title: Treatment and outcome of children and adolescents with N-methyl-D-aspartate receptor encephalitis.
abstract: 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.
authors.affiliation: French Reference Center of Paraneoplastic Neurological Syndromes, Hospices Civils de Lyon, Hôpital Neurologique, 69677, Bron, France, anastasia.zek@gmail.com.
authors.forename: Anastasia
authors.initials: A
authors.lastname: Zekeridou
authors.forename: Evgenia
authors.initials: E
authors.lastname: Karantoni
authors.forename: Aurélien
authors.initials: A
authors.lastname: Viaccoz
authors.forename: François
authors.initials: F
authors.lastname: Ducray
authors.forename: Cyril
authors.initials: C
authors.lastname: Gitiaux
authors.forename: Frédéric
authors.initials: F
authors.lastname: Villega
authors.forename: Kumaran
authors.initials: K
authors.lastname: Deiva
authors.forename: Veronique
authors.initials: V
authors.lastname: Rogemond
authors.forename: Elodie
authors.initials: E
authors.lastname: Mathias
authors.forename: Géraldine
authors.initials: G
authors.lastname: Picard
authors.forename: Marc
authors.initials: M
authors.lastname: Tardieu
authors.forename: Jean-Christophe
authors.initials: JC
authors.lastname: Antoine
authors.forename: Jean-Yves
authors.initials: JY
authors.lastname: Delattre
authors.forename: Jerome
authors.initials: J
authors.lastname: Honnorat
mesh_terms: D000293:Adolescent; D000367:Age Factors; D060426:Anti-N-Methyl-D-Aspartate Receptor Encephalitis / Q000503:physiopathology / Q000628:therapy*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007167:Immunotherapy / Q000379:methods*; D007223:Infant; D008297:Male; D017063:Outcome Assessment, Health Care; D010951:Plasma Exchange / Q000379:methods; D011379:Prognosis; D012189:Retrospective Studies; D012720:Severity of Illness Index