• International Journal of Oral Biology
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• v.31 no.2
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• pp.33-43
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• 2006

Dysplasia-associated seizure disorders are markedly resistant to pharmacological intervention. Relatively little research has been conducted studying the effects of antiepileptic drugs(AEDs)on seizure activity in a rat model of dysplasia. We have used rats exposed to methylazoxymethanol acetate(MAM) in utero, an animal model featuring nodular heterotopia, to investigate the effects of AEDs in the dysplastic brain. Pilocarpine was used to induce acute seizure in MAM-exposed and age-matched vehicle-injected control animals. Field potential recordings were used to monitor amplitude and numbers of population spikes, and paired pulse inhibition in response to stimulation of commissural pathway. Two commonly used AEDs were tested: diazepam 5, 2.5 mg/kg; phenytoin 40, 60 mg/kg. Diazepam(DZP) and phenytoin(PHT) reduced the amplitude of population spike in control and MAM-exposed rats. However, the amplitude of population spike was nearly eliminated in control rats as compared to the MAM-exposed rats. Pharmaco-resistance was tested by measuring seizure latencies in awake rats after pilocarpine administration(320 mg/kg, i.p.) with and without pretreatment with AEDs. Pre-treatment with PHT 60 mg prolonged seizure latency in control rats, but not in MAM-exposed animals. The main findings of this study are that acute seizures initiated in MAM-exposed rats are relatively resistant to standard AEDs assessed in vivo. These data suggest that animal model with cortical dysplasia can be used to screen the effects of potential AEDs.

• Journal of Korean Neurosurgical Society
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• v.56 no.3
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• pp.248-253
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• 2014

Objective : Cortical dysplasia (CD) is one of the common causes of epilepsy surgery. However, surgical outcome still remains poor, especially with frontal lobe epilepsy (FLE), despite the advancement of neuroimaging techniques and expansion of surgical indications. The aim of this study was to focus on surgical strategies in terms of extent of resection to improve surgical outcome in the cases of FLE with CD. Methods : A total of 11 patients of FLE were selected among 67 patients who were proven pathologically as CD, out of a total of 726 epilepsy surgery series since 1992. This study categorized surgical groups into three according to the extent of resection : 1) focal corticectomy, 2) regional corticectomy, and 3) partial functional lobectomy, based on the preoperative evaluation, in particular, ictal scalp EEG onset and/or intracranial recordings, and the lesions in high-resolution MRI. Surgical outcome was assessed following Engel's classification system. Results : Focal corticectomy was performed in 5 patients and regional corticectomy in another set of 5 patients. Only 1 patient underwent partial functional lobectomy. Types I and II CD were detected with the same frequency (45.45% each) and postoperative outcome was fully satisfactory (91%). Conclusion : The strategy of epilepsy surgery is to focus on the different characteristics of each individual, considering the extent of real resection, which is based on the focal ictal onset consistent with neuroimaging, especially in the practical point of view of neurosurgery.

• Investigative Magnetic Resonance Imaging
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• v.11 no.1
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• pp.49-53
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• 2007

Fibrous dysplasia is a skeletal developmental anomaly of the bone-forming mesenchyme that manifests as a defect in osteoblastic differentiation and maturation. It is a nonhereditary disorder of unknown cause. In fibrous dysplasia, the medullary bone is replaced by fibrous tissue, which appears various imaging findings. It is usually an incidental finding, generally not requiring further investigation. However, fibrous dysplasia may be complicated by pathologic fracture, and rarely by malignant degeneration. We present the image findings of a 44-year-old man who had a chondroblastic osteosarcoma arising from polyostotic fibrous dysplasia in the femur. Evidence of cortical destruction on plain radiography and soft tissue mass in the lesion on MR images suggested a tumor of malignant transformation.

• Clinical and Experimental Pediatrics
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• v.53 no.1
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• pp.106-110
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• 2010

Hippocampal sclerosis (HS) is one of the most common features of intractable temporal lobe epilepsy. Generally it can be identified through brain magnetic resonance imaging (MRI) with high degree of sensitivity and specificity. Typical brain MRI findings of HS are hippocampal atrophy with hyperintense signal confined to the lesion. On the other hand cortical dysplasia exhibits blurring of the gray-white matter junction and abnormal white matter signal intensity. We present a case where preoperative brain MRI strongly suggested the presence of diffuse cortical dysplasia in the left temporal lobe but postoperative pathology revealed the temporal lesion to be unremarkable except for hippocampal sclerosis.

• Investigative Magnetic Resonance Imaging
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• v.14 no.2
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• pp.126-133
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• 2010

Purpose : The study was to evaluate the localization of the abnormal gyral and sulcal patterns obtained by means of brain surface rendering imaging. Materials and Methods : Nineteen patients with cortical dysplasia who underwent brain surface rendering MR imaging were included in this study. We acquired MP-RAGE sequence and created the 3-D surface rendering MR images by using $VoxelPlus^{(R)}$. Anatomical locations and configurations of abnormal gyri and sulci were reviewed. Results : Abnormal gyral and sulcal patterns were seen 18 in 19 patients. The configuration and orientation of affected gyri and sulci were clearly evaluated in the brain surface rendering images. In a lissencephaly, the a cortex was not delineated and showed markedly thick and smooth gyral pattern. In a schizencephaly, there were wheel shaped broad gyral pattern around the cleft. In a hemimegalencephaly, an affected hemisphere were enlarged and displayed thick and wide gyral pattern. In CBPS, the insular cortex was exposed and the gyri of the lesion were thickened. In focal cortical dysplasia, there were irregular serrated or thick and enlarged gyri. Conclusion : Brain surface rendering MR imaging is useful for the evaluation of a detailed gyral pattern and accurate involvement site of abnormal gyri.

• Journal of Korean Academy of Oral and Maxillofacial Radiology
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• v.28 no.1
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• pp.27-36
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• 1998

The purpose of this study was to obtain information on the clinical and radiographic features of the fibro-osseous lesions in the jaws. For this study, the author examined and analysed the clinical records and radiographs of 71 cases of 68 patients in fibrous dysplasia, 35 cases of ossifying fibroma and 30 cases of 16 patients of periapical cemental dysplasia diagnosed by clinical and radiographic or histopathological examinations. The obtained results were as followings: L Fibrous dysplasia occurred most frequently in the 2nd decade (30.0％), ossifying fibroma in the 3rd-4th decades, periapical cemental dysplasia in the 4th decade, and all of three lesions showed slight predilection in females. In most cases, chief complaints were painless facial swelling in fibrous dysplasia and ossifying fibroma, and periapical cemental dysplasia was found accidentally in radiographs. 2. Fibrous dysplasia was occurred more frequently in maxilla, ossifying fibroma in mandible and both lesions in premolar-molar area. Periapical cemental dysplasia was occurred most frequently in the mandibular anterior area. The size of fibrous dysplasia was larger than that of ossifying fibroma, and the shape of ossifying fibroma was more round and elliptical than fibrous dysplasia whose was fusiform. 3. Fibrous dysplasia was shown homogeneous radiopaque shadow of 57.6％ and ossifying fibroma & periapical cemental dysplasia were shown mixed appearance of radiolucency and radiopacity shadows at 74.2％, 60.0％, respectively. 4. Fibrous dysplasia was entirely shown poorly defined at 87.7％, but ossifying fibroma & periapical cemental dysplasia were shown well outlined at 60.0％, 70.0％, respectively. 5. Cortical thinning and expansion were observed in fibrous dysplasia and ossifying fibroma, and severe in ossifying fibroma than fibrous dysplasia, and those signs were not seen in periapical cemental dysplasia. Loss of lamina dura was dominant in fibrous dysplasia and root resorption was dominant in ossifying fibroma. Displacement of mandibular canal and the degree of the increase of vertical dimension were alike in both lesions. Displacement of maxillary sinus or nasal cavity, thinning & expansion of the maxillary sinus were dominant in fibrous dysplasia. 6. Polyostotic fibrous dysplasia was occurred at 5.9％, Multiple periapical cemental dysplasia at 43.7％. Occurrence rate in the edentulous area of fibrous dysplasia and ossifying fibroma were 7.0％, 8.6％, respectively.

• 대한핵의학회:학술대회논문집
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• 1997.05a
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• pp.177-177
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• 1997

• Journal of Korean Neurosurgical Society
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• v.29 no.7
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• pp.861-867
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• 2000

Purpose : Neuronal migration disorder(NMD) is a major underlying pathology of patients with intractable epilepsy. The role of NMD on seizure susceptibility or epileptogenecity, however, has not been documented. Methods : External irradiation of total amount of 250 cGY was performed to the fetal rats on days 16(E16) and 17(E17) of gestation. After delivery, the rats of 230-260g were decapitated for the histopathologic study. Epileptog-enecity of the NMD was studied by observing electroclinical events after intraperitoneal kainic acid(KA) injection in the control rats and NMD rats. Results : Histopathologic findings revealed focal and/or diffuse cortical dysplasia consisting of dyslamination of the cerebral cortex and appearance of the cytomegalic neurons, neuronal heterotopia in the periventricular white matter, dispersion of the pyramidal layer and the dentate gyrus of the hippocampus, and agenesis of the corpus callosum. Abnormal expression of neurofilaments protein(NF-M/H) was characteristically observed in the dysplastic neurons of the neocortex and hippocampus. Early ictal onset and prolonged ictal activity on EEG and clinical seizures were observed from the NMD rats unlike with the control rats. Conclusions : Exteranl irradiation on the fetal rats produced NMD. And the rats with NMD were highly susceptible to kainic acid provoked seizures. This animal model would be useful to study the pathophysiology of clinically relevant NMDs.

• The Korean Journal of Physiology and Pharmacology
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• v.10 no.1
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• pp.13-17
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• 2006

Experimentally induced cortical disorganization exhibits many anatomical features which are characteristic of cortical malformations in children with early-onset epilepsy. We used an immunocytochemical technique and extracellular field potential recordings from the dorsal hippocampus to determine whether the excitability of the CA1 pyramidal cells was enhanced in rats with exnerimentallv induced hippocampal dysplasia. Compared with control rats, the MAM-treated rats displayed a decrease of paired pulse inhibition. When $GABA_A$ receptor antagonists were blocked with $10{\mu}M$ bicuculline the amplitude of the second population spike of the MAM-treated of rats was similar to that of the first population spike, as was in the control rats. The MAM-treated rats had fewer somatostatin and parvalbumin-immunoreactive neurons than the control rats. These results suggest that the enhanced neuronal responsiveness of the in vivo recording of the CA1 in this animal model may involve a reduction of CA1 inhibition.

• The Journal of Advanced Prosthodontics
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• v.3 no.2
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• pp.96-100
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• 2011

An 18-year-old male presented severe hypodontia due to hypohidrotic ectodermal dysplasia was treated with Le Fort I maxillary osteotomy with simultaneous sinus floor augmentation using the mixture of cortical autogenous bone graft harvested from iliac crest and organic Bio-Oss to position the maxilla in a right occlusal plane with respect to the mandible, and to construct adequate bone volume at posterior maxilla allowing proper implant placement. Due to the poor bone quality at other sites, ridge augmentation with onlay graft was done to construct adequate bone volume allowing proper implant placement, using tissue harvested from the iliac bone. Seven implants were placed in the maxilla and 7 implants were inserted in the mandible and screw-retained metal ceramic FPDs were fabricated. The two year follow up data showed that dental implants should be considered as a good treatment modality for patients with ectodermal dysplasia.