Baboon Model of Genetic Generalized Epilepsy

The epileptic baboon, once studied because of its photosensitivity, has regained importance as a model for human genetic generalized epilepsy (GGE). Because of its electroclinical presentation, natural history, and response to antiepileptic medications, the genetic epilepsy closely resembles juvenile myoclonic epilepsy (JME). By combining intracranial EEG, structural, and functional MRI, it is evident that the occipital lobe plays a more central role in the baboon model of epilepsy, not only by mediating the photoepileptic response, but as a generator of ictal and interictal epileptic discharges. In contrast to earlier hypotheses suggesting that GGE in baboons and humans is due to an abnormal frontothalamic network, changes in cortical structure, whether developmental or seizure-induced, and in functional connectivity, indicate a more diffuse, multilobar disease. In this chapter, we will review EEG, neuroimaging findings, and histopathology in the epileptic baboon, exploring new directions of research into epileptogenesis, behavioral complications, and mortality associated with epilepsy, as well as novel therapies for GGE.