Zenha, R.Costa, A.Costa, M.Rodrigues, M.Chorão, R.2011-06-012011-06-012010Nascer e Crescer 2010; 19(1): 46-470872-0754http://hdl.handle.net/10400.16/663ABSTRACT We present the case of a boy with in utero diagnosis of tuberous sclerosis (TS), suspected by the finding of cardiac rabdomyomas and confirmed by fetal brain MRI with typical subependymal nodules and sucortical tubers. We emphasise that epileptic spasms are very frequent and usually the presenting seizures is TS, as occurred in our patient. Electroencephalogram revealed an atypical hypsarrythmia, and the patient responded to vigabatrin. The need to be aware of this epileptic encephalopathy in TS and the importance of an early treatment are underlined.porTuberous sclerosisprenatal diagnosiselectroencephalogramepileptic spasmsjournal article