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Introduction: Tuberous sclerosis complex (TSC), is a multisystem autosomic dominant genetic disorder, which is clinically diagnosed. The disease manifestations vary significantly among affected individuals. The main neurological symptoms are seizures, cognitive impairment, neuronal migration defects and brain tumors (cortical/subcortical tubers, subependymal nodules [SEN], and subependymal giant cell astrocytomas [SEGA]). The genetic origin of TSC is located in 2 genes encoding hamartin (TSC1) and tuberin (TSC2), which act by regulating cell growth. The incidence varies depending on the series between 1/5800 and 1/6000 births. Objective: To report the clinical presentations of TSC in Paraguay and our experience in the clinical management of them. Methodology: Review of cases of patients treated at the Department of Neurology between January 2011 and August 2013. Cases Reports: We report eight patients from Paraguay, 4 males and 4 females, aged between 1 and 13 years, diagnosed and treated at the Neurology Department, between January 2011 and August 2013. Conclusion: The TSC is a relatively low incidence disease, but with a lot neurological symptoms. The knowledge of the disease is critical to Neuropaediatricians, for improve the quality of life of the patients and to prevent the future affectations that are usually presented in this disease.

Tuberous Sclerosis Complex, Seizures, Cognitive Impairment, Neuronal Migrations Defects, SEGA.