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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Thema: Patienten mit AQP4-IgG-positiver NMOSD und Neudiagnose einer malignen hämatologischen Erkrankung unter Immuntherapie mit Azathioprin.

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References

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.

An expanded parenchymal CD8+ T cell clone in GABAA receptor encephalitis.

Bracher A, Alcalá C, Ferrer J, et al. Ann Clin Transl Neurol. 2020;7(2):239‐244.

Routine diagnostics for neural antibodies, clinical correlates, treatment and functional outcome

Bien CG, Bien CI, Dogan Onugoren M, et al. [published online ahead of print, 2020 Apr 3] [published correction appears in J Neurol. 2020 May 12;:]. J Neurol. 2020;10.1007/s00415-020-09814-3.



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