Autoimmune seizures and epilepsy
Christian Geis,1 Jesus Planagumà,2 Mar Carreño,3 Francesc Graus,2,3 and Josep Dalmau2,3,4,5
1Department of Neurology, Jena University Hospital, Jena, Germany.
2Institut D’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS) and
3Hospital Clinic, University of Barcelona, Barcelona, Spain.
4Catalan Institution for Research and Advanced Studies (ICREA), Barcelona, Spain.
5Department of Neurology, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
Address correspondence to: Josep Dalmau, IDIBAPS–Hospital Clínic, University of Barcelona, Casanova, 143; Floor 3ª, Barcelona 08036, Spain. Phone: 34.932.271.738; Email: jdalmau@clinic.cat.
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1Department of Neurology, Jena University Hospital, Jena, Germany.
2Institut D’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS) and
3Hospital Clinic, University of Barcelona, Barcelona, Spain.
4Catalan Institution for Research and Advanced Studies (ICREA), Barcelona, Spain.
5Department of Neurology, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
Address correspondence to: Josep Dalmau, IDIBAPS–Hospital Clínic, University of Barcelona, Casanova, 143; Floor 3ª, Barcelona 08036, Spain. Phone: 34.932.271.738; Email: jdalmau@clinic.cat.
Find articles by Planagumà, J. in: JCI | PubMed | Google Scholar
1Department of Neurology, Jena University Hospital, Jena, Germany.
2Institut D’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS) and
3Hospital Clinic, University of Barcelona, Barcelona, Spain.
4Catalan Institution for Research and Advanced Studies (ICREA), Barcelona, Spain.
5Department of Neurology, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
Address correspondence to: Josep Dalmau, IDIBAPS–Hospital Clínic, University of Barcelona, Casanova, 143; Floor 3ª, Barcelona 08036, Spain. Phone: 34.932.271.738; Email: jdalmau@clinic.cat.
Find articles by Carreño, M. in: JCI | PubMed | Google Scholar
1Department of Neurology, Jena University Hospital, Jena, Germany.
2Institut D’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS) and
3Hospital Clinic, University of Barcelona, Barcelona, Spain.
4Catalan Institution for Research and Advanced Studies (ICREA), Barcelona, Spain.
5Department of Neurology, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
Address correspondence to: Josep Dalmau, IDIBAPS–Hospital Clínic, University of Barcelona, Casanova, 143; Floor 3ª, Barcelona 08036, Spain. Phone: 34.932.271.738; Email: jdalmau@clinic.cat.
Find articles by Graus, F. in: JCI | PubMed | Google Scholar
1Department of Neurology, Jena University Hospital, Jena, Germany.
2Institut D’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS) and
3Hospital Clinic, University of Barcelona, Barcelona, Spain.
4Catalan Institution for Research and Advanced Studies (ICREA), Barcelona, Spain.
5Department of Neurology, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
Address correspondence to: Josep Dalmau, IDIBAPS–Hospital Clínic, University of Barcelona, Casanova, 143; Floor 3ª, Barcelona 08036, Spain. Phone: 34.932.271.738; Email: jdalmau@clinic.cat.
Find articles by Dalmau, J. in: JCI | PubMed | Google Scholar
First published February 4, 2019 - More info
J Clin Invest. 2019;129(3):926–940. https://doi.org/10.1172/JCI125178.
Copyright © 2019, American Society for Clinical Investigation
The rapid expansion in the number of encephalitis disorders associated with autoantibodies against neuronal proteins has led to an incremental increase in use of the term “autoimmune epilepsy,” yet has occurred with limited attention to the physiopathology of each disease and genuine propensity to develop epilepsy. Indeed, most autoimmune encephalitides present with seizures, but the probability of evolving to epilepsy is relatively small. The risk of epilepsy is higher for disorders in which the antigens are intracellular (often T cell–mediated) compared with disorders in which the antigens are on the cell surface (antibody-mediated). Most autoantibodies against neuronal surface antigens show robust effects on the target proteins, resulting in hyperexcitability and impairment of synaptic function and plasticity. Here, we trace the evolution of the concept of autoimmune epilepsy and examine common inflammatory pathways that might lead to epilepsy. Then, we focus on several antibody-mediated encephalitis disorders that associate with seizures and review the synaptic alterations caused by patients’ antibodies, with emphasis on those that have been modeled in animals (e.g., antibodies against NMDA, AMPA receptors, LGI1 protein) or in cultured neurons (e.g., antibodies against the GABAb receptor).
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ISSN: 0021-9738 (print), 1558-8238 (online)