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Inicio Medicina Clínica (English Edition) Encephalitis associated with antibodies against the NMDA receptor
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Vol. 151. Issue 2.
Pages 71-79 (July 2018)
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Vol. 151. Issue 2.
Pages 71-79 (July 2018)
Review
Encephalitis associated with antibodies against the NMDA receptor
Encefalitis por anticuerpos contra el receptor de NMDA
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6
Mar Guaspa, Josep Dalmaua,b,c,d,
Corresponding author
Jdalmau@clinic.cat

Corresponding author.
a Servei de Neurologia, Institut Clínic de Neurociències, Hospital Clínic, Universitat de Barcelona, Barcelona, Spain
b Laboratori d’Immunologia Clínica i Experimental, Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain
c Department of Neurology, University of Pennsylvania, Philadelphia, United States
d Institució Catalana de Recerca i Estudis Avançats (ICREA), Barcelona, Spain
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Table 1. Diagnostic criteria for anti-NMDAR encephalitis.
Table 2. Differential diagnosis of anti-NMDAR encephalitis.
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Abstract

The encephalitis associated with antibodies against the N-methyl-d-aspartate receptor (NMDAR) is characterized by the presence of antibodies against the GluN1 subunit of this receptor, resulting in symptoms that are similar to those observed in models of genetic or pharmacologic reduction of NMDARs. Patients are usually young adults, predominantly women, and children who develop, in a sequential manner, rapidly progressive symptoms including psychosis, abnormal movements, autonomic dysfunction, and coma. Epileptic seizures are variable and can occur throughout the course of the disease. The disease is often mistaken as viral encephalitis, primary psychiatric disorders, drug abuse, or neuroleptic malignant syndrome. About 50% of young women have an ovarian teratoma; in young girls and men the presence of a tumour is infrequent. In some patients, the disease is triggered by herpes simplex encephalitis. The recognition of anti-NMDAR encephalitis is important because, despite its severity, most patients respond to immunotherapy.

Keywords:
Antibodies
Autoimmune
Encephalitis
NMDA
Receptor
Resumen

La encefalitis asociada a anticuerpos contra el receptor N-metil-D-aspartato (NMDAR) se caracteriza por la presencia de anticuerpos contra la subunidad GluN1 del NMDAR, resultando en síntomas parecidos a los observados en modelos de alteración genética o antagonistas farmacológicos del receptor. Los pacientes suelen ser adultos jóvenes, predominantemente mujeres, y niños/as que presentan de manera rápida y secuencial: psicosis, movimientos anormales, disfunción autonómica y coma. Las crisis epilépticas son variables y pueden ocurrir en cualquier momento de la evolución. La enfermedad suele confundirse con encefalitis virales, procesos psiquiátricos primarios, ingesta de drogas y síndrome neuroléptico maligno. El 50% de las mujeres jóvenes tienen un teratoma de ovario; en niñas y varones, la presencia de un tumor es infrecuente. En algunos pacientes la enfermedad es iniciada por una encefalitis herpética. El reconocimiento de la encefalitis anti-NMDAR es importante porque, a pesar de su gravedad, la mayoría de los pacientes responden a la inmunoterapia.

Palabras clave:
Anticuerpo
Autoinmune
Encefalitis
NMDA
Receptor

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