• Journal of Korean Neurosurgical Society
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• 제30권11호
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• pp.1300-1307
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• 2001

Purposes : This study reports the possible causes of seizure recurrence in patients underwent previous epilepsy surgery, and surgical strategy for resection of the additional epileptogenic zone locating at the distant area to the site of first resection. Methods : A total of 10 patients with previous surgery due to intractable epilepsy were studied. Five of these underwent standard temporal lobectomy, four extratemporal resection, and one corticoamygdalectomy. Seizure outcome of these were class III-IV. Evaluation methods for reoperation included MRI, 3D-surface rendering of MRI, PET, prologned video-EEG recording with surface electrodes and subdural grid electrodes. Additional resection was done in the frontal lobe in two, in the temporal lobe in three, in the parietal lobe in two, and in the supplementary sensori-motor area in two. Tumor in the superior frontal gyrus in the left hemisphere was removed in one patient. Extent of resection was decided based on the results of ictal subdural grid EEGs and MRI findings. Awake anesthesia and electrocortical stimulation were performed in the two patients for defining the eloquent area. Results : Histopathologic findings revealed extratemporal cortical dysplasia in six, hippocampal sclerosis and cortical dysplasia of the temporal neocortex in one, neuronal gliosis in two, and meningioma in one. Previous pathology of the five patients with cortical dysplasia in the second operation was hippocampal sclerosis plus cortical dysplasia of the temporal neocortex. After reoperation, seizure outcomes were class I in six, class II in three, class III in one at the mean follow-up period of 17.5 months. Characteristically, patients in class II-III after reoperation showed histopathologic findings of hippocampal sclerosis plus temporal neocortical cortical dysplasia plus extratemporal cortical dysplasia. Conclusions : Seizure recurrence after epilepsy surgery was related with the presence of an additional epileptogenic zone distant to the site of first operation, and the majority of the histopathology of the surgical specimens was cortical dysplasia. In particular, hippocampal sclerosis plus temporal neocortical cortical dysplasia was highly related with seizure recurrence in patients with previous operation. In these patients, multimodal evaluation methods were necessary in defining the additional epileptogenic zone.

• Journal of Korean Neurosurgical Society
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• 제62권3호
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• pp.288-295
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• 2019

In 2011, the International League against Epilepsy (ILAE) proposed a first international consensus of the classification of focal cortical dysplasia (FCD). This FCD classification had been widely used in worldwide. In this review paper, the authors would like to give helpful comments for better understanding of the current FCD classification. Especially, the basic concepts of FCD type I, such as "radial", "tangential" and "microcolumn" will be discussed with figures. In addition, the limitations, genetic progress and prospect of FCD will be suggested.

• BMB Reports
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• 제49권2호
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• pp.71-72
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• 2016

Focal cortical dysplasia type II (FCDII) is a focal malformation of the developing cerebral cortex and the major cause of intractable epilepsy. However, since the molecular genetic etiology of FCD has remained enigmatic, the effective therapeutic target for this condition has remained poorly understood. Our recent study on FCD utilizing various deep sequencing platforms identified somatic mutations in MTOR (existing as low as 1% allelic frequency) only in the affected brain tissues. We observed that these mutations induced hyperactivation of the mTOR kinase. In addition, focal cortical expression of mutant MTOR using in utero electroporation in mice, recapitulated the neuropathological features of FCDII, such as migration defect, cytomegalic neuron and spontaneous seizures. Furthermore, seizures and dysmorphic neurons were rescued by the administration of mTOR inhibitor, rapamycin. This study provides the first evidence that brain somatic activating mutations in MTOR cause FCD, and suggests the potential drug target for intractable epilepsy in FCD patients.

• 치과방사선
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• 제29권1호
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• pp.55-63
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• 1999

The author observed and compared the radiographic features of 49 cases of the fibrous dysplasia and 14 cases of the ossifying fibroma in the osteoblastic or mature stage radiologically and histopathologically. The obtained results were as follows: 1. Fibrous dysplasia occurred most frequently in the 2nd decade, but ossifying fibroma in the 3rd and 4th decades, and both lesions occurred with slight predilection in females. 2. In most cases, chief complaints were painless facial swelling. And 61.1％ of fibrous dysplasia occurred in the maxilla, 92.9％ of ossifying fibroma in the mandible, and most of these lesions occurred in the premolar-molar region. 3. In the mandibular lesions, ossifying fibroma was shown more oval and round shape. but fibrous dysplasia was shown fusiform shape. 4. Fibrous dysplasia was shown homogeneously distributed. complete radiopaque shadow at 63％, and ossifying fibroma was shown concentric. mixed appearance of radiolucent and radiopaque shadow at 92.9％. 5. Fibrous dysplasia was entirely shown poorly outlined and blended to normal surrounding bone, but ossifying fibroma was shown well-defined border. 6. Cortical thinning and expansion were observed in these lesions. but degree of cortical expansion was more severe in ossifying fibroma than fibrous dysplasia. 7. Loss of lamina dura. tooth displacement. and displacement of mandibular canal were observed in both lesions. but root resorption was observed in ossifying fibroma only.

• 치과방사선
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• 제16권1호
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• pp.113-126
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• 1986

This study was undertaken to document and better define this condition to help clarify this clinical and radiographic appearances by the analysis of clinical and radiographic features of cementoma. A study was made of a series of 104 cases with cementoma. This investigation of cementoma revealed the following features: 1. The female occurred in 73% of periapical cemental dysplasia and benign cementoblastoma, and 80% of cementifying fibroma. 2. 40% of periapical cemental dysplasia occurred in the fifth decades, and 73% of benign cementoblastoma during the second and third decades, while there was no age predilection in the cementifying fibroma. 3. 63% of periapical cemental dysplasia occurred in the mandibular anterior region. 91% of benign cementoblastoma and 80% of cementifying fibroma occurred in the mandibular premolar and/or molar region. 4. There were no cases complaining the associated clinical signs and subjective symptoms in the periapical cemental dysplasia, however the patient complained the pain in 36% of benign cementoblastoma and 40% of cementifying fibroma. 5. There were no cases expanding the cortical plates in the periapical cemental dysplasia, however 73% of benign cementoblastoma and all of 5 cases of cementifying fibroma showed the expansion of cortical plates. 6. Several radiographic features of the periapical cemental dysplasia were shown: a. 29% of the cases had multiple lesions. b. 53% of the cases were in the mature stage. c. During the osteolytic stage, the alveolar lamina dura was lost in 89% of the cases.

• Annals of Clinical Neurophysiology
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• 제20권2호
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• pp.85-88
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• 2018

Septo-optic dysplasia is a congenital anomaly with diverse phenotypes from normal to mixtures of visual abnormality, endocrine dysfunction, psychomotor retardations and epileptic seizures. It is characterized by optic atrophy, pituitary dysfunction and midline structure abnormalities in corpus callosum or septum pellucidum. Diagnosis of septo-optic dysplasia plus is made when cortical malformations accompanied. Here we report a middle-aged woman with septo-optic dysplasia plus having unilateral optic atrophy, agenesis of septum pellucidum and cortical malformations.

• Journal of Korean Neurosurgical Society
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• 제52권5호
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• pp.466-471
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• 2012

Objective : The aim of this study was to devise an objective clustering method for magnetoencephalography (MEG) interictal spike sources, and to identify the prognostic value of the new clustering method in adult epilepsy patients with cortical dysplasia (CD). Methods : We retrospectively analyzed 25 adult patients with histologically proven CD, who underwent MEG examination and surgical resection for intractable epilepsy. The mean postoperative follow-up period was 3.1 years. A hierarchical clustering method was adopted for MEG interictal spike source clustering. Clustered sources were then tested for their prognostic value toward surgical outcome. Results : Postoperative seizure outcome was Engel class I in 6 (24%), class II in 3 (12%), class III in 12 (48%), and class IV in 4 (16%) patients. With respect to MEG spike clustering, 12 of 25 (48%) patients showed 1 cluster, 2 (8%) showed 2 or more clusters within the same lobe, 10 (40%) showed 2 or more clusters in a different lobe, and 1 (4%) patient had only scattered spikes with no clustering. Patients who showed focal clustering achieved better surgical outcome than distributed cases (p=0.017). Conclusion : This is the first study that introduces an objective method to classify the distribution of MEG interictal spike sources. By using a hierarchical clustering method, we found that the presence of focal clustered spikes predicts a better postoperative outcome in epilepsy patients with CD.

• Journal of Korean Neurosurgical Society
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• 제62권3호
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• pp.321-327
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• 2019

Focal cortical dysplasia (FCD) is the major cause of intractable focal epilepsy in childhood leading to epilepsy surgery. The overall seizure freedom after surgery ranges between 50-75% at 2 years after surgery and the long-term seizure freedom remain relatively stable. Seizure outcome after surgery depends on a various factors such as pathologic etiologies, extent of lesion, and types of surgery. Therefore, seizure outcome after surgery for FCD should be analyzed carefully considering cohorts' characteristics. Studies of pediatric epilepsy surgery emphasize the early surgical intervention for a better cognition. Early surgical intervention and cessation of seizure activity are important for children with intractable epilepsy. However, there are limited data on the cognitive outcome after surgery in pediatric FCD, requiring further investigation. This paper reviews the seizure and cognitive outcomes of epilepsy surgery for FCD in children. Several prognostic factors influencing seizure outcome after surgery will be discussed in detail.

• Investigative Magnetic Resonance Imaging
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• 제3권1호
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• pp.60-65
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• 1999

목적 : 뇌백질 신호억제를 위한 중간시간 반전회복(Medium Tau Inversion Recovery, MTIR)영상에서 뇌회질과 뇌배질의 대조도를 다른 기법의 MR영상과 비교해 보고 뇌피질에 이상이 있는 환자에서 MTIR영상의 유용성을 평가하고자 하였다. 대상 및 방법 : 2명의 정상 지원자와 뇌피질 이형성증을 포함한 뇌피질 질환이 있는 21명을 대상으로 뇌회질과 뇌백질의 신호의 차이를 관심영역에서 대조도 백분율과 대조도 잡음비로 츠정하여 MTIR영상과 여러 가지 다른 MR영상을 비교하였다. 또한 시각적으로 병변이 뚜렷함, 새로운 병변의 발견여부를 시각적으로 비교 평가하였다. 결과 : MTIR영상은 다른 MR영상에 비해 대조도 백분율, 대조도 잡음비가 높아 뇌회질과 뇌백질의 신호의 차이가 가장 뚜렷하였다. 신경이주이상을 포함한 21명의 뇌피질 환자에서는 MTIR영상에서 다른 영상보다 병변이 뚜려사고 병변의 묘사(delineation)을 증가 시켰으나 새로운 병변은 발견하지 못해다. 결론 : MTIR영상은 뇌회질과 뇌백질의 대조도를 증가시키는 영상 기법이며 뇌피질을 침범한 질환을 특히 뇌피질 이형성증의 병변을 매우 잘 나타냈다. 기존의 T1강조영상 또는 3D-MPRAG에서 뇌피질-백질의 구별이 어려운 경우에는 보완적으로 이용가치가 있는 영상기법으로 생각된다.

• Investigative Magnetic Resonance Imaging
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• 제7권1호
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• pp.47-55
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• 2003

목적 : 뇌피질 이형성증의 다양한 자기공명영상소견을 유형별로 분류하고 병리적 등급 및 유형과의 연관성을 분석한다. 대상 및 방법 : 수술 후 병리적으로 뇌피질 이형성증이 확진된 97명을 대상으로 하였다. 수술 전 MR 영상을 후향적으로 분석하였으며, 이상 소견의 유무에 따라 MR 양성군과 MR 음성군으로 나누었다. MR 양성군에서는 뇌회와 인접지주막하 공간의 크기, 뇌피질 두께, 피질하백질의 신호 강도, 뇌피질과 백질 경계부의 명확성에 의하여 MR 이상 소견을 분류하였다. 병리적 소견 역시 영상 소견을 모르는 상태에서 후향적으로 분석하였으며, 경도, 중등도, 고도와 비풍선세포형, 풍선세포형으로 나누었다. MR 양성군과 음성군 사이에서 그리고 MR 양성군의 네 가지 유형 내에서 각각 병리적 등급 및 유형의 차이가 있는지 분석하였다. 결과: MR 양성군과 음성군은 각각 39 (40%) 명, 58 (60%) 명이었다. MR 양성군 중 위축형은 13 (33%)명, 피질띠형은 9 (23%)명, 내측 만곡형은 9 (23%)명, 그리고 비특이적 형태는 8 (21%)명이었다. MR 양성군과 음성군 사이에서 병리적 등급은 의미 있는 차이가 없었으나, MR음성군에 비하여 MR 양성군에서 고도의 병리적 등급의 빈도가 높은 경향을 보였으며 또한 풍선 세포형의 빈도 역시 유의하게 높았다. (5% Vs 2l%, p＜0.05). 특히 MR 양성군 중에 내측 만곡형은 고도의 병리적 등급과 풍선세포형의 빈도가 각각 78% (7/9), 56% (5/9)인 반면에, 위축형은 경도의 등급과 비풍선세포형의 빈도가 각각 77% (10/13), 100%(13/13)이었다. 결론: 뇌피질이형성증 환자의 MR 영상에서 반 이하에서만 이상 소견을 발견할 수 있었고, 특히 내측 만곡형은 병리적으로 고도의 등급 및 풍선세포형, 그리고 위축형은 경도의 등급 및 비풍선세포형과 높은 연관성을 보여 주었다.