Kang Joon Won, Rhie Seon Kyeong, Yu Rita, Eom Soyong, Hong William, Kim Shin Hye, Kang Hoon-Chul, Lee Joon Soo, Lee Young-Mock, Kim Heung Dong
Department of Pediatrics, Chungnam Naional University Hospital, Chungnam National University College of Medicine, Daejeon, Republic of Korea.
Brain Dev. 2013 Sep;35(8):816-20. doi: 10.1016/j.braindev.2013.06.013. Epub 2013 Jul 16.
This study sought to evaluate the seizure outcome of infantile spasms (IS) with focal cortical dysplasia (FCD).
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.
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 1year of age, but 45 patients (88.2%) became confirmed to FCD by MRI after the age of 1year. 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 1year of age.
KD and surgery should be considered in medically refractory IS with FCD.
本研究旨在评估伴局灶性皮质发育不良（FCD）的婴儿痉挛症（IS）的癫痫发作结局。
我们回顾性分析了2004年至2010年患有FCD的婴儿痉挛症患者。我们研究了抗癫痫药物（AED）、生酮饮食（KD）、切除性手术的癫痫发作结局，并根据个体影像学研究分析结果。
在404例IS患者中，51例确诊为FCD。回顾性分析脑部MRI，仅21例（41.2%）在1岁前被怀疑患有FCD，但45例（88.2%）在1岁后经MRI确诊为FCD。一旦痉挛不能被1或2种AED控制，使用额外的第三种或更多药物实现无癫痫发作的机会非常低（2.3%）。接受生酮饮食治疗的患者无癫痫发作率为33.3%（7/21），手术患者为73.3%（22/30），这两组患者对AED均难治。1岁前MRI阴性和阳性的生酮饮食及手术患者的无癫痫发作率无显著差异。
对于药物难治性伴FCD的IS，应考虑生酮饮食和手术治疗。
