The optimal treatment for medically
refractory epilepsy in
Aicardi syndrome (AS) is still unclear. Palliative surgical treatment, including
vagus nerve stimulation and corpus callosotomy, has therefore been used. There is limited data on the role of resective
epilepsy surgery as a treatment choice in patients with AS. Here, we describe the
seizures, anatomo-pathological findings, and neurodevelopmental outcome of palliative
epilepsy surgery in two children with AS who had resective
epilepsy surgery at the Cleveland Clinic. The related literature is also reviewed. Case 1 had a left
functional hemispherectomy and was free of
seizures and
hypsarrhythmia for six months after surgery. Her gross motor skills improved after surgery. Outcome at 43 months was 1-3 isolated
spasms per day. Case 2 had a right fronto-parietal lobectomy. Her
seizures improved in frequency and severity, but remained daily after
epilepsy surgery. Neurodevelopment changes included improved alertness and recognition of caregivers. This patient died 21 months after
epilepsy surgery of unclear causes. Surgical pathology in both cases showed
focal cortical dysplasia associated with other findings, such as nodular heterotopia and
polymicrogyria.
Epilepsy surgery could be an alternative
palliative treatment choice in selective cases of AS, but studies on a larger patient cohort are needed to identify the possible role of surgery in children with AS. The complexity of the pathological findings may offer an explanation for the severity of
seizures in AS.