Pediatric Anti- N -methyl-D-Aspartate Receptor Encephalitis—Clinical Analysis and Novel Findings in a Series of 20 Patients

Objective To report the clinical features of 20 pediatric patients with anti- N -methyl-D-aspartate receptor (NMDAR) encephalitis. Study design Review of clinical data, long-term follow-up, and immunologic studies performed in a single center in Spain in the last 4 years. Results The median age of t...

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Published in:	The Journal of pediatrics 2013-04, Vol.162 (4), p.850-856.e2
Main Authors:	Armangue, Thaís, MD, Titulaer, Maarten J., MD, PhD, Málaga, Ignacio, MD, PhD, Bataller, Luis, MD, PhD, Gabilondo, Iñigo, MD, Graus, Francesc, MD, PhD, Dalmau, Josep, MD, PhD
Format:	Article
Language:	eng
Subjects:	Adolescent adults Anti-N-Methyl-D-Aspartate Receptor Encephalitis - blood Anti-N-Methyl-D-Aspartate Receptor Encephalitis - diagnosis Brain - pathology Child Child, Preschool children cyclophosphamide electroencephalography encephalitis Female herpes simplex Humans immunoglobulins Immunotherapy - methods Infant intravenous injection Magnetic Resonance Imaging - methods Male patients Pediatrics Pediatrics - methods seizures Spain speech steroids Time Factors Treatment Outcome
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Summary:	Objective To report the clinical features of 20 pediatric patients with anti- N -methyl-D-aspartate receptor (NMDAR) encephalitis. Study design Review of clinical data, long-term follow-up, and immunologic studies performed in a single center in Spain in the last 4 years. Results The median age of the patients was 13 years (range, 8 months-18 years), 70% were female. In 12 patients (60%), the initial symptoms were neurologic, usually dyskinesias or seizures, and in the other 40% psychiatric. One month into the disease, all patients had involuntary movements and alterations of behavior and speech. All patients received steroids, intravenous immunoglobulin or plasma exchange, and 7 rituximab or cyclophosphamide. With a median follow up of 17.5 months, 85% had substantial recovery, 10% moderate or severe deficits, and 1 died. Three patients had previous episodes compatible with anti-NMDAR encephalitis, 2 of them with additional relapses after the diagnosis of the disorder. Ovarian teratoma was identified in 2 patients, 1 at onset of encephalitis and the other 1 year later. Two novel observations (1 patient each) include, the identification of an electroencephalographic pattern (“extreme delta brush”) considered characteristic of this disorder, and the development of anti-NMDAR encephalitis as post herpes simplex encephalitis choreoathetosis. Conclusions The initial symptoms of pediatric anti-NMDAR encephalitis vary from those of the adults (more neurologic and less psychiatric in children), the development of a mono-symptomatic illness is extremely rare (except in relapses), and most patients respond to treatment. Our study suggests a link between post herpes simplex encephalitis choreoathetosis and anti-NMDAR encephalitis.
ISSN:	0022-3476 1097-6833