PREVENTIVE ANTIEPILEPTIC THERAPY IN PATIENTS WITH TUBEROUS SCLEROSIS

Abstract

Background . Early detection of epileptiform activity on the electroencephalogram (EEG) and preventive antiepileptic therapy in patients with tuberous sclerosis (TS) help to avoid the development of severe forms of epilepsy as well as cognitive disorders associated with it. Materials and methods . Seventeen patients with TS underwent preventive antiepileptic treatment in the Research and Clinical Institute for Pediatrics named after Y.E Vel’tishev. All patients were diagnosed with TS during their first months of life. The diagnosed was based on the detection of cardiac rhabdomyomas and hypopigmentation spots and then confirmed by a genetic test. Antiepileptic drugs (AED) (vigabatrin in 7 patients, valproic acid in 7 patients and levetiracetam in 2 patients) were administered after detection of epileptiform activity in the routine EEG (EEG was performed monthly). Results . Preventive therapy with valproic acid and levetiracetam was found to be ineffective: 4 out of 7 patients on valproic acid and 1 out of 2 patients on levetiracetam developed epilepsy. All of them were later diagnosed with delayed psychological development and delayed speech development. Among 7 patients receiving vigabatrin, only 1 patient developed epilepsy, but did not have any developmental disorders later. Conclusion . Despite vigabatrin is not registered in the Russian Federation, it should be used as an agent for preventive antiepileptic therapy in patients with TS. This approach ensures more favorable long-term results in terms of cognitive functions and psychological development, thus, providing a better quality of life.