Abstract

Several genetic models of absence epilepsy have been developed, since the 1960s, in both mice and rats, based on the observation that some animals display spontaneous spike-and-wave discharges during cortical EEG recording, concomitant with behavioral arrests. For most of these models, the electrophysiological, behavioral, and pharmacological features are quite reminiscent of those observed in human patients with typical absence epilepsy. Here, we describe the different models, how they have been discovered and developed, how seizures occur, which comorbidities are associated, and how they can be used to better understand the physiopathology of absence epilepsy and its genesis. In particular, these models offer the opportunity to explore the neural circuits involved in the generation of seizures at different levels of integration, from unitary recording to functional magnetic resonance imaging. In addition, they allow performance of proof of concept experiments for innovative therapeutic strategies, and identification of early biomarkers.

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