Video 1.

Catatonia and epileptic seizures in mice after intrathecal infusion of GABAAR mAbs. (A) Control mouse receiving high-dose control IgG showed normal explorative behavior 3 d after intrathecal infusion. (B) In contrast, infusion of high-dose GABAAR mAb #113-115 led to severe catatonia and focal epileptic seizures after 3 d. (C) Direct comparison of spontaneous behavior in mice receiving either GABAAR (left) or control mAb #mGO53 (right). (D) In mice receiving lower doses of GABAAR mAb, catatonia and focal seizures occurred later (day 6) and were less pronounced. (E) Worsening of motor abnormalities at day 10 in animals receiving high-dose GABAAR mAb with hyperexcitability and increased seizure frequency. (F) In contrast, control mice remained healthy and showed normal explorative behavior after 14 d of intrathecal mAb infusion. conc., concentration; d3, day 3; d4, day 4; HL, heavy λ; HK, heavy κ.


Encephalitis patient-derived monoclonal GABAA receptor antibodies cause epileptic seizures

Jakob Kreye, Sukhvir K. Wright, Adriana van Casteren, Laura Stöffler, Marie-Luise Machule, S. Momsen Reincke, Marc Nikolaus, Scott van Hoof, Elisa Sanchez-Sendin, Marie A. Homeyer, César Cordero Gómez, Hans-Christian Kornau, Dietmar Schmitz, Angela M. Kaindl, Philipp Boehm-Sturm, Susanne Mueller, Max A. Wilson, Manoj A. Upadhya, Divya R. Dhangar, Stuart Greenhill, Gavin Woodhall, Paul Turko, Imre Vida, Craig C. Garner, Jonathan Wickel, Christian Geis, Yuko Fukata, Masaki Fukata, and Harald Prüss

DOI: 10.1084/jem.20210012
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