Dr. Franziska Thaler

Keywords: autoimmune encephalitis, antibodies, B cells, immune repertoire

Research description: Autoantibodies against neuro-glial proteins have been identified in several forms of autoimmune encephalitis (AE). They can be directed against neuronal surface antigens or against intracellular proteins. Clinical presentations range from acute to subacute encephalopathy with neurological and psychiatric symptoms to acute life threatening conditions. Although much progress has been made in the treatment of these conditions, there are still many patients suffering from progressive cognitive decline, therapy-refractive seizures, progressive cerebellar ataxia and other symptoms. We therefore aim to understand mechanisms of dysregulated immune responses in the periphery as well as in the central nervous system in these patients in order to develop more effective and specific therapies.

GSN Students: Michelle Biljecki, Katharina Eisenhut

Selected publications:

Thaler FS, Zimmermann L, Kammermeier S, Strippel C, Ringelstein M, Kraft A, Sühs K-W, Wickel J, Geis C, Markewitz R, Urbanek C, Sommer C, Doppler K, Penner L, Lewerenz J, Rößling R, Finke C, Prüss H, Melzer N, Wandinger K-P, Leypoldt F, Kümpfel T. Rituximab treatment and long-term outcome of patients with autoimmune encephalitis: real-world evidence from the GENERATE registry. Neurol Neuroimmunol Neuroinflamm. 2021 Oct 1;8(6):e1088. doi: 10.1212/NXI.0000000000001088 

Thaler FS, Thaller AL, Schuh E, Biljecki M, Winklmeier S, Mahler CF, Gerhards R, Völk S, Schnorfeil F, Subklewe M, Hohlfeld R, Kümpfel T, Meinl E. Abundant GAD-reactive B cells in GAD-antibody-associated neurological disorders. Ann Neurol. 2019 Jan 11. Mar;85(3):448-454. doi: 10.1002/ana.25414

Thaler FS, Laurent SA, Huber M, Mulazzani M, Dreyling M, Ködel U, Kümpfel T, Straube A, Meinl E, von Baumgarten L. Soluble TACI and soluble BCMA as biomarkers in primary central nervous system lymphoma. Neuro Oncol. 2017 Nov 29;19(12):1618-1627. doi: 10.1093/neuonc/nox097

Laurent SA, Hoffmann FS°, Kuhn PH°, Cheng Q, Chu Y, Schmidt-Supprian M, Hauck SM, Schuh E, Krumbholz M, Rübsamen H, Wanngren J, Khademi M, Ols-son T, Alexander T, Hiepe F, Pfister HW, Weber F, Jenner D, Wekerle H, Hohlfeld R, Lichtenthaler SF, Meinl E. ° FSH and P-HK contributed equally to this work. γ-Secretase directly sheds the survival receptor BCMA from plasma cells. Nat Commun. 2015 Jun 11;6:7333. doi: 10.1038/ncomms8333

Hoffmann FS, Kuhn P-H, Laurent SA, Hauck SM, Berer K, Krumbholz M, Khademi M, Olsson T, Dreyling M, Pfister H-W, Alexander T, Hiepe F, Kümpfel T, Wekerle H, Hohlfeld R, Lichtenthaler SF, Meinl E. The immunoregulator soluble TACI is released by ADAM10 and reflects B cell activation in autoimmunity. J Immunol. 2015 Jan 15;194(2):542-52. doi: 10.4049/jimmunol.1402070