Seizure freedom with vagus nerve stimulation in neurofibromatosis type 1: A case report

Abstract

Epileptic seizures in neurofibromatosis type 1 (NF1) have been a subject of investigation of numerous studies, however, their etiology has not yet been elucidated. They are usually well controlled with antiseizure medications (ASMs), but in some pharmacoresistant patients, vagus nerve stimulation (VNS) might present a complementary treatment modality. We present a 24-year-old male patient with NF1 who had temporal lobe seizures -focal autonomic seizures with impaired awareness and oroalimentary automatisms, as well as focal motor seizures with retained awareness, in addition to moderate intellectual disability. The most significant magnetic resonance (MRI) abnormalities included infiltrative changes of medulla oblongata, pons and cerebellum, as well as signal intensity changes with mild compression in the apex of the temporal lobes, insular cortex, putamen and medial part of the frontal lobe, all more prominent on the right, along with right mesial temporal sclerosis. Interictal electroencephalogram (EEG) showed two independent epileptic foci -one in the right frontocentrotemporal region and the other in the left centrotemporal region. Throughout the years, he had been treated with several ASMs in monotherapy or polytherapy without success in seizure control. After an extensive preoperative evaluation, VNS implantation was performed and the patient has been seizure free for almost two years. Although a palliative intervention, VNS could be a powerful tool in the treatment of these patients and even lead to seizure freedom. To the best of our knowledge, this is the first case report where seizure freedom was achieved in a patient with NF1 following VNS implantation.