Tuberous sclerosis complex (TSC) is a genetic multisystem disorder characterized 
by widespread hamartomas in several organs, including the brain, heart, skin, 
eyes, kidney, lung, and liver. The affected genes are TSC1 and TSC2, encoding 
hamartin and tuberin respectively. The hamartin-tuberin complex inhibits the 
mammalian-target-of-Rapamycin (mTOR) pathway, which controls cell growth and 
proliferation. Variations in the distribution, number, size, and location of 
lesions cause the clinical syndrome to vary even between relatives. About 85% of 
children and adolescents with TSC have CNS complications, including epilepsy, 
cognitive impairment, challenging behavioral problems, and autism-like symptoms. 
Epilepsy generally begins during the first year of life, with focal seizures and 
spasms. The discovery of the mTOR pathway upregulation in TSC-associated lesions 
presents new possibilities for treatment strategy. Increasing understanding of 
the molecular abnormalities caused by TSC may enable improved management of the 
disease.