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Chapter 6 - Limbic 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

Limbic encephalitis is characterized by the subacute onset, usually <3 months, of memory and cognitive deficits, behavioural changes, and seizures. The most typical deficit is impairment of short-term memory. Brain MRI shows FLAIR and T2 signal abnormalities involving bilaterally, less frequently unilaterally, the hippocampus and amygdala. Limbic encephalitis was initially considered a paraneoplastic syndrome, but after the discovery of several immunological subtypes we now know that >60% of cases are non-paraneoplastic and usually associated to LGI1 antibodies. Paraneoplastic limbic encephalitis mainly associates with small-cell lung cancer and Hu or GABAbR antibodies, testicular seminoma and Ma2 antibodies, Hodgkin disease and mGluR5 antibodies, and thymoma and AMPAR antibodies. Limbic encephalitis may be triggered by treatment of cancer with immune checkpoint inhibitors. The response to immunotherapy and outcome vary according to the type of antibody and presence or absence of an underlying tumour. In patients with antibodies against intracellular antigens (onconeural, AK5), immunotherapy is usually ineffective. In contrast, most patients with LGI1 antibodies show substantial improvement after treatment with steroids and immunotherapy. Patients with cancer and GABAbR or AMPAR antibodies respond better to treatment than those with the same type of cancer and onconeural antibodies, and worse than those of patients with LGI antibodies.

Keywords

Limbic encephalitisonconeural antibodies (Hu, Yo, Ri, CV2, SOX1, Ma2)LGI1 antibodiesGABAbR antibodiesAMPAR antibodiesmGluR5 antibodiesadenylate kinase 5 (AK5) antibodiesparaneoplastic neurological syndromes
Type
Chapter
Information
Autoimmune Encephalitis and Related Disorders of the Nervous System , pp. 167 - 190
Publisher: Cambridge University Press
Print publication year: 2022

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  • Limbic 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/10.1017/9781108696722.007
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  • Limbic 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/10.1017/9781108696722.007
Available formats
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  • Limbic 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/10.1017/9781108696722.007
Available formats
×