INTRODUCTION: The mechanism of epilepsy in Sturge-Weber syndrome (SWS) has not been elucidated, since it is difficult to detect epileptic discharges on scalp EEG records. To determine the electrophysiological characteristics of SWS, we evaluated epileptic discharges using intracranial electrocorticography (ECoG). CASE SUMMARY: We report a 1-year and 3-month-old boy diagnosed with SWS whose psychomotor development was impaired despite good control of his seizures with several antiepileptic drugs (AEDs). Prolonged video ECoG monitoring was performed to determine whether subclinical seizures had induced his developmental delay. DISCUSSION: Frequent seizures were detected as motionless staring and respiratory distress. His seizures originated from multiple areas along the Sylvian fissure, propagated very slowly, and lasted for 10 to 30 min. Left hemispherotomy was performed, and he was confirmed to be completely seizure-free after surgery. Our findings indicate that long-lasting and barely discernible seizures may continue in patients with SWS who appear to have good control of epilepsy with AEDs.
INTRODUCTION: The mechanism of epilepsy in Sturge-Weber syndrome (SWS) has not been elucidated, since it is difficult to detect epileptic discharges on scalp EEG records. To determine the electrophysiological characteristics of SWS, we evaluated epileptic discharges using intracranial electrocorticography (ECoG). CASE SUMMARY: We report a 1-year and 3-month-old boy diagnosed with SWS whose psychomotor development was impaired despite good control of his seizures with several antiepileptic drugs (AEDs). Prolonged video ECoG monitoring was performed to determine whether subclinical seizures had induced his developmental delay. DISCUSSION: Frequent seizures were detected as motionless staring and respiratory distress. His seizures originated from multiple areas along the Sylvian fissure, propagated very slowly, and lasted for 10 to 30 min. Left hemispherotomy was performed, and he was confirmed to be completely seizure-free after surgery. Our findings indicate that long-lasting and barely discernible seizures may continue in patients with SWS who appear to have good control of epilepsy with AEDs.
Authors: A A Arzimanoglou; F Andermann; J Aicardi; C Sainte-Rose; M A Beaulieu; J G Villemure; A Olivier; T Rasmussen Journal: Neurology Date: 2000-11-28 Impact factor: 9.910
Authors: F E Jansen; A C van Huffelen; ThD Witkamp; A Couperus; N Teunissen; G H Wieneke; O van Nieuwenhuizen Journal: Clin Neurophysiol Date: 2002-07 Impact factor: 3.708