Treatment and outcome of children and adolescents with N-methyl-d-aspartate receptor encephalitis
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.
KeywordsPediatric autoimmune encephalitis NMDA receptor encephalitis
We would like to thank all of the physicians, patients and relatives who helped to collect the clinical data. CSF samples were collected with the help of Neurobiotec Bank and the Hospices Civils de Lyon.
Conflicts of interest
On behalf of all authors, the corresponding authors state that there is no conflict of interest.
This study has been approved by the institutional review board and ethics committee of the University Claude Bernard Lyon 1/Hospices Civils de Lyon.
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