title: The genetic absence epilepsy rat from Strasbourg as a model to decipher the neuronal and network mechanisms of generalized idiopathic epilepsies.
abstract: First characterized in 1982, the genetic absence epilepsy rat from Strasbourg (GAERS) has emerged as an animal model highly reminiscent of a specific form of idiopathic generalized epilepsy. Both its electrophysiological (spike-and-wave discharges) and behavioral (behavioral arrest) features fit well with those observed in human patients with typical absence epilepsy and required by clinicians for diagnostic purposes. In addition, its sensitivity to antiepileptic drugs closely matches what has been described in the clinic, making this model one of the most predictive. Here, we report how the GAERS, thanks to its spontaneous, highly recurrent and easily recognizable seizures on electroencephalographic recordings, allows to address several key-questions about the pathophysiology and genetics of absence epilepsy. In particular, it offers the unique possibility to explore simultaneously the neural circuits involved in the generation of seizures at different levels of integration, using multiscale methodologies, from intracellular recording to functional magnetic resonance imaging. In addition, it has recently allowed to perform proofs of concept for innovative therapeutic strategies such as responsive deep brain stimulation or synchrotron-generated irradiation based radiosurgery.
authors.affiliation: Inserm, U836, F-38000 Grenoble, France; Univ. Grenoble Alpes, Grenoble Institut des Neurosciences, F-38000 Grenoble, France; CHU de Grenoble, Hôpital Michallon, F-38000 Grenoble, France. Electronic address: antoine.depaulis@ujf-grenoble.fr.
authors.forename: Antoine
authors.initials: A
authors.lastname: Depaulis
authors.affiliation: Inserm, U836, F-38000 Grenoble, France; Univ. Grenoble Alpes, Grenoble Institut des Neurosciences, F-38000 Grenoble, France.
authors.forename: Olivier
authors.initials: O
authors.lastname: David
authors.affiliation: Brain and Spine Institute, Pitié-Salpêtrière Hospital, Paris, France; Pierre and Marie Curie University, Paris, France.
authors.forename: Stéphane
authors.initials: S
authors.lastname: Charpier
mesh_terms: D000200:Action Potentials; D000818:Animals; D004195:Disease Models, Animal; D004832:Epilepsy, Absence / Q000503:physiopathology*; D008959:Models, Neurological; D009415:Nerve Net / Q000503:physiopathology*; D009474:Neurons / Q000378:metabolism*; D051381:Rats; D055647:Rats, Transgenic / Q000502:physiology*; D013003:Somatosensory Cortex / Q000503:physiopathology*
keywords: Absence epilepsy; Animal model; Cortex; Electrophysiology; Epilepsy; Magnetic resonance imaging; Neural network