Prolonged Q-T interval syndrome presenting as idiopathic epilepsy

We report the case of a 4 1/2-year-old girl admitted to our Hospital because of repeated tonic convulsions. These attacks were triggered by noxious stimuli as well as by emotional stress. Since patient's history was not typical of idiopathic epilepsy, and several electroencephalograms failed to reveal any abnormality, a cardiac basis for the clinical picture was suspected. Resting electrocardiograms and 48-hour ECG recording showed a prolonged Q-T interval, usually responsible for severe cardiac arrhythmias (e.g., ventricular fibrillation or "torsades de pointe"). In our patient the neurologic paroxysmal symptomatology, wrongly considered as idiopathic epilepsy, should be interpreted being due to the underlying cardiac abnormality.