Epilepsy surgery in tuberous sclerosis: The Dutch experience

SummaryIntroductionEpilepsy associated with tuberous sclerosis complex (TSC) is drug resistant in more than half of the patients. Epilepsy surgery may be an alternative treatment option, if the epileptogenic tuber can be identified reliably and if seizure reduction is not at the expense of cognitive or other functions. We report the pre-surgical identification of the epileptogenic tuber and post-surgical outcome of patients with TSC in The Netherlands.MethodsTwenty-five patients underwent the pre-surgical evaluation of the Dutch Comprehensive Epilepsy Surgery Programme, including a detailed seizure history, interictal and ictal video EEG registrations, 3D FLAIR MRI scans and neuropsychological testing. Suitability of the candidates was decided in consensus. Seizure outcome, scored with the Engel classification, and cognition were reassessed at fixed post-surgery intervals.ResultsEpilepsy surgery was performed in six patients. At follow-up, four patients had Engel classification 1, two had classification 4. Improved development and behaviour was perceived by the parents of two patients. Epilepsy surgery was not performed in 19 patients because seizures were not captured, ictal onset zones could not be localised or were multiple, interictal EEG, video EEG and MEG results were not concordant, or seizure burden had diminished during decision making. A higher cognition index was found in the surgical patients compared to the non-surgical candidates.ConclusionsEpilepsy surgery can be performed safely and successfully in patients in whom semiology, interictal EEG, ictal EEG, MEG and the location of tubers are concordant. In other cases the risk of surgery should be weighed against the chance of seizure relief and in case of children subsequent impact on neurodevelopment

Not only a matter of epilepsy: Early problems of cognition and behavior in children with "epilepsy only" - A prospective, longitudinal, controlled study starting at diagnosis

Objective. To understand early educational and behavioral predicament in childhood "epilepsy only." Methods. A multicenter, prospective, longitudinal study was conducted of 51 outpatient schoolchildren with newly diagnosed idiopathic or cryptogenic epilepsy and 48 sex-matched classmate control subjects. All children underwent neuropsychological assessment 3 times within the first year after diagnosis; parents and teachers completed behavior questionnaires, and patients' parents were interviewed to inventory contextual adversity. Principal components analysis of cognition and behavior disclosed 6 major components that were related with the interview data ( repeated measures analysis of variance). Results. Despite similar intelligence and educational background, significantly more patients (51%) than control subjects (27%) required special educational assistance. Patients obtained worse scores across components of cognition and behavior. Parents and teachers perceived patients to have more behavioral problems. Differences between groups existed at pretreatment baseline. Over time, notwithstanding stable percentages of poor scores in both groups, nonpersistence of poor scores was impressive ( each time other children scored poorly in other domains). Rather than epilepsy characteristics, contextual adversities were significant risk factors. Conclusion. Already in the earliest stage of the illness, children with epilepsy are liable to vicissitudes in cognitive and behavioral functioning. Contextual variables are all-important