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Epileptogenic Properties of Balloon Cells in Cortical Tubers of Tuberous Sclerosis : Upregulation of Drug Resistance Proteins  

Kang, Nam-Gu (Department of Neurosurgery, Gwangju Christian Hospital)
Chang, Hong-Joen (Department of Neurosurgery, Gwangju Christian Hospital)
Ok, Young-Cheol (Department of Neurosurgery, Gwangju Christian Hospital)
Lee, Rae-Seop (Department of Neurosurgery, Gwangju Christian Hospital)
Park, Seung-Kyu (Department of Neurosurgery, Gwangju Christian Hospital)
Lim, Jun-Seob (Department of Neurosurgery, Gwangju Christian Hospital)
Cho, Kyu-Yong (Department of Neurosurgery, Gwangju Christian Hospital)
Kim, Hyung-Ihl (Department of Neurosurgery, Jeonju Presbyterian Hospital)
Kim, Jae-Hyoo (Departments of Neurosurgery, Chonnam National University Medical School)
Oh, Hyun-Sik (Departments of Pathology, Chonnam National University Medical School)
Lee, Min-Cheol (Departments of Pathology, Chonnam National University Medical School)
Publication Information
Journal of Korean Neurosurgical Society / v.41, no.6, 2007 , pp. 397-402 More about this Journal
Abstract
Objective : Balloon cells and dysplastic neurons are histopathological hallmarks of the cortical tubers of tuberous sclerosis complex [TSC] and focal cortical dysplasia [FCD] of the Taylor type. They are believed to be the epileptogenic substrate and cause therapeutic drug resistant epilepsy in man. P-glycoprotein [P-gp] is the product of multidrug resistance gene [MDR1], and it maintains intracellular drug concentration at a relatively low level. The authors investigated expression of P-gp in balloon cells and dysplastic neurons of cortical tubers in patients with TSC. Methods : An immunohistochemical study using the primary antibody for P-gp, as an indicative of drug resistance, was performed in the cortical tuber tissues in two patients of surgical resection for epilepsy and six autopsy cases. Results : Balloon cells of each lesion showed different intensity and number in P-gp immunopositivity. P-gp immunopositivity in balloon cells were 28.2%, and dysplastic neurons were 22.7%. These immunoreactivities were more prominent in balloon cells distributed in the subpial region than deeper region of the cortical tubers. Capillary endothelial cells within the cortical tubers also showed P-gp immunopositivity. Conclusion : In this study, the drug resistance protein P-glycoprotein in balloon cells and dysplastic neurons might explain medically refractory epilepsy in TSC.
Keywords
Balloon cells; Dysplastic neurons; Multidrug resistance; P-glycoprotein; Tuberous sclerosis complex (TSC);
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