Abstract
Focal cortical dysplasia (FCD) II and I are major causes for drug-resistant epilepsy. In order to gain insight into the possible correlations between FCD II and FCD I, different clinical characteristics and immunohistochemical expression characteristics in FCD I and II were analyzed. The median age of onset and duration of epilepsy in FCD I and FCD II patients were 2.1 years and 5.3 years vs 2.4 years and 4.5 years. Therefore, the median age of onset and duration of epilepsy were similar in the two groups. Pathological lesions were predominantly located in frontal lobe in FCD II and temporal in FCD I. Significantly more signal abnormalities in FLAIR and T2 images were demonstrated in FCD II than FCD I. The rate of satisfied seizure outcome was relative higher in FCDII patients (95.12%) than that in FCDI group (84.6%). Furthermore, we detected expressions of progenitor cell proteins and the mammalian target of rapamycin (mTOR) cascade activation protein in FCDs. Results showed that sex-determiningregion Y-box 2(SOX2), Kruppel-likefactor 4 (KLF4) and phospho-S6 ribosomal proteins (ser240/244 or ser235/236) were expressed in FCDII group but not in FCD I. Overall, this study unveils FCD I and II exhibit distinct clinical and immunohistochemical expression characteristics, revealing different pathogenic mechanisms.
Highlights
Focal cortical dysplasia (FCD) represent a common cause for pediatric epilepsy [1, 2]
FCD I is primarily characterized by focal disruption of normal intracortical lamination and columnar organization, while FCD II exhibits dramatic laminar disorganization and dysmorphic neurons (DNs) and/or additional Balloon cells (BCs) [3, 4, 5]
Compared to FCD I, FCD II was more likely to locate in frontal lobe and the difference was statistically significant (χ2=10.64, p < 0.01)
Summary
FCD I is primarily characterized by focal disruption of normal intracortical lamination and columnar organization, while FCD II exhibits dramatic laminar disorganization and dysmorphic neurons (DNs) and/or additional Balloon cells (BCs) [3, 4, 5]. Both FCD types are associated with intractable epilepsy while display distinct histopathological and clinical characteristics. Activation of mTOR pathway results in enhanced expression of transcriptional activator c-Myc [11, 12]. C-Myc is expression associated with transcriptional regulation of certain stem cell markers, www.impactjournals.com/oncotarget Activation of mTOR pathway results in enhanced expression of transcriptional activator c-Myc [11, 12]. c-Myc is expression associated with transcriptional regulation of certain stem cell markers, www.impactjournals.com/oncotarget
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