• 대한핵의학회:학술대회논문집
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• 1999.11a
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• pp.39.1-39.1
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• 1999

• Journal of Korean Neurosurgical Society
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• v.30 no.1
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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.

• The Korean Journal of Nuclear Medicine
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• v.32 no.4
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• pp.314-324
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• 1998

Purpose: This study was performed to evaluate the outcome of encephalo-duro-arterio-synangiosis (EDAS) surgery with rest/acetazolamide Tc-99m HMPAO SPECT in moyamoya disease. Materials and Methods: Rost/acetazolamide subtraction SPECT with consecutive acquisition were done before and 2 months after 21 EDAS surgeries in 18 patients. Perfusion decrease was graded visually for 14 areas of each hemisphere as 0 (normal) to 3 (defect) using 4 point scoring system. Postoperative rest perfusion or perfusion reserve was compared with preoperative ones. Results: Among 294 areas of 21 hemispheres, rest perfusion abnormality was found in 91 areas of 15 hemispheres. Decrease of perfusion reserve was found in 146 areas of 18 hemispheres. Six hemispheres having normal rest perfusion and 12 of 15 hemispheres having rest perfusion abnormality showed reserve decrease. Three having rest perfusion defect did not change after acetazolamide in preoperative SPECT. After operation, 16 patients (89%) demonstrated clinical improvement. Fifteen among 18 hemispheres (83%) with decreased reserve improved. Rest perfusion abnormality improved in 6 among the 15 hemispheres (40%). The areas having rest perfusion and/or reserve decrease improved in 87 among 146 areas (60%). Decrease of reserve, improved in 85% (68/80). However, areas without reserve decrease also improved in 29% (19/66). The better was preoperative rest perfusion in involved areas or the more decreased vascular reserve, the more improved perfusion and reserve after operation. Conclusion: We conclude that assessment of perfusion and Perfusion reserve using rest/acetazolamide brain perfusion SPECT predict the surgical outcome in patients with moyamoya disease.

• Nuclear Medicine and Molecular Imaging
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• v.42 no.3
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• pp.192-200
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• 2008

To evaluate the hemodynamic changes and the predictive factors of the clinical outcome in pediatric patients with moyamoya disease, we analyzed pre/post basal/acetazolamide stress brain perfusion SPECT with automated volume of interest (VOIs) method. Methods: Total fifty six (M:F = 33:24, age $6.7{\pm}3.2$ years) pediatric patients with moyamoya disease, who underwent basal/acetazolamide stress brain perfusion SPECT within 6 before and after revascularization surgery (encephalo-duro-arterio-synangiosis (EDAS) with frontal encephalo-galeo-synangiosis (EGS) and EDAS only followed on contralateral hemisphere), and followed-up more than 6 months after post-operative SPECT, were included. A mean follow-up period after post-operative SPECT was $33{\pm}21$ months. Each patient's SPECT image was spatially normalized to Korean template with the SPM2. For the regional count normalization, the count of pons was used as a reference region. The basal/acetazolamide-stressed cerebral blood flow (CBF), the cerebral vascular reserve index (CVRI), and the extent of area with significantly decreased basal/acetazolamide- stressed rCBF than age-matched normal control were evaluated on both medial frontal, frontal, parietal, occipital lobes, and whole brain in each patient's images. The post-operative clinical outcome was assigned as good, poor according to the presence of transient ischemic attacks and/or fixed neurological deficits by pediatric neurosurgeon. Results: In a paired t-test, basal/acetazolamide-stressed rCBF and the CVRI were significantly improved after revascularization (p<0.05). The significant difference in the pre-operative basal/acetazolamide-stressed rCBF and the CVRI between the hemispheres where EDAS with frontal EGS was performed and their contralateral counterparts where EDAS only was done disappeared after operation (p<0.05). In an independent student t-test, the pre-operative basal rCBF in the medial frontal gyrus, the post-operative CVRI in the frontal lobe and the parietal lobe of the hemispheres with EDAS and frontal EGS, the post-operative CVRI, and ${\Delta}CVRI$ showed a significant difference between patients with a good and poor clinical outcome (p<0.05). In a multivariate logistic regression analysis, the ${\Delta}CVRI$ and the post-operative CVRI of medial frontal gyrus on the hemispheres where EDAS with frontal EGS was performed were the significant predictive factors for the clinical outcome (p =0.002, p =0.015), Conclusion: With probabilistic map, we could objectively evaluate pre/post-operative hemodynamic changes of pediatric patients with moyamoya disease. Specifically the post-operative CVRI and the post-operative CVRI of medial frontal gyrus where EDAS with frontal EGS was done were the significant predictive factors for further clinical outcomes.

• Journal of Korean Neurosurgical Society
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• v.56 no.4
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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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• v.38 no.6
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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.

• The Korean Journal of Nuclear Medicine
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• v.28 no.1
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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.