Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2021 Feb;62(2):303-324.
doi: 10.1111/epi.16788. Epub 2020 Dec 14.

Inflammation, ictogenesis, and epileptogenesis: An exploration through human disease

Tracie Huey-Lin Tan  1   2   3 , Piero Perucca  1   2   3 , Terence J O'Brien  1   2   3 , Patrick Kwan  1   2 , Mastura Monif  1   2   3
Affiliations
Review

Inflammation, ictogenesis, and epileptogenesis: An exploration through human disease

Tracie Huey-Lin Tan et al. Epilepsia. 2021 Feb.

Abstract

Epilepsy is seen historically as a disease of aberrant neuronal signaling manifesting as seizures. With the discovery of numerous auto-antibodies and the subsequent growth in understanding of autoimmune encephalitis, there has been an increasing emphasis on the contribution of the innate and adaptive immune system to ictogenesis and epileptogenesis. Pathogenic antibodies, complement activation, CD8+ cytotoxic T cells, and microglial activation are seen, to various degrees, in different seizure-associated neuroinflammatory and autoimmune conditions. These aberrant immune responses are thought to cause disruptions in neuronal signaling, generation of acute symptomatic seizures, and, in some cases, the development of long-term autoimmune epilepsy. Although early treatment with immunomodulatory therapies improves outcomes in autoimmune encephalitides and autoimmune epilepsies, patient identification and treatment selection are not always clear-cut. This review examines the role of the different components of the immune system in various forms of seizure disorders including autoimmune encephalitis, autoimmune epilepsy, Rasmussen encephalitis, febrile infection-related epilepsy syndrome (FIRES), and new-onset refractory status epilepticus (NORSE). In particular, the pathophysiology and unique cytokine profiles seen in these disorders and their links with diagnosis, prognosis, and treatment decision-making are discussed.

Keywords: autoimmune diseases of the nervous system; cytokines; epilepsy; seizures.

PubMed Disclaimer

References

REFERENCES

    1. Wandinger KP, Leypoldt F, Junker R. Autoantibody-mediated encephalitis. Dtsch Arztebl Int. 2018;5(115):666-73.
    1. Spatola M, Dalmau J. Seizures and risk of epilepsy in autoimmune and other inflammatory encephalitis. Curr Opin Neurol. 2017;30:345-53.
    1. Nabbout R, Vezzani A, Dulac O, Chiron C. Acute encephalopathy with inflammation-mediated status epilepticus . Lancet Neurol. 2011;10:99-108.
    1. Gaspard N, Foreman BP, Alvarez V, Cabrera Kang C, Probasco JC, Jongeling AC, et al. New-onset refractory status epilepticus: etiology, clinical features, and outcome. Neurology. 2015;3(85):1604-13.
    1. Kramer U, Chi CS, Lin KL, Specchio N, Sahin M, Olson H, et al. Febrile infection-related epilepsy syndrome (FIRES): pathogenesis, treatment, and outcome: a multicenter study on 77 children. Epilepsia. 2011;52:1956-65.

Publication types

MeSH terms