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NMDA Receptor Antibody Encephalitis

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Abstract

N-methyl-d-aspartate receptor (NMDAR) antibody encephalitis is a recently described immunotherapy-responsive panencephalitis with characteristic features that include a psychiatric onset and a later movement disorder. This entity was first described as a paraneoplastic phenomenon in young women with ovarian teratomata. However, more recently it has become clear that the majority of patients, particularly children, do not harbor a tumor and that males can also be affected. With the development of the NMDAR antibody assay, now available worldwide, a few patients with classical limbic encephalitis and early psychosis and epilepsy have also been found to harbor these antibodies. Early diagnosis followed by immunotherapies and tumor removal, when relevant, expedite recovery from the condition. Antibody levels correlate with the clinical severity of the disease in individual patients, and the antibodies have been shown to substantially reduce NMDA receptors on hippocampal neurons both in vitro and in vivo, supporting the likely direct pathogenicity of the NMDAR antibodies.

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Acknowledgment

S.R. Irani was supported by the National Institute of Health Research (NIHR), Department of Health, United Kingdom.

Disclosure

Conflicts of interest: S.R. Irani: royalties may be available in the future for Caspr2 and Lgi1 antibody testing; A. Vincent: has been a consultant for Athena Diagnostics, receives a part-time salary from University of Oxford and University College London, has received a grant from Euroimmun AG and from NIHR United Kingdom for development of antibody assays, and royalties may be available in the future for Caspr2 and Lgi1 antibody testing.

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Authors and Affiliations

  1. Nuffield Department of Clinical Neurosciences, Level 6, West Wing, John Radcliffe Hospital, Headley Way, Oxford, OX3 9DS, UK

    Sarosh R. Irani & Angela Vincent

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  1. Sarosh R. Irani
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  2. Angela Vincent
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Correspondence to Sarosh R. Irani.

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Irani, S.R., Vincent, A. NMDA Receptor Antibody Encephalitis. Curr Neurol Neurosci Rep 11, 298–304 (2011). https://doi.org/10.1007/s11910-011-0186-y

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