Abstracts

Rationale: Tuberous sclerosis complex (TSC) is a genetic disorder characterized by the widespread development of benign tumors in multiple organs. It is caused by mutations or deletions of either the TSC1 or TSC2 genes. While affecting multiple organs, the most common neurological manifestations of TSC are epilepsy, mental retardation, and autism. Epilepsy in particular is seen in up to 80-90% of patients and is often intractable, with a poor response to antiseizure medications. The rationale of this study is to define the natural history of TSC and frequency of status epilepticus in pediatric patients with TSC treated with levetiracetam. Status Epilepticus is defined as a seizure of greater than thirty minutes duration.Methods: In this retrospective evaluation, we acquired patient data from two sites specializing in the care of children with TSC: Vanderbilt Children’s Hospital (Nashville, Tennessee) and St. Louis Children’s Hospital (St. Louis, Missouri). A patient database containing fields for the neurological and developmental manifestations of TSC was acquired and analyzed using SPSS software. We enrolled 53 patients with TSC. The age range is from 11 months to 31 years. This group was then retrospectively analyzed for presence of status epilepticus and treatment with levetiracetam.Results: 17 (32%) of the patients were currently on levetiracetam , 11 (20%) failed levetiracetam secondary to lack of efficacy and behavior problems and 25 (48%) were never tried on levetiracetum. Suprisingly the incidence of status epilepticus was low in our patient population seen in only 8 patients (15%). 6 of these 8 patients had no further incidences of status epilepticus after the initiation of levetiracetum. The episodes of status epilepticus range from a single to four episodes. Mean follow up after initiation of levetiracetum was 17 months with a range of 9-28 months. The remaining 2 were never tried on levetiracetam. Conclusions: The use of levetiracetam as adjunctive antiepileptic therapy may reduce the incidence of status epilepticus in patients with tuberous sclerosis complex. This research was supported by Young Investigator Research Program (YIRP) of UCB Pharma.