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EEC 2022 | Cerebral organoids of focal cortical dysplasia

Iscia Lopes-Cendes, MD, PhD, University of Campinas, Campinas, Brazil, discusses the development of organoids for studying focal cortical dysplasia (FCD). FCD is the most frequent cause of severe epilepsy in children and not far behind as the second most common cause in adults. FCD is also associated with epilepsy that is hard to treat with current medications. A limitation in investigating treatment options for this type of epilepsy is the fact that animal models of FCD do not sufficiently reproduce the hallmarks of the disease. Therefore, there has been a movement towards constructing a model closer to the reality in patients. For the first time, in vitro cortical organoids, created using materials from the patients, are showing potential in this setting. These human models are more representative of the emergence of FCD during developmental stages of the cerebral cortex compared to animal models. The two main findings derived from assessing these cerebral organoids were that it: 1) recapitulated characteristics of patients with FCD, including pathological hallmarks, such as lesions, dysmorphic neurons, and impaired cell proliferation, and 2) showed the abnormal formation of neuronal circuitry causing discharges as observed in FCD. Due to this, the organoids have prospects in drug testing and in addition, have increased our understanding of the molecular abnormalities that cause the developmental malformation. This interview took place at the European Epilepsy Congress (EEC) 2022.