• Journal of Korean Neurosurgical Society
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• 제41권6호
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• pp.397-402
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• 2007

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.

• Clinical and Experimental Pediatrics
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• 제56권7호
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• pp.275-281
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• 2013

Temporal lobe epilepsy (TLE) is the most common type of medically intractable epilepsy in adults and children, and mesial temporal sclerosis is the most common underlying cause of TLE. Unlike in the case of adults, TLE in infants and young children often has etiologies other than mesial temporal sclerosis, such as tumors, cortical dysplasia, trauma, and vascular malformations. Differences in seizure semiology have also been reported. Motor manifestations are prominent in infants and young children, but they become less obvious with increasing age. Further, automatisms tend to become increasingly complex with age. However, in childhood and especially in adolescence, the clinical manifestations are similar to those of the adult population. Selective amygdalohippocampectomy can lead to excellent postoperative seizure outcome in adults, but favorable results have been seen in children as well. Anterior temporal lobectomy may prove to be a more successful surgery than amygdalohippocampectomy in children with intractable TLE. The presence of a focal brain lesion on magnetic resonance imaging is one of the most reliable independent predictors of a good postoperative seizure outcome. Seizure-free status is the most important predictor of improved psychosocial outcome with advanced quality of life and a lower proportion of disability among adults and children. Since the brain is more plastic during infancy and early childhood, recovery is promoted. In contrast, long epilepsy duration is an important risk factor for surgically refractory seizures. Therefore, patients with medically intractable TLE should undergo surgery as early as possible.

• Journal of Korean Neurosurgical Society
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• 제62권3호
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• pp.353-360
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• 2019

Epilepsy surgery that eliminates the epileptogenic focus or disconnects the epileptic network has the potential to significantly improve seizure control in patients with medically intractable epilepsy. Magnetic resonance-guided laser interstitial thermal therapy (MRgLITT) has been an established option for epilepsy surgery since the US Food and Drug Administration cleared the use of MRgLITT in neurosurgery in 2007. MRgLITT is an ablative stereotactic procedure utilizing heat that is converted from laser energy, and the temperature of the tissue is monitored in real-time by MR thermography. Real-time quantitative thermal monitoring enables titration of laser energy for cellular injury, and it also estimates the extent of tissue damage. MRgLITT is applicable for lesion ablation in cases that the epileptogenic foci are localized and/or deep-seated such as in the mesial temporal lobe epilepsy and hypothalamic hamartoma. Seizure-free outcomes after MRgLITT are comparable to those of open surgery in well-selected patients such as those with mesial temporal sclerosis. Particularly in patients with hypothalamic hamartoma. In addition, MRgLITT can also be applied to ablate multiple discrete lesions of focal cortical dysplasia and tuberous sclerosis complex without the need for multiple craniotomies, as well as disconnection surgery such as corpus callosotomy. Careful planning of the target, the optimal trajectory of the laser probe, and the appropriate parameters for energy delivery are paramount to improve the seizure outcome and to reduce the complication caused by the thermal damage to the surrounding critical structures.

• 대한소아신경학회지
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• 제26권4호
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• pp.280-283
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• 2018

뇌전증 환자에서 자기공명영상은 기질적인 병변을 확인하기 위해 권장된다. 그러나 청소년기 결신 발작을 포함한 특발성 전신 발작에서 이상소견은 대개 자기공명영상과 같은 뇌 영상 기법을 사용하여 확인할 수 없다. 발작기 동안 자기공명영상 이상은 뇌전증 지속증에 의한 이차성으로 가장 흔히 보고되었으며, 부분발작과 전신 강직간대 발작환자에서 거의 나타나지 않는다. 결신 발작 동안 자기공명영상의 이상은 매우 드물다. 5세 소녀가 sodium valproate로 계속 치료를 했음에도 불구하고 3일간 발생한 결신 발작으로 내원하였다. 뇌파 검사에서 과다호흡 이후 3 Hz 극서파를 보였다. T2 강조 뇌 자기공명영상에서 좌측 외측구 주변의 비정상 피질 비후가 확인되었고, 피질 형성 이상이나 종양이 의심되었다. 환자는 lamotrigine으로 치료를 시작하였고 이후 발작은 없었다. 2개월 후 추적관찰로 뇌 자기공명영상을 시행하였고 이전의 비정상 부분은 확인되지 않았다. 저자들은 유년기 결신 발작 시 확인된 외측구 주변의 가역적인 뇌 자기공명영상 이상에 대해 보고하는 바이며 이것은 결신 발작 후 일과성 자기공명영상 이상 소견의 첫 번째 보고로 의미가 있다. 결신 발작과 부분 발작에서 자기공명영상 이상은 일시적인 이상 일 수 있으며 다른 질환과 감별 시 고려해야 할 것이다.