Initial clinical presentation of young children with N-methyl-d-aspartate receptor encephalitis. (May 2018)

• Record Type: Journal Article
• Authors: Favier, Marion; Joubert, Bastien; Picard, Géraldine; Rogemond, Véronique; Thomas, Laure; Rheims, Sylvain; Bailhache, Marion; Villega, Frédéric; Pédespan, Jean-Michel; Berzero, Giulia; Psimaras, Dimitri; Antoine, Jean-Christophe; Desestret, Virginie; Honnorat, Jérôme
• Is Part Of: European journal of paediatric neurology. Volume 22:Number 3(2018:May)
• Page Start: 404
• Page End: 411
• Publication Date: 2018-05
• Subjects: Autoimmune encephalitis -- N-methyl-d-aspartate receptor -- Child -- Focal seizures; Abs antibodies -- CSF cerebrospinal fluid -- EEG electroencephalogram -- ENT ear, nose and throat -- FDG-PET fluoro-d-glucose positron emission tomography -- FLAIR fluid attenuated inversion recovery -- HSV Herpes Simplex Virus -- IQR interquartile range -- MRI magnetic resonance imaging -- mRS modified Rankin Scale -- NMDA N-methyl-d-aspartate -- NMDA-R-Abs anti-N-methyl-d-aspartate receptor antibodies; Pediatric neurology -- Periodicals; Nervous System Diseases -- Periodicals; Child -- Periodicals; Infant -- Periodicals; Neurologie pédiatrique -- Périodiques; Pediatric neurology; Electronic journals; Periodicals; Electronic journals; 618.928
• DOI: 10.1016/j.ejpn.2017.12.014
• Languages: English
• ISSNs: 1090-3798

Abstract

Abstract: Autoimmune encephalitis with anti-N-methyl-d -aspartate receptor autoantibodies (NMDA-R-Abs) is a recently described disease affecting adult and pediatric patients. Symptoms of the disease are now perfectly described in the adult population but the clinical presentation is less known in young children. The aim of the present study was to describe the clinical presentation and the specificities of symptoms presented by young children with NMDA-R-Abs encephalitis to improve diagnosis of this disease, and to compare these to a series of previously published female adult patients. Fifty cases of children younger than twelve years of age diagnosed with NMDA-R-Abs encephalitis between January 1, 2007 and December 31, 2016 (27 females and 23 males) were retrospectively studied. The first neurological symptoms observed in young children with NMDA-R-Abs encephalitis were characterized by seizure (72%), especially focal seizure (42%), within a median of 15 days before other encephalitis symptoms; other patients mostly had behavioral disorders (26%). The seizures were frequently difficult to diagnose because of the transient unilateral dystonic or tonic posturing presentation or sudden unilateral pain in the absence of clonic movements. A post-ictal motor deficit was also frequently observed. This clinical presentation is different from that observed in adult females with NMDA-R-Abs encephalitis who initially present mainly psychiatric disorders (67%) or cognitive impairment (19%), and less frequently seizures (14%). The diagnosis of NMDA-R-Abs encephalitis should be systematically considered in young children of both sexes who present neurological symptoms suggesting recent seizures (focal or generalized) without obvious other etiology. Highlights: First clinical symptom of young children with NMDA-R-Abs encephalitis is different than adult female. Generalized or focal seizures are the most frequent first neurological symptom of young children with NMDA-R-Abs encephalitis. Focal seizures are difficult to diagnose because of presentation with transient unilateral dystonic or tonic posturing.