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(Abst. 3.288), 2017

Vigabatrin for Focal Seizures in Tuberous Sclerosis
Authors: John M. Schreiber, Children's National Health System; Nancy Elling, Children's National Health System; Tesfaye Zelleke, Children's National Medical Center; William D. Gaillard, Children’s National Medical Center, George Washington University School of Medicine and Health Sciences, Washington, DC, United States.; and William McClintock, Children's National Health System
Content: Rationale: Vigabatrin (VGB) is well-established as the first-line therapy for infantile spasms (IS) in association with tuberous sclerosis (TS), but less is known about its role in focal seizures due to tuberous sclerosis. The risk for irreversible, cumulative dose-dependent visual field constriction due to phororeceptor toxicity limits its use in this context, where focal seizures often persist without definitive surgical intervention. We aimed to examine our institutional use of VGB for focal seizures in tuberous sclerosis and to assess outcomes. Methods: We queried the tuberous sclerosis database (n=112) to identify all children with tuberous sclerosis enrolled at Children's National Health System (CNHS) who were administered VGB for focal seizures. Data points collected include patient demographics, history of IS and treatment with VGB, duration of therapy, evidence of clinical improvement for focal seizures, reason for discontinuation, and epilepsy surgery. Results: We retrospectively identified 22 patients with TS who received VGB for focal seizures, starting VGB in June 1989 and continuing through the present time: Twelve (55%) were female with median age 8 months at VGB initiation (range 4 months - 9 years). Nineteen (86%) had a history of IS and all except the two oldest, born in 1986, received VGB for IS. Eleven of these individuals exhibited improvement in or resolution of IS. Sixteen out of 17 with IS remained on vigabatrin to treat focal seizures; one was non-compliant with VGB after resolution of spasms and was later restarted on VGB for intractable focal seizures. Eighteen out of the 22 in this cohort (82%) had improvement in focal seizures. Mean duration of VGB therapy was 5.7 years (range 0.25-17 years); seven continue taking VGB. Reasons for discontinuing include 9 in whom there was a concern for vision loss, 3 who underwent epilepsy surgery, 2 in whom VGB was ineffective, and one in whom alternative anti-seizure medication was subsequently effective. Families often preferred to remain on vigabatrin despite the risk to vision. Conclusions: VGB is used for focal seizures in TS and may be an effective therapy in patients who fail to respond adequately to other anti-seizure medications while awaiting definitive epilepsy surgery. The risk for vision loss due to photoreceptor toxicity continues to limit prolonged use. Funding: The Tuberous Sclerosis Alliance supports the patient database