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Eur J Paediatr Neurol. 2018 May;22(3):404-411. doi: 10.1016/j.ejpn.2017.12.014. Epub 2017 Dec 28.

Initial clinical presentation of young children with N-methyl-d-aspartate receptor encephalitis.

Author information

1
Service des Urgences Pédiatriques, Centre Hospitalier Universitaire de Bordeaux Groupe Pellegrin, Place Amélie Raba-Léon, 33076 Bordeaux Cedex, France.
2
Centre Français de Référence des Syndromes Neurologiques Paranéoplasiques, Hospices Civils de Lyon, Hôpital Neurologique, F-69677 Bron, France; Université Claude Bernard Lyon 1, CNRS UMR-5310, INSERM U-1217, Institut NeuroMyoGène, F-69003 Lyon, France.
3
Centre Français de Référence des Syndromes Neurologiques Paranéoplasiques, Hospices Civils de Lyon, Hôpital Neurologique, F-69677 Bron, France.
4
Service de Neurologie Fonctionnelle et d'Epileptologie, Hospices Civils de Lyon, 59 Boulevard Pinel, Hôpital Neurologique, F-69677 Bron, France.
5
Service de Neurologie Pédiatrique, Centre Hospitalier Universitaire de Bordeaux Groupe Hospitalier Pellegrin, Place Amélie Raba-Léon, 33076 Bordeaux Cedex, France.
6
Centre Français de Référence des Syndromes Neurologiques Paranéoplasiques, Hospices Civils de Lyon, Hôpital Neurologique, F-69677 Bron, France; Service de Neurologie Mazarin, Groupe Hospitalier Pitié-Salpêtrière, APHP, Université Pierre et Marie Curie-Paris 6, Centre de Recherche de l'Institut du Cerveau et de la Moelle épinière, UMR S975, CNRS, UMR, 7225 Paris, France.
7
Centre Français de Référence des Syndromes Neurologiques Paranéoplasiques, Hospices Civils de Lyon, Hôpital Neurologique, F-69677 Bron, France; Service de Neurologie, CHU de Saint-Etienne, 42055 Saint-Etienne Cedex, France.
8
Centre Français de Référence des Syndromes Neurologiques Paranéoplasiques, Hospices Civils de Lyon, Hôpital Neurologique, F-69677 Bron, France; Université Claude Bernard Lyon 1, CNRS UMR-5310, INSERM U-1217, Institut NeuroMyoGène, F-69003 Lyon, France. Electronic address: jerome.honnorat@chu-lyon.fr.

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.

KEYWORDS:

Autoimmune encephalitis; Child; Focal seizures; N-methyl-d-aspartate receptor

PMID:
29310866
DOI:
10.1016/j.ejpn.2017.12.014
[Indexed for MEDLINE]

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