Encephalitis associated with antibodies against the NMDA receptor
Med Clin (Barc). 2018 Jul 23;151(2):71-79.
doi: 10.1016/j.medcli.2017.10.015.
Epub 2017 Nov 26.
[Article in
English,
Spanish]
Affiliations
- 1 Servei de Neurologia, Institut Clínic de Neurociències, Hospital Clínic, Universitat de Barcelona, Barcelona, España.
- 2 Servei de Neurologia, Institut Clínic de Neurociències, Hospital Clínic, Universitat de Barcelona, Barcelona, España; Laboratori d'Immunologia Clínica i Experimental, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, España; Department of Neurology, University of Pennsylvania, Philadelphia, EE. UU.; Institució Catalana de Recerca i Estudis Avançats (ICREA), Barcelona, España. Electronic address: Jdalmau@clinic.cat.
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; Anticuerpo; Autoimmune; Autoinmune; Encefalitis; Encephalitis; NMDA; Receptor.
Copyright © 2017 Elsevier España, S.L.U. All rights reserved.
Publication types
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Research Support, Non-U.S. Gov't
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Review
MeSH terms
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Anti-N-Methyl-D-Aspartate Receptor Encephalitis* / diagnosis
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Anti-N-Methyl-D-Aspartate Receptor Encephalitis* / immunology
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Anti-N-Methyl-D-Aspartate Receptor Encephalitis* / psychology
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Anti-N-Methyl-D-Aspartate Receptor Encephalitis* / therapy
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Child
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Diagnosis, Differential
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Encephalitis, Viral / complications
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Encephalitis, Viral / diagnosis
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Female
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Humans
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Male
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Mental Disorders / diagnosis
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Neuroleptic Malignant Syndrome / diagnosis
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Ovarian Neoplasms / complications
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Prognosis
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Receptors, N-Methyl-D-Aspartate / immunology*
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Seizures / etiology
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Sex Factors
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Substance-Related Disorders / diagnosis
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Teratoma / complications
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Young Adult
Substances
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Receptors, N-Methyl-D-Aspartate