• Journal of Korean Neurosurgical Society
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• v.30 no.11
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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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• v.62 no.3
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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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• v.49 no.2
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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.

• Journal of Korean Academy of Oral and Maxillofacial Radiology
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• v.29 no.1
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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.

• Journal of Korean Academy of Oral and Maxillofacial Radiology
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• v.16 no.1
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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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• v.20 no.2
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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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• v.52 no.5
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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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• v.62 no.3
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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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• v.3 no.1
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• pp.60-65
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• 1999

Purpose : The purpose of this study was to evaluate the image quality, contrast characteristics, and possible clinical utility of Medium Tau Inversion Recovery(MTIR) sequence with white matter suppression in patients with brain cortical lesion. Materials and methods : Two normal volunteers and twenty-one patients with cortical lesion were scanned with MTIR as well as other MR imaging sequences. Gray-white matter contrast was evaluated objectively using region-of-interest calculations, including percent contrast and contrast-to-noise ratio(CNR). MTIR sequence was visually compared with other sequences in 21 patients with cortical lesion including conspicuity and detection rate. Results : MTIR sequence had the highest present contrast and CNR between the gray matter and white matter. In twenty-one cases of cortical lesion including cortical dysplasia, MTIR sequence improved delineation and conspicuity of lesion, but MTIR sequence could not detect new lesions. Conclusion : The MTIR sequence well delineated the cortical lesions, particularly in including cortical dysplasia. It may be used as an adjunctive imaging sequence in case of poor gray and white matter differentiation with conventional T1-weighted sequences.

• Investigative Magnetic Resonance Imaging
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• v.7 no.1
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• pp.47-55
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• 2003

Purpose : Cortical dysplasia is known to be of variety of MR imaging findings. We attempted to classify MR imaging findings of cortical dysplasia into several types and to correlate those with histopathologic grades and subtypes. Materials and Methods : Preoperative MR images of 97 patients with pathologically-proven cortical dysplasia were retrospectively reviewed with knowledge of the diagnosis and operative sites. The patients were divided into MR-positive and MR-negative groups based on the presence or absence of MR imaging abnormalities. In MR-positive group, MR imaging features were arbitrarily classified into four types (atrophic, cortical-band, inward-rounding, and nonspecific types) on the basis of size of the gyrus and adjacent CSF space, cortical thickness, signal intensity of the subcortical white matter, and blurring of the gray-white matter junction. The pathologic findings were also retrospectively reviewed without knowledge of MR imaging findings and divided into three grades (mild, moderate, and severe) and two subtypes (nonballoon-cell and balloon-cell). Pathologic grades and subtypes we re compared between MR-positive and MR-negative groups. Four MR types of the MR-positive group were correlated with the pathologic grades and subtypes. Results : MR-positive and MR-negative groups consisted of 39 (40%) and 58 (60%) patients, respectively. Of the MR-positive group, atrophic type was seen in 13 patients (33 %), cortical-band type in 9 (23%), inward-rounding type in 9 (23%), and nonspecific type in 8 (21%). There was no significant difference in the pathologic grades between MR-positive and MR-negative groups, although MR-positive group tended to have higher pathologic grades than MR-negative group did. Balloon-cell subtype was found significantly higher in MR-positive group than in MR-negative group (p<0 .05): 21% (8/39) versus 5% (3/58). The inward-rounding type corresponded to the pathologically severe grade and balloon-cell subtype in 78% (7/9) and 56% (5/9) of the patients, respectively, while the atrophic type to the mild grade and nonballoon-cell subtype in 77% (10/13) and 100% (13/13), respectively. Conclusion : A variety of MR imaging abnormalities were found in 40% of the patients with cortical dysplasia and those were classified into four types (atrophic, cortical-band, inward-rounding, and nonspecific types), of which the inward-rounding type correlated well with the pathologically severe grade and balloon-cell subtype, whereas the atrophic type with the mild grade and nonballoon-cell subtype.