Abstracts

Aicardi[apos]s syndrome is manifested by retinal lacunae, severe developmental delays, infantile spasms and partial seizures in females. Bain imaging shows heterotopias, choroid plexus cysts, and microgyrias. The seizures are frequently refractory to medical therapy, and these girls are not candidates for resective seizure surgery given the multifocal nature of their seizures. A national parents support group for Aicardi[apos]s syndrome was canvassed by telephone for members whose children had received vagal nerve stimulation for control of seizures. Eight Aicardi[apos]s syndrome patients were found, and these 10 patients were asked information relevant to this form of therapy. Four of the eight patients had been exposed to the ketogenic diet, and stopped it due to lack of efficacy (2) or complications (2). No patient had undergone resective seizure surgery. Time of VNS therapy was 0.5 to 5 years. Four patients were responders ([underline][gt][/underline] 50% reduction) and one patient had more seizures. Three of the eight found the magnet current helpful in terminating a seizure. On a one to five scale, five being much better, the general wellbeing of the patient was rated [underline][gt][/underline] 4 in all save one patient on this therapy. Three patients had no change in concurrent antiepileptic drugs (AEDs), three eliminated one AED, and two increased the number of AEDs. The only reported complication was an infection. All eight patients are still being treated wuith VNS. VNS is well tolerated in this small population. The responder rate is probably the same as reported in the preapproval trials for children with other epilepsies (J Pediatr 1999;134:563-566). Most patients had improvement in wellbeing. When compared to the Cyberonics-maintained registry, the latter contains 5 paatients, but the responder rate is 4 of 5. VNS is a valid consideration in patients with Aicardi[apos]s syndrome and medically refractory epilepsy.