Abstracts

RATIONALE: Patients with band heterotopias have multifocal and generalized seizures refractory to currently available medications. Inadequate control of seizures impacts negatively on quality of life, and drop attacks predispose patients to injury. Vagus nerve stimulation (VNS)is a treatment option with minmal surgical risk compared to corpus callosotomy and has demonstrated efficacy in patients with refractory epilepsy. Limited case series of patients with Lennox Gastaut syndrome and refractory primary generalized epilepsy suggest that VNS may be of great benefit in the treatment of these syndromes. METHODS: 2 patients with the double cortex syndrome were identified during the routine evaluation of refractory seizures on the basis of MRI studies revealing extensive band heterotopias. Both patients were offered VNS after the EEG revealed multifocal spikes and generalized seizures. They had previously been treated with a number of appropriate anitconvulsant medications in maximally tolerated doses. Patient 1 was evaluated and implanted prior to her move to Kentucky and patient 2 received her evaluation and implant at UK. Stimulation parameters were set according to protocol, and titrated as tolerated. Seizure frequency was monitored by caregiver report and seizure diaries. Medical manipulation was minimized. RESULTS: Prior to implantation, patient 1 suffered an average of 6 drop attacks, 4 complex partial and at least 1 generalized seizure each day. 2 years post implant, she has 7-9 drop and 4-5 staring seizures each month with no generalized seizures since one month post implant. Patient 2 had 4-5 drop and 4-5 staring seizures each day prior to implantation. Two months postoperatively, she had staring seizures once a day and only one drop attack. 6 months post-op, she has 2 stares per day and had two clusters of drop attacks in 3 months. CONCLUSIONS: Two patients with the double cortex syndrome (band heterotopias) had a prompt and marked reduction in seizure frequency and severity with VNS, with a particularly marked reduction in drop attacks.