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Chapter 8 - Anti-NMDAR Encephalitis

from Section 3 - Specific Syndromes and Diseases

Published online by Cambridge University Press:  27 January 2022

Josep Dalmau  and
Francesc Graus
Josep Dalmau
Affiliation:
Universitat de Barcelona
Francesc Graus
Affiliation:
Universitat de Barcelona
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Summary

Anti-NMDAR encephalitis is the most frequent autoimmune encephalitis. It predominantly occurs in children and young females. Up to 80% of patients present with severe insomnia and psychiatric and behavioural symptoms that resemble those of psychotic episodes caused by primary psychiatric diseases. In addition to the psychiatric manifestations, patients develop neurological symptoms including seizures, abnormal movements, reduced verbal output, and dysautonomic features. Up to 50% of young females have an underlying ovarian teratoma that contains nervous tissue and NMDAR, which probably trigger the immune response. Less frequently, the encephalitis is triggered by an episode of herpes simplex encephalitis probably through the release of antigens by neurons damaged by the virus. The diagnosis of anti-NMDAR encephalitis requires the demonstration of the antibodies in CSF. Up to 14% of patients do not have detectable antibodies in serum. A positive result in serum but negative in CSF must be taken with caution as these patients do not present clinical features of encephalitis and many represent false positive results. Between 80% and 90% of patients respond to treatment which includes immunotherapy and removal of the tumour when it applies.

Keywords

Autoimmune encephalitisNMDAR antibodiesmovement disordersseizurespsychosisovarian teratomaherpes simplex encephalitis
Type
Chapter
Information
Autoimmune Encephalitis and Related Disorders of the Nervous System , pp. 210 - 254
Publisher: Cambridge University Press
Print publication year: 2022

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  • Anti-NMDAR Encephalitis
  • Josep Dalmau, Universitat de Barcelona, Francesc Graus, Universitat de Barcelona
  • Book: Autoimmune Encephalitis and Related Disorders of the Nervous System
  • Online publication: 27 January 2022
  • Chapter DOI: https://doi.org/10.1017/9781108696722.009
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  • Anti-NMDAR Encephalitis
  • Josep Dalmau, Universitat de Barcelona, Francesc Graus, Universitat de Barcelona
  • Book: Autoimmune Encephalitis and Related Disorders of the Nervous System
  • Online publication: 27 January 2022
  • Chapter DOI: https://doi.org/10.1017/9781108696722.009
Available formats
×

Save book to Google Drive

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Google Drive.

  • Anti-NMDAR Encephalitis
  • Josep Dalmau, Universitat de Barcelona, Francesc Graus, Universitat de Barcelona
  • Book: Autoimmune Encephalitis and Related Disorders of the Nervous System
  • Online publication: 27 January 2022
  • Chapter DOI: https://doi.org/10.1017/9781108696722.009
Available formats
×