Vagus Nerve Stimulator in a Patient with Adenylosuccinate Lyase Deficiency: a Case Report

Abstract

Adenylosuccinate lyase deficiency (ADSL) is a rare inborn error of metabolism in which epilepsy is a common feature, and often seizures are intractable. We present the first documented case of a female patient with intractable epilepsy secondary to ADSL who had exacerbation of her seizures following implantation of a vagus nerve stimulator (VNS). In addition, using PubMed search engine, we reviewed the current literature on treatment of epilepsy associated with ADSL as well as VNS therapy in patients with epilepsy associated with inborn errors of metabolism. VNS has shown to be a safe and efficacious treatment option for patients with intractable epilepsies of various causes; however, there are no published cases of VNS in this particular syndrome. There are limited data on the efficacy of VNS in patients with epilepsy associated with inborn errors of metabolism, and further study is needed.