Abstracts

Rationale: Early life seizures especially epileptic spasms has been shown to be one of the risk factor for severe to profound  cognitive impairment and autism-like behaviour in children with tuberous sclerosis. Whether treating these children prior to clinical onset of epileptic spasms has positive effect on behavioural outcome is not clear. We decided to test this hypothesis with prophylactic use of vigabatrin in infants with tuberous sclerosis and abnormal EEG before the onset of epileptic spasms. Methods: We selected four infants with tuberous sclerosis having abnormal EEG studies in a prospective manner over last 2 years. The upper limit of enrolment age was 3 months (1 to 3 months). Infants with epileptic spasms were excluded from the study. Case 1 had experienced 3 episodes of focal motor seizures while rest had no seizures prior to enrolment. Abnormalities on EEG were either in the form of focal or multifocal epileptiform discharges without background abnormalities. Vigabatrin was started with dose of 50 mg/kg/day and was titrated to 100 mg/kg/day over 2 weeks. The drug was maintained till 9 months of age and then tapered off as we wanted to cover the early onset window of epileptic spasm. Developmental assessment was done every three monthly. Results: Case 2 experienced epileptic spasms on vigabatrin. Case 1 continued to get focal seizures while case 3 and 4 never experienced seizures. EEG pattern was changed from multifocal epileptiform discharges to modified hypsarrhythmia in case 2 while EEG abnormalities remained more or less same in other infants. Case 2 developed severe autistic features and cognitive impairment. Case 4 who never experienced seizures, showed language delay and minimal reduction of social interaction after 9 months of age. Case 3 has so far normal neuro-developmental course. Conclusions: As sample size was too small and there was significant inter case variation no definite conclusion can be drawn from this study. However there seems to be no additional benefit of prophylactic vigabatrin in terms of final neurobehavioral outcome. Funding: Nil