Introduction Tuberous sclerosis (TS) is a genetic, multisystemic, likely to cause central nervous system tumors. Seizures are common manifestations are the main therapeutic problem. Objetive: To describe the epileptic characteristics, of pediatric patients with diagnosed with tuberous sclerosis complex (TSC), controlled in the Pediatric Neurology Department University Hospital Institute of Los Andes. Methods: was performed an observational, retrospective, case series, with ET and Epilepsy. Described: sex, age of the diagnosis and initiation of crisis, reason for visit, seizure type, electroencephalographic findings and images, behavioral disorders, severity of intellectual engagement and dermatologic manifestations. Results: Twelve patients met the criteria diagnostic CET, 10 (83%) were epileptic, of these 50% passed with epilepsy of difficult control. 60% had partial seizures (40%) generalized. The 100% showed EEG abnormalities, hypsarrhythmic pattern 30% . The 50% of cases had structural abnormalities, 80% cortical tuber type. In 70% was achieved crisis control with valproic acid and in one case was required strict ketogenic diet .The extraneurological sign more constant were the hypochromic macules (100%). Conclusion: Although seizures are not part of the diagnostic criteria, are the most frequent reason for consultation in partnership with hypochromic macules to suspect a diagnosis of ET. The variety, refractoriness and early onset of crisis, often require polytherapy to control, which favors the patient's prognosis.
Tópico:
Tuberous Sclerosis Complex Research
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FuenteArchivos Venezolanos de Puericultura y Pediatría