Seizure outcome of infantile spasms with focal cortical dysplasia

Purpose: This study sought to evaluate the seizure outcome of infantile spasms (IS) with focal cortical dysplasia (FCD). Methods: We retrospectively reviewed infantile spasms patients with FCD from 2004 to 2010. We investigated seizure outcome from antiepileptic drug (AED), ketogenic diet (KD), resective surgery, and analyzed the results according to individual imaging studies. Results: Among 404 patients of IS, FCD was confirmed in 51 patients. In retrospective review of brain MRI, only 21 patients (41.2%) were suspected of FCD before 1. year of age, but 45 patients (88.2%) became confirmed to FCD by MRI after the age of 1. year. Once the spasms were not controlled by 1 or 2 AEDs, the chance of becoming seizure free with additional third or more drugs was very low (2.3%). The seizure free rate was 33.3% (7/21) in patients treated with ketogenic diet, and 73.3% (22/30) in surgical patients, who were both intractable to AEDs. There were no significant differences in seizure free rate in both ketogenic diet and surgical patients, between MRI negative and positive patients prior to 1. year of age. Conclusions: KD and surgery should be considered in medically refractory IS with FCD.