TY - CHAP M1 - Book, Section TI - Tuberous Sclerosis Complex A1 - Koh, Susan A1 - Duchowny, Michael A2 - Duchowny, Michael A2 - Cross, J. Helen A2 - Arzimanoglou, Alexis Y1 - 2017 N1 - T2 - Pediatric Epilepsy 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 SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/04/19 UR - accesspediatrics.mhmedical.com/content.aspx?aid=1138411056 ER -