• Journal of Korean Neurosurgical Society
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• 제56권4호
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• pp.356-360
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• 2014

The coexistence of moyamoya disease (MMD) with an arteriovenous malformation (AVM) is exceedingly rare. We report two cases of AVM associated with MMD. The first case was an incidental AVM diagnosed simultaneously with MMD. This AVM was managed expectantly after encephalo-duro-arterio-synangiosis (EDAS) as the main feeders stemmed from the internal carotid artery, which we believed would be obliterated with the progression of MMD. However, the AVM persisted with replacement of the internal carotid artery feeders by new external carotid artery feeders from the EDAS site. The AVM was eventually treated with gamma knife radiosurgery considering an increasing steal effect. The second case was a de novo AVM case. The patient was initially diagnosed with MMD, and acquired an AVM eight years later that was slowly fed by the reconstituted anterior cerebral artery. Because the patient remained asymptomatic, the AVM is currently being closely followed for more than 2 years without further surgical intervention. Possible differences in the pathogenesis and the radiologic presentation of these AVMs are discussed with a literature review. No solid consensus exists on the optimal treatment of MMD-associated AVMs. Gamma knife radiosurgery appears to be an effective treatment option for an incidental AVM. However, a de novo AVM may be managed expectantly considering the possible risks of damaging established collaterals, low flow characteristics, and probably low risks of rupture.

• Childhood Kidney Diseases
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• 제17권2호
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• pp.143-148
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• 2013

신경섬유종은 드문 전신 질환으로 여러 장기를 침범하며 특히 다양한 크기의 혈관을 침범하여 혈관병을 발생시켜 대동맥 협착, 모야모야병, 신동맥 협착 등을 일으킨다. 이로 인하여 약 0.4-6.4%의 환자에서 고혈압 증상이 있으며 이의 원인으로 신혈관 협착이 가장 흔하다. 특히 약물에도 조절되지 않는 고혈압은 혈관 협착 등이 원인이 될 수 있기 때문에 도플러 초음파 검사나 전산화 혈관 조영술 등의 정밀 검사가 필요하다. 이에 대한 치료는 약물 치료, 피부경유 혈관경유혈관성형술, 수술적 치료 등의 병합 요법으로 이루어지며 재협착 발생률이 높기 때문에 주의 깊은 추적 관찰이 필요하다. 본 저자들은 제 1형 신경섬유종증에 합병된 모야모야병에서 신장동맥 협착을 동반한 고혈압을 진단받고 치료한 1례를 경험하였기에 보고하는 바이다.

• Journal of Korean Neurosurgical Society
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• 제38권6호
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• pp.413-418
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• 2005

Objective : Although an encephaloduroarteriosynangiosis procedure using the superficial temporal artery [STA-EDAS] is an effective indirect bypass method in children with moyamoya disease[MMD], there is still a need for an additional bypass operation that can cover the area of the posterior circulation. The goal of this study is to evaluate the efficacy of the EDAS procedure using the occipital arteries [OA-EDAS]. Methods : From August 2003 to April 2004, We performed OA-EDAS in sixteen patients with MMD who have a circulatory insufficiency in the territory of the posterior cerebral artery[PCA]. The medical records were reviewed retrospectively. The surgical outcomes, including the changes in neurological status and imaging studies, with the degree of neovascularization on the cerebral angiogram, and the hemodynamic changes on single-photon emission computed tomography[SPECT], were analyzed. Results : These 16 children consisted of 5 boys and 11 girls aged 2 to 9 years. The clinical outcome of their PCA symptoms, such as visual transient ischemic attacks[TIAs] or visual field defect, was favorable in 14 patients of 16. Nine patients of 11 who underwent follow up magnetic resonance imaging[MRI] showed favorable MRI changes. On angiogram most of the patients exhibited good or fair revascularization of the PCA territory [7 of 8]. The hemodynamic changes on SPECT in the PCA territory after surgery showed improved vascular reserve in 13 of the 16 territories. Conclusion : OA-EDAS is a safe and efficacious revascularization procedure in patients with MMD who have compromised cerebral perfusion in PCA territory, or with visual TIAs.

• Journal of Korean Neurosurgical Society
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• 제66권6호
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• pp.642-651
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• 2023

Objective : Endothelial colony-forming cells (ECFCs) have been reported to play an important role in the pathogenesis of moyamoya disease (MMD). We have previously observed stagnant growth in MMD ECFCs with functional impairment of tubule formation. We aimed to verify the key regulators and related signaling pathways involved in the functional defects of MMD ECFCs. Methods : ECFCs were cultured from peripheral blood mononuclear cells of healthy volunteers (normal) and MMD patients. Low-density lipoproteins uptake, flow cytometry, high content screening, senescence-associated β-galactosidase, immunofluorescence, cell cycle, tubule formation, microarray, real-time quantitative polymerase chain reaction, small interfering RNA transfection, and western blot analyses were performed. Results : The acquisition of cells that can be cultured for a long time with the characteristics of late ECFCs was significantly lower in the MMD patients than the normal. Importantly, the MMD ECFCs showed decreased cellular proliferation with G1 cell cycle arrest and cellular senescence compared to the normal ECFCs. A pathway enrichment analysis demonstrated that the cell cycle pathway was the major enriched pathway, which is consistent with the results of the functional analysis of ECFCs. Among the genes associated with the cell cycle, cyclin-dependent kinase inhibitor 2A (CDKN2A) showed the highest expression in MMD ECFCs. Knockdown of CDKN2A in MMD ECFCs enhanced proliferation by reducing G1 cell cycle arrest and inhibiting senescence through the regulation of CDK4 and phospho retinoblastoma protein. Conclusion : Our study suggests that CDKN2A plays an important role in the growth retardation of MMD ECFCs by inducing cell cycle arrest and senescence.

• Korean Journal of Radiology
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• 제24권8호
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• pp.784-794
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• 2023

Objective: To determine whether dynamic susceptibility contrast-enhanced (DSC) perfusion magnetic resonance imaging (MRI) can be used to evaluate posterior cerebral circulation in pediatric patients with moyamoya disease (MMD) who underwent anterior revascularization. Materials and Methods: This study retrospectively included 73 patients with MMD who underwent DSC perfusion MRI (age, 12.2 ± 6.1 years) between January 2016 and December 2020, owing to recent-onset clinical symptoms during the follow-up period after completion of anterior revascularization. DSC perfusion images were analyzed using a dedicated software package (NordicICE; Nordic NeuroLab) for the middle cerebral artery (MCA), posterior cerebral artery (PCA), and posterior border zone between the two regions (PCA-MCA). Patients were divided into two groups; the PCA stenosis group included 30 patients with newly confirmed PCA involvement, while the no PCA stenosis group included 43 patients without PCA involvement. The relationship between DSC perfusion parameters and PCA stenosis, as well as the performance of the parameters in discriminating between groups, were analyzed. Results: In the PCA stenosis group, the mean follow-up duration was 5.3 years after anterior revascularization, and visual disturbances were a common symptom. Normalized cerebral blood volume was increased, and both the normalized time-topeak (nTTP) and mean transit time values were significantly delayed in the PCA stenosis group compared with those in the no PCA stenosis group in the PCA and PCA-MCA border zones. TTP_PCA (odds ratio [OR] = 6.745; 95% confidence interval [CI] = 2.665-17.074; P < 0.001) and CBV_PCA-MCA (OR = 1.567; 95% CI = 1.021-2.406; P = 0.040) were independently associated with PCA stenosis. TTP_PCA showed the highest receiver operating characteristic curve area in discriminating for PCA stenosis (0.895; 95% CI = 0.803-0.986). Conclusion: nTTP can be used to effectively diagnose PCA stenosis. Therefore, DSC perfusion MRI may be a valuable tool for monitoring PCA stenosis in patients with MMD.

• Korean Journal of Radiology
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• 제20권6호
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• pp.985-996
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• 2019

Objective: To determine the correlation between cerebral blood flow (CBF) on arterial spin labeling (ASL) MRI and the degree of postoperative revascularization assessed on digital subtraction angiography in children with moyamoya disease (MMD). Materials and Methods: Twenty-one children (9 boys and 12 girls; mean age, 8.4 ± 3.6 years; age range, 3-16 years) with MMD who underwent both pseudocontinuous ASL MRI at 1.5T and catheter angiography before and after superficial temporal artery encephaloduroarteriosynangiosis were included in this retrospective study. The degree of revascularization in the middle cerebral artery (MCA) territory was evaluated on external carotid angiography and was graded on a 3-point scale. On ASL CBF maps, regions of interest were manually drawn over the MCA territory of the operated side at the level of the centrum semi-ovale and over the cerebellum. The normalized CBF (nCBF) was calculated by dividing the CBF of the MCA territory by the CBF of the cerebellum. Changes in nCBFs were calculated by subtracting the preoperative nCBF values from the postoperative nCBF values. The correlation between nCBF changes measured with ASL and the revascularization grade from direct angiography was evaluated. Results: The nCBF value on the operated side increased after the operation (p = 0.001). The higher the degree of revascularization, the greater the nCBF change was: poor revascularization (grade 1), -0.043 ± 0.212; fair revascularization (grade 2), 0.345 ± 0.176; good revascularization (grade 3), 0.453 ± 0.182 (p = 0.005, Jockheere-Terpstra test). The interobserver agreement was excellent for the measured CBF values of the three readers (0.91-0.97). Conclusion: The nCBF values of the MCA territory obtained from ASL MRI increased after the revascularization procedure in children with MMD, and the degree of nCBF change showed a significant correlation with the degree of collateral formation evaluated via catheter angiography.

• 대한디지털의료영상학회논문지
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• 제12권1호
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• pp.27-31
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• 2010

The purpose of this study was to investigated the usefulness of MR perfusion image comparing with SPECT image. A total of pediatric 30 patients(average age : 7.8) with Moyamoya disease were performed MR Perfusion with 32 channel body coil at 3T from March 01, 2010 to June 10, 2010. The MRI sequences and parameters were as followed : gradient Echo-planar imaging(EPI), TR/TE : 2000ms/50ms, FA : $90^{\circ}$, FOV : $240{\times}240$, Matrix : $128{\times}128$, Thickness : 5mm, Gap : 1.5mm. Images were obtained contrast agent administrated at a rate of 1mL/sec after scan start 10s with a total of slice 1000 images(50 phase/1 slice). It was measured with visual color image and digitize data using MRDx software(IDL version 6.2) and also, it was compared of measurement with values of normal and abnormal ratio to analyze hemodynamic change, and a comparison between perfusion MR with technique using Warm Color at SPECT examination. On MR perfusion examination, the color images from abnormal region to the red collar with rCBV(relative cerebral blood volume) and rCBF(relative cerebral blood flow) caused by increase cerebral blood flow with brain vascular occlusion in surrounding collateral circulation advancement, the blood speed relatively was depicted slowly with blue in MTT(Mean Transit Time) and TTP(Time to Peak) images. The region which was visible abnormally from MR perfusion examination visually were detected as comparison with the same SPECT examination region, would be able to confirm the identical results in MMD(Moyamoya disease)judgments. Hymo-dynamic change in MR perfusion examination produced by increase and delay cerebral blood flow. This change with digitize data and being color imaging makes enable to distinguish between normal and abnormal area. Relatively, MR perfusion examination compared with SPECT examination could bring an excellent image with spatial resolution without radiation expose.

• 대한핵의학회지
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• 제32권4호
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• pp.314-324
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• 1998

목적: 이 연구의 목적은 어린이 모야모야병 환자에서 휴식기/아세타졸아미드 부하 Tc-99m-HMPAO SPECT가 Encephalo-duro-arterio-synangiosis (EDAS) 수술 후의 호전여부를 평가할 수 있는지를 알아보고자 함이었다. 대상 및 방법: 어린이 모야모야병 18명에서 21개의 대뇌반구에 EDAS 수술을 하였다. 수술 전과 수술 후 3개월에 휴식기/아세타졸아미드 부하 Tc-99in-HMPAO SPECT를 시행하여 휴식기관류와 관류예비능을 평가하여 비교하였다. 결과: 대뇌반구 모두에서 휴식기관류 또는 관류예비능 감소가 관찰되었다. 84개 뇌엽과 294개 영역 중52%의 뇌엽과 50%의 영역에 관류이상이 나타났다. 수술 후 휴식기 관류 또는 관류예비능이 감소한 영역의 60%가 호전되었고 반구는 71%가 호전되었다. 수술 전 휴식기 관류 감소가 심하지 않았을 때 수술후 호전가능성이 높았다. 수술 전 휴식 부하기 관류의 차이가 있는 경우 즉 관류예비능이 감소하였을 때 수술 후 관류 호전 가능성이 높았다. 결론: 어린이 모야모야병에서 수술 전 휴식기/아세타졸아미드 Tc-99m-HMPAO SPECT로 수술 후 호전여부를 예측할 수 있다.

• 대한장애인치과학회지
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• 제15권1호
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• pp.40-44
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• 2019

본 증례는 다수의 치아우식증을 주소로 내원한 모야모야병 환자의 전신마취 하 치과치료에 대한 보고이다. 모야모야병은 치과치료 동안 상당히 주의를 필요로 하는 다양한 전신질환과 관련이 있다. 여러 과의 의사들과 협진이 필요하고, 치과 예방치료에 초점을 맞추면서 적절한 시기에 치료하는 것이 중요하다. 모야모야 환자에서 울음과 과호흡은 저칼륨혈증을 일으킬 수 있고, 대뇌 혈관 수축 효과를 일으킬 수 있다. 치과치료 시 뇌졸중 발생을 예방하기 위해서 통증과 불안을 조절하는 것이 매우 중요하다. 비협조적이거나 매우 어린 모야모야병 환자에게 치과치료를 하기 위해서는 전신마취가 필요할 수 있다.

• Journal of Korean Neurosurgical Society
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• 제29권8호
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• pp.1136-1139
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• 2000