What is autoimmune encephalitis?

Autoimmune encephalitis is the term used to describe a broadening group of autoimmune inflammatory diseases that primarily affect the grey matter of the central nervous system. This includes the limbic system, neocortex, basal ganglia, cerebellum and brain stem. The various autoimmune encephalitides can lead to epileptic seizures, movement disorders, coordination disturbances as well as neurocognitive and psychiatric disorders.

On magnetic resonance imaging (MRI) scans, the different autoimmune encephalitides are often characterized by temporary or persistent T2/FLAIR signal intensity and increased volume (swelling) of the predominantly affected structures, which subsequently can result in a volume reduction (atrophy). Frequently, however, no changes will show up on the MRI.

In a proportion of patients, the cerebrospinal fluid (CSF) displays low-grade inflammatory changes such as an increased number of inflammatory cells mainly consisting of B and T lymphocytes, monocytes and macrophages, indicative of lymphocytic and monocytic pleocytosis. A disturbance of the blood-CSF barrier may also be observed involving an increased concentration of protein in the CFS and antibody production triggered by B lymphocytes entering into the blood and CSF, with intrathecal immunoglobulin synthesis being one key feature. In other patients, such inflammatory changes are not detectable.

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CONNECT-GENERATE im aktuellen Newsletter des BMFTR

„Wenn das Gehirn gegen sich selbst kämpft“ – unter diesem Titel berichtet der aktuelle Newsletter des Bundesministeriums für Forschung, Technologie und Raumfahrt (BMFTR) über die Forschung zu Autoimmunen Enzephalitiden und stellt dabei auch CONNECT-GENERATE vor.

Zum Newsletter Gesundheitsforschung BMFTR

Book Recommondation Ratgeber Autoimmune Enzephalitis

Book cover Ratgeber Autoimmune Enzephalitis © Springer
© Springer

Editors: Markus Krämer, Frank Leypoldt

  • Beantwortet Fragen von Betroffenen der Autoimmunenzephalitis
  • Tipps zu Alltagsthemen von Ernährung bis Fahreignung
  • Empfohlen von der Dagmar Joy Schmidt Stiftung und dem Verein GENERATE e.V.

Erweitern Sie Ihr Wissen und sichern Sie sich jetzt Ihr eBook oder gedrucktes Exemplar auf Springer Nature Link.

A Review of “Ratgeber Autoimmune Enzephalitis”

NEMOS Patient:innentag 2025

Datum: 01.10.2025
Tagungsort und Hotel: Sportschule und Bildungsstätte, Frankfurt am Main


References

Translational imaging of TSPO reveals pronounced innate inflammation in human and murine CD8 T cell-mediated limbic encephalitis.

Gallus M, Roll W, Dik A, Barca C, Zinnhardt B, Hicking G, Mueller C, Naik VN, Anstötz M, Krämer J, Rolfes L, Wachsmuth L, Pitsch J, van Loo KMJ, Räuber S, Okada H, Wimberley C, Strippel C, Golombeck KS, Johnen A, Kovac S, Groß CC, Backhaus P, Seifert R, Lewerenz J, Surges R, Elger CE, Wiendl H, Ruck T, Becker AJ, Faber C, Jacobs AH, Bauer J, Meuth SG, Schäfers M, Melzer N. Translational imaging of TSPO reveals pronounced innate inflammation in human and murine CD8 T cell-mediated limbic encephalitis. Sci Adv. 2023 Jun 9;9(23):eabq7595. doi: 10.1126/sciadv. abq7595. Epub 2023 Jun 9. PMID: 37294768; PMCID: PMC10256169.

Crebrospinal fluid proteomics indicates immune dysregulation and neuronal dysfunction in antibody associated autoimmune encephalitis.

Räuber S, Schroeter CB, Strippel C, Nelke C, Ruland T, Dik A, Golombeck KS, Regner-Nelke L, Paunovic M, Esser D, Münch C, Rosenow F, van Duijn M, Henes A, Ruck T, Amit I, Leypoldt F, Titulaer MJ, Wiendl H, Meuth SG, Meyer Zu Hörste G, Melzer N. Cerebrospinal fluid proteomics indicates immune dysregulation and neuronal dysfunction in antibody associated autoimmune encephalitis. J Autoimmun. 2023 Feb;135:102985. doi: 10.1016/j.jaut.2022.102985. Epub 2023 Jan 6. PMID: 36621173.

Cross-reactivity of a pathogenic autoantibody to a tumor antigen in GABAA receptor encephalitis

Brändle SM, Cerina M, Weber S et al. PNAS March 2, 2021 118 (9) e1916337118.

Dieses Paper wurde vom Research4Rare Verbund zum Paper of the Month gewählt und erscheint im April dieses Jahres im BMBF Newsletter!

A Therapeutic Non-self-reactive
SARS-CoV-2 Antibody
Protects from Lung Pathology
in a COVID-19 Hamster Model

Kreye J, Momsen Reincke S, Kornau HC et al.
Cell. 2020 Nov 12;183(4):1058-1069.e19.

CD8+ T-Lymphocyte–Driven Limbic
Encephalitis Results in Temporal Lobe
Epilepsy

Pitsch J, van Loo KMJ, Gallus M et al.
Ann Neurol. 2021; 00:1-20.

Low CSF CD4/CD8+ T-cell proportions are associated with blood-CSF barrier dysfunction in limbic encephalitis.

Hansen N, Schwing K, Önder D, et al. Epilepsy Behav. 2020;102:106682.



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