale (mRS)
was used to assess outcome. Thirty-six children and adolescents with NMDA-R
encephalitis were studied. All of the patients received first-line immunotherapy
(corticosteroids, intravenous immunoglobulins or plasma exchange), and 81%
received second-line immunotherapy (rituximab or cyclophosphamide). Median time
between first-line and second-line treatment was 26 days. During the first 24
months, 30 of 36 patients (83%) achieved a good outcome (mRS ≤ 2) and 20 of 36
patients (56%) achieved complete recovery (mRS = 0). Median time to good outcome
and to complete recovery was 6 and 24 months, respectively. Three patients (8%)
relapsed, one patient died. In multivariate analysis, age >12 years was a
predictor of good outcome and initial mRS ≤ 3 was a predictor of complete
recovery. Despite a higher rate of patients who received second-line
immunotherapy, the outcome of the patients in the present series was very similar
to the outcome reported in previous series. The present study highlights the need
for clinical trials to determine the optimal treatment of NMDA-R encephalitis.