새로운 국소 피질이형성 분류의 장단점

새로운 국소 피질이형성 분류의 장단점

Significance and defect of new focal cortical dysplasia (FCD) classification

(구연):
Release Date : 2014. 10. 25(토)
Hye Eun Kwon1, Hoon-Chul Kang2, Joon Soo Lee2 , Heung Dong Kim2
Yonsei Univeresity Gangnam Severance Hospital Department of Pediatrics1
Yonsei Univeresity Severance Children's Hospital Division of Pediatric Neurology2
권혜은1, 강훈철2, 이준수2 , 김흥동2
연세의대 강남세브란스병원 소아청소년과1
연세의대 세브란스 어린이병원 소아신경과2

Abstract

New International League Against Epilepsy (ILAE) classification of focal cortical dysplasia (FCD) has introduced in 2011. Important differences from classification by Palmini et al. were mild forms of cortical malformations (mMCDs) was excluded in new classification in terms of no mature agreement and new subgroup FCD type III was introduced. This study aimed to focus on the significance and defect of new FCD classification. Method: One hundred six intractable pediatric epilepsy patients who underwent resective surgery and confirmed with cortical malformation by pathological findings. Results: There were 31 subjects with mMCDs by Palmini et al. and 11 had FCD type I, 34 had FCD type IIa, 19 had FCD type IIb, and 11 had FCD type III by new FCD classification. In other words, 31 patients were not classified to any subtype of new classification, but these patients showed comparable degree of epileptogenicity. When we compare the clinical feature of mMCDs with FCD subtypes, the mMCDs showed similar clinical features to FCD type I than FCD type IIa/type IIb. This study showed different degrees of severity of migration patterns in FCD subtypes. Although, patients in FCD subgroups did not differ in age at seizure onset, seizure frequency, or number of antiepileptic drug (AED) trial before surgery. FCD type I, which is commonly regarded as a mild form of FCD, had a comparable degree of epileptogenicity to other subtypes. FCD type IIb showed a notably higher rate of Engel I outcome compared to other pathological groups [FCD type IIb; 16 cases (84%), other pathologies; 32 cases (57%), P=0.034]. Moreover, severe epileptic burden presenting with a high rate of epileptic encephalopathy could be caused by even mild dysplastic pathology, including FCD type I. Among the pathological subtypes, clinical features of FCD type IIb are distinctive and fairly consistent, and they have highest rate of focal epilepsy, excellent surgical outcomes, success of AED discontinuation after surgery, and positive MRI findings. Conclusion: Although mMCDs was excluded in new FCD classification, this study showed that important pathological finding related to comparable degree of epileptogenicity and Engel class I outcome. Thus, we propose that mMCDs should be included in the new classification.

Keywords: mild forms of cortical malformations , new focal cortical dysplasia classification, pediatric epilepsy surgery