RT Book, Section A1 Koh, Susan A1 Duchowny, Michael A2 Duchowny, Michael A2 Cross, J. Helen A2 Arzimanoglou, Alexis SR Print(0) ID 1138411056 T1 Tuberous Sclerosis Complex T2 Pediatric Epilepsy YR 2017 FD 2017 PB McGraw-Hill Education PP New York, NY SN 9780071496216 LK neurology.mhmedical.com/content.aspx?aid=1138411056 RD 2024/03/28 AB Tuberous sclerosis complex (TSC) is an inherited neurocutaneous disease of cell differentiation and proliferation affecting multiple organs with hamartomas or abnormal neuronal migration. It was first described by Bourneville in 1880, and the incidence of tuberous sclerosis in the population is reported to be 1/6000 to 1/10,000.1,2 Cortical tubers and subependymal nodules (SEN) are the hallmark pathological findings in children. TSC is caused by aberrant neuronal migration and differentiation. Radial glial fibers guide migration of neurons during development from the 3rd to 5th month of gestation, and if there is a disruption in one tract, hamartomas may develop. This process of abnormal differentiation also explains the occurrence of hamartomas outside the CNS.3