• 대한핵의학회:학술대회논문집
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• 대한핵의학회 1999년도 제38차 추계학술대회
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• pp.39.1-39.1
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• 1999

• Journal of Korean Neurosurgical Society
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• 제30권1호
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• pp.5-11
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• 2001

Objectives and Importance : The present study investigated the levels of basic fibroblast growth factor(bFGF) in the CSF of patients with moyamoya disease and its clinical significance. Methods : The levels of bFGF in CSF, taken from 26 hemispheres of 14 moyamoya patients and 20 patients without vascular anomaly(control group), were measured by an enzyme-linked immunosorbent assay. We analyzed the correlation between the level of bFGF and the clinical factors such as age, onset pattern, development of neovascularization, and cerebral circulation. Results : The CSF of moyamoya patients contained a high concentration of bFGF to a significant extent. The bFGF level was apparently elevated in the patients in whom neovascularization from indirect revascularization, such as modified encephalo-duro-arterio-synangiosis(EDAS) was well developed. A linear correlation between the values of bFGF and clinical progression was noted. Conclusions : The elevation of bFGF in moyamoya disease seems to be specific. Clinically, the bFGF level may be considered a useful indicator to predict the efficacy of indirect revascularization.

• 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.

• Nuclear Medicine and Molecular Imaging
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• 제42권3호
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• pp.192-200
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• 2008

목적: 본 연구의 목적은 소아 모야모야병 환자에 있어서, 수술 전후 기저/아세타졸아미드 부하 뇌혈류 단일광자방출 단층촬영(SPECT) 분석에 확률뇌지도를 이용하여 수술로 인한 뇌혈역학적 변화 및 예후 예측인자 분석을 하여 뇌확률지도의 유용성을 평가하는 것이다. 대상 및 방법: 연구대상으로 서울대어린이 병원에서 소아 모야모야병으로 진단받고, 수술받은 56명(남:여=32:24, 나이 $6.7{\pm}3.2$세)이 포함되었다. 각각의 환자는 기저/아세타졸아미드 부하 뇌혈류 SPECT를 수술 전후 6-12개월 사이에 시행하였다. 각각의 환자는 한 측 반구에 encephalo-duro-arterio-synangiosis (EDAS)와 encephalo-galeo-synangiosis (EGS)를 우선적으로 시행받았고, 그 후 순차적으로 반대측 반구에 EDAS를 시행하였다. 환자들은 수술 후 $33{\pm}21$개월 추적 관찰하였다. 환자들의 SPECT 영상을 SPM에서 공간정규화 하고 뇌교의 계수를 기준으로 계수정규화한 후 한국표준확률뇌지도 (Koreans Statistical Probabilistic Map, K-SPAM)를 이용하여 부위별 혈류를 정량화 하였다. 각각 정류화된 혈류를 수술 전후, 대뇌반구간, 그리고 임상결과에 따라 비교하였다. 또한 임상결과가 좋은 군과 나쁜 군 사이에 차이가 있는 요소를 이용하여 회귀분석을 시행하였다. 결과: 수술 후 양측 내측 전두엽이랑, 전두엽, 두정엽, 측두엽, 내측경동맥 영역, 전뇌의 기저/아세타졸아미드 부하 뇌혈류가 유의하게 호전되었다(p<0.05). 대뇌반구간 비교에서는 수술전 기저/아세타졸아미드 부하 뇌혈류 및 혈류예비능지표에 차이가 있었으나, 수술 후 이 차이는 사라졌다(p<0.05). 임상결과가 좋은 환자군의 수술 전 EDAS와 EGS를 시행한 내측 전두엽 뇌이랑의 기저 뇌혈류, 동측 전두엽, 측두엽, 그리고 전뇌의 수술 후 혈류예비능지표 및 수술전후 혈류예비능지표차가 더 우수하였다(p<0.05). 회귀분석결과에 의하면, EDAS와 EGS를 시행한 내측 전두엽 뇌이랑의 수술 전 혈류예비능지표와 전뇌의 수술 전후 혈류예비능지표차가 수술 후 임상결과를 예측할 수 있는 유의한 인자이다(p=0.002, p=0.015). Conclusion: 뇌확률지도를 이용하여 소아 모야모야병 환자의 기저/아세타졸아미드 부하 뇌혈류 SPECT를 정량화하여 분석할 수 있었다. 이 방법으로 수술에 의한 뇌혈류역학적 변화를 객관적으로 평가할 수 있었으며, 모야모야병환자의 수술 결과의 예측인자를 평가할 수 있었다.

• 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.

• 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.

• 대한핵의학회지
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• 제28권1호
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• pp.22-30
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• 1994

Encephalo-duro-arterio-synangiosis (EDAS) is a relatively new surgical procedure for treatment of childhood moyamoya disease. We assessed regional cerebral perfusion in moyamoya patients before (1.3 mo) and after (6.8 mo) EDAS with $^{99m}Tc$-HMPAO brain SPECT. A total of 21 EDAS operations in 17 moyamoya patients was included. Preoperative CT or MRI showed cerebral infarction in 14 patients and carotid angiography showed Suzuki grade I to V stenosis in 6%, 9%, 62%, 12% and 12% of the hemispheres respectively. Preoperative SPECT showed regional hypoperfusion in all patients, bilateral frontal and temporal lobes being the most frequently involved site. $4{\times}4$ pixel sized ROIs were applied on the frontotemporal cortex in 3 slice averaged transverse tomographic images. An index of regional perfusion was measured as: PI (%)=average F-T activity/average cerebellar activity${\times}100$ Pre-EDAS ipsilateral PI ranged from 23.7 to 98.4% (mean: $74.3{\pm}17%$) and increased significantly after operation ($81.4{\pm}17%$, p<0.001). Individual post-EDAS PI improved in 15/21 cases, showed no significant change in 5 and was slightly aggravated in 1. The amount of clinical improvement (${\Delta}CI$) was graded with a scale of 0 to 4 based on frequency and severity of TIA attacks. When patients were grouped according to pre-EDAS PI, group II (PI 70-89) showed a significantly higher ${\Delta}CI$ (3.3) compared to group I (PI< 70, 1.57) or group III (PI >90, 0.5) (P< 0.001). The amount of perfusion improvement (${\Delta}PI$) showed significant correlation with ${\Delta}CI$ (r=0.42, p=0.04). ${\Delta}PI$ did not, however, correlate with the amount of neovascularization assessed angiographically in 8 patients. Serial HMPAO SPECT is an useful noninvasive study for assessing perfusion improvement after EDAS in childhood moyamoya patients.