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

Marion Favier, Bastien Joubert, Géraldine Picard, Véronique Rogemond, Laure Thomas, Sylvain Rheims, Marion Bailhache, Frédéric Villega, Jean-Michel Pédespan, Giulia Berzero, Dimitri Psimaras, Jean-Christophe Antoine, Virginie Desestret, Jérôme Honnorat
European Journal of Paediatric Neurology. 2018-05-01; 22(3): 404-411
DOI: 10.1016/j.ejpn.2017.12.014

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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.

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