Ketogenic Diet & Vagal Nerve Stimulation In Children With Tuberous Sclerosis Complex (tsc) With Drug-Refractory Epilepsy

Tuberous sclerosis complex (TSC) is associated with early onset, drug refractory epilepsy (DRE). Ketogenic diet (KD) and vagal nerve stimulation (VNS) are non-pharmacological therapies that are commonly used in these patients. There are only a small number of studies looking at the effectiveness of dietary therapies and VNS in TSC. The aim of this study was to determine the efficacy of dietary therapies and VNS in DRE associated with TSC.

A retrospective chart review was conducted which included children with TSC and DRE who were treated with dietary therapy or had VNS implanted at five pediatric academic centers from 2010 to 2020. Seizure outcomes and adverse effects were assessed.

Forty-two children were included, the median age at seizure onset was 0.4 months. The majority of children had daily seizures at initiation of dietary therapy and at VNS implantation; the most common seizure type was focal seizures. TSC associated neuropsychiatric disorder (TAND) features were identified in all children. Thirty children were treated with dietary therapies; 13/28 (46%) had >50% reduction in seizure frequency. The most common dietary adverse effects were constipation (47%) and vomiting (40%). Sixteen children were implanted with VNS; 8/16 (50%) had >50% reduction in seizures. VNS therapy was generally well tolerated.

In this cohort of children with TSC and DRE, nearly half of the children had >50% seizure reduction on dietary therapies and half had >50% seizure reduction after VNS implantation. VNS was better tolerated than dietary therapies.