Hye Eun Kwon1, Soyong Eom1, Hoon-Chul Kang1, Joon Soo Lee1, Se Hoon Kim1, Dong Seok Kim1, Heung Dong Kim2. 1. From the Department of Pediatrics (H.E.K.), International St. Mary's Hospital, Catholic Kwandong University College of Medicine, Incheon; and the Division of Pediatric Neurosurgery (D.S.K.), the Division of Pediatric Neurology (H.-C.K., J.S.L., H.D.K.), Department of Pediatrics, Severance Children's Hospital, Epilepsy Research Institute (S.E.), and Department of Pathology (S.H.K.), Severance Hospital, Yonsei University College of Medicine, Seoul, Korea. 2. From the Department of Pediatrics (H.E.K.), International St. Mary's Hospital, Catholic Kwandong University College of Medicine, Incheon; and the Division of Pediatric Neurosurgery (D.S.K.), the Division of Pediatric Neurology (H.-C.K., J.S.L., H.D.K.), Department of Pediatrics, Severance Children's Hospital, Epilepsy Research Institute (S.E.), and Department of Pathology (S.H.K.), Severance Hospital, Yonsei University College of Medicine, Seoul, Korea. hdkimmd@yuhs.ac.
Abstract
OBJECTIVE: To analyze the clinical presentation and outcomes of surgically treated focal cortical dysplasia (FCD) in children. METHODS: We reviewed 75 cases of confirmed FCD by pathology after resective surgery. We used the pathologic classification proposed by the International League Against Epilepsy and included clinical profile and seizure and neurodevelopmental outcomes in analyses. RESULTS: There were 11 cases of FCD type I, 34 of type IIa, 19 of type IIb, and 11 of type III. Fifty-one cases (68.0%) presented clinically as focal epilepsy (FE) and 24 (32.0%) as epileptic encephalopathy (EE), including 16 with Lennox-Gastaut syndrome and 8 with West syndrome. We observed EE in 7 cases (63.6%) in FCD type I, 14 (41.2%) in type IIa, 2 (10.5%) in type IIb, and 1 (9.1%) in type III. We found the following more frequently in EE: seizure onset occurring at younger than 2 years (EE: 20 [83.3%], FE: 19 [37.3%]; p < 0.001), presence of intellectual disability before surgery (EE: 22 [91.7%], FE: 29 [56.9%]; p = 0.003), and multilobar resections (EE: 19 [79.2%], FE: 15 [29.4%]; p < 0.001). Forty-eight cases (64.0%) were seizure-free; 66.7% (34/51) in FE, 58.3% (14/24) in EE. Neurodevelopmental level showed more improvement (11/48 vs 0/27, p = 0.011) and less deterioration (2/48 vs 9/27, p = 0.001) in the seizure-free group compared to the non-seizure-free group. CONCLUSIONS: FCD can cause FE and EE in pediatric age, and resective surgery should be considered as a treatment option for both types of epilepsy.
OBJECTIVE: To analyze the clinical presentation and outcomes of surgically treated focal cortical dysplasia (FCD) in children. METHODS: We reviewed 75 cases of confirmed FCD by pathology after resective surgery. We used the pathologic classification proposed by the International League Against Epilepsy and included clinical profile and seizure and neurodevelopmental outcomes in analyses. RESULTS: There were 11 cases of FCD type I, 34 of type IIa, 19 of type IIb, and 11 of type III. Fifty-one cases (68.0%) presented clinically as focal epilepsy (FE) and 24 (32.0%) as epilepticencephalopathy (EE), including 16 with Lennox-Gastaut syndrome and 8 with West syndrome. We observed EE in 7 cases (63.6%) in FCD type I, 14 (41.2%) in type IIa, 2 (10.5%) in type IIb, and 1 (9.1%) in type III. We found the following more frequently in EE: seizure onset occurring at younger than 2 years (EE: 20 [83.3%], FE: 19 [37.3%]; p < 0.001), presence of intellectual disability before surgery (EE: 22 [91.7%], FE: 29 [56.9%]; p = 0.003), and multilobar resections (EE: 19 [79.2%], FE: 15 [29.4%]; p < 0.001). Forty-eight cases (64.0%) were seizure-free; 66.7% (34/51) in FE, 58.3% (14/24) in EE. Neurodevelopmental level showed more improvement (11/48 vs 0/27, p = 0.011) and less deterioration (2/48 vs 9/27, p = 0.001) in the seizure-free group compared to the non-seizure-free group. CONCLUSIONS:FCD can cause FE and EE in pediatric age, and resective surgery should be considered as a treatment option for both types of epilepsy.
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Authors: Till S Zimmer; Diede W M Broekaart; Mark Luinenburg; Caroline Mijnsbergen; Jasper J Anink; Nam Suk Sim; Iliana Michailidou; Floor E Jansen; Peter C van Rijen; Jeong Ho Lee; Liesbeth François; Jonathan van Eyll; Stefanie Dedeurwaerdere; Erwin A van Vliet; Angelika Mühlebner; James D Mills; Eleonora Aronica Journal: Neuropathol Appl Neurobiol Date: 2021-06-08 Impact factor: 8.090