Abstracts

Rationale: Epileptic spasms (ES, also known as infantile spasms) is a potentially devastating form of childhood epilepsy, with limited treatment options beyond hormonal therapies (i.e. prednisolone and ACTH) and vigabatrin. Using a large cohort of children with epileptic spasms, we set out to describe our center’s experience with vagus nerve stimulation (VNS) for treatment of highly refractory ES.  Methods: Subjects were retrospectively identified using a clinical database that includes all children with ES and all patients who have undergone VNS treatment. We then identified patients with ongoing spasms at the time of VNS placement. Among these patients, we tabulated the burden of spasms and hypsarrhythmia before and after VNS placement, and catalogued all prior and concomitant medications, as well as VNS settings, at each follow-up visit. Responders were defined as patients with video-electroencephalography (VEEG) confirmed freedom from ES and hypsarrhythmia.  Results: We identified 24 children with history of intractable ES who underwent VNS. VNS implantation occurred at a median age of 5.6 years, after a long duration of ES (median 53.5 months) and numerous treatment failures (median 8). Although parents or practitioners reported substantial reductions in spasms burden and/or hypsarrhythmia in 13 (54%) cases, there was only 1 patient with prompt, VEEG-proven resolution of hypsarrhythmia and spasms. In this patient, for whom etiology is unknown, ES began at 3 months and initially responded to ACTH. However, relapse occurred at age 18 months. Leading up to VNS placement at age 4 years, the patient had failed 11 total treatments, including vigabatrin, and was maintained on valproic acid, clobazam, pyridoxine, and the ketogenic diet. VEEG prior to VNS implantation demonstrated both hypsarrhythmia and ES and parents reported at least daily clusters of spasms. Spasms resolved immediately following VNS implantation, including before initial programming of the device. Follow-up VEEG demonstrated resolution of both hypsarrhythmia and ES. The patient remained seizure-free until age 7 years, at which time there was return of ES. At most recent follow at age 8 years, with VEEG, there are continued ES as well as intermittent hypsarrhythmia. Conclusions: This study suggests that VNS may be a viable treatment for ES. Further prospective study is warranted, ideally in a larger, younger, and less refractory population.  Funding: None