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Seizure outcome of infantile spasms with focal cortical dysplasia
Authors:Joon Won Kang  Seon Kyeong Rhie  Rita Yu  Soyong Eom  William Hong  Shin Hye Kim  Hoon-Chul Kang  Joon Soo Lee  Young-Mock Lee  Heung Dong Kim
Affiliation:1. Department of Pediatrics, Chungnam Naional University Hospital, Chungnam National University College of Medicine, Daejeon, Republic of Korea;2. Department of Pediatrics, Cha Bundang Medical Center, CHA University, Bundang, Republic of Korea;3. Division of Pediatric Neurology, Pediatric Epilepsy Clinics, Severance Children’s Hospital, Department of Pediatrics, Epilepsy Research Institute, Brain Research Institute, Yonsei University College of Medicine, Seoul, Republic of Korea;4. Department of Pediatric Psychiatry, Severance Children’s Hospital, Yonsei University College of Medicine, Seoul, Republic of Korea;5. Rutgers University – New Jersey Medical School, Newark, NJ, USA;6. Department of Pediatrics, Myongji Hospital, Goyang, Republic of Korea;g Department of Pediatrics, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Republic of Korea
Abstract: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.
Keywords:Infantile spasms   Epilepsy surgery   Focal cortical dysplasia   Ketogenic diet
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