• Journal of Korean Neurosurgical Society
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• 제56권6호
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• pp.492-495
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• 2014

A ruptured distal lenticulostriate artery (LSA) aneurysm is detected occasionally in moyamoya disease (MMD) patients presented with intracerebral hemorrhage. If the aneurysm is detected in hemorrhage site on angiographic evaluation, its obliteration could be considered, because it rebleeds frequently, and is associated with poorer outcome and mortality in MMD related hemorrhage. In this case report, the authors present two MMD cases with ruptured distal LSA aneurysm treated by endovascular embolization.

• Journal of Korean Neurosurgical Society
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• 제59권6호
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• pp.537-543
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• 2016

A better understanding of moyamoya disease (MMD), such as natural clinical course, surgical outcomes and research, has been obtained. This review article focuses on an giving an update for adult MMD in the Korean population. In this paper, we mainly discuss the results of our domestic investigations including meta-analysis, and related subjects from other countries.

• Journal of Korean Neurosurgical Society
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• 제49권1호
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• pp.65-67
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• 2011

The detailed clinical characteristics of unilateral moyamoya disease (MMD) have not been fully elucidated. It has been reported that some patients with unilateral MMD progress to bilateral involvement, while others remain with the unilateral variant In this series, we present a case of unilateral MMD that progressed to bilateral involvement over the course of just one month.

• Annals of Clinical Neurophysiology
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• 제18권1호
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• pp.1-6
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• 2016

Background: Moyamoya disease is characterized by a progressive stenosis or occlusion of the intracranial internal carotid artery and/or the proximal portion of the anterior cerebral artery and middle cerebral artery. Whether the onset time was childhood or adulthood, the bony carotid canal diameter might be different, but reflects the size of internal carotid artery passing through the bony carotid canal. In this study, we aimed to identify the relationship between bony carotid canal diameter and clinical manifestation. Methods: 146 consecutive patients diagnosed with moyamoya disease by brain imaging studies were included. We measured the diameter of a transverse portion of bony carotid canal on bone window of a brain computed tomography(CT) image. Patients were divided into two groups, ischemic or hemorrhagic stroke according to clinical manifestation. As a result, 115 patients were included. The Suzuki stage was used as criteria for disease progression. Results: Bony carotid canal diameter was $3.6{\pm}0.5$ (right) and $3.6{\pm}0.4$ (left) in the hemorrhagic stroke group, and $3.7{\pm}0.4$ (right) and $3.6{\pm}0.4$ (left) in the ischemic stroke group. The bony carotid canal diameter of the moyamoya vessels (3.6 mm) was smaller than the diameter of non-moyamoya vessels (3.8 mm), significantly (p = 0.042). However, there was no difference in the collateral patterns and clinical manifestation in a comparison of both groups. Conclusions: In our study, there was no significant difference of clinical manifestations and collateral patterns depend on the bony carotid canal diameter in patients with moyamoya disease. These findings suggest that the clinical presentations of moyamoya disease are not related to the onset time of the disease.

• Journal of Korean Neurosurgical Society
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• 제57권6호
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• pp.415-421
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• 2015

Moyamoya disease (MMD) is an arteriopathy of the intracranial circulation predominantly affecting the branches of the internal carotid arteries. Heterogeneity in presentation, progression and response to therapy has prompted intense study to improve the diagnosis and prognosis of this disease. Recent progress in the development of moyamoya-related biomarkers has stimulated marked interest in this field. Biomarkers can be defined as biologically derived agents-such as specific molecules or unique patterns on imaging-that can identify the presence of disease or help to predict its course. This article reviews the current categories of biomarkers relevant to MMD-including proteins, cells and genes-along with potential limitations and applications for their use.

• Journal of Korean Neurosurgical Society
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• 제46권3호
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• pp.257-260
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• 2009

Intraventricular hemorrhage long after successful encephaloduroarterio synangiosis (EDAS) is very rare. The effect of revascularization surgery for preventing hemorrhagic event of moyamoya disease remains controversial. We report a 17-year-old female with intracerebral hemorrhage and intraventricular hemorrahge 10 years after successful EDAS. Even though cerebral vessels angiography showed good collateral circulations without specific weak points, a cerebral hemorrhage could occur in patient with ischemic type of moyamoya disease long after successful indirect bypass operations. Good collateralization of cerebral angiography or magnetic resonance perfusion image after indirect bypass surgery would ensure against ischemic symptoms, not a hemorrhage. And, thus a life-time follow-up strategy might be necessary even if a good collateral circulation has been established.

• Clinical and Experimental Pediatrics
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• 제46권1호
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• pp.86-90
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• 2003

Alagille 증후군은 간내 담도 형성 부전으로 인한 만성 담즙 정체, 말초 폐동맥 협착, 골격 이상, 눈의 이상, 특징적인 얼굴 모양 등을 주요 증상으로 하는 상염색체 우성 유전 질환으로 말초 폐동맥 협착 이외에도 여러 말초 혈관 병변들이 동반된다. 관련된 유전자로 20번 염색체 위에 존재하는 JAG1이 발견되었고, 이 유전자의 결손으로 인한 Notch 신호전달체계의 결함이 혈관형성에 영향을 미친다고 생각되고 있다. Alagille 증후군에서 만성 뇌동맥 폐색질환인 moyamoya병이 발생한 예들이 보고된 바 있는데, Alagille 증후군의 혈관병증의 또 다른 한 발현으로 이해되어야 할 것이다. 저자들은 생후 2개월에 Alagille 증후군을 진단받고 추적 관찰 중에 갑자기 편측 마비가 발생하여 뇌혈관 조영술을 통해 moyamoya병을 진단받은 25개월 여아를 경험하였기에 이를 보고하는 바이다.

• Journal of Korean Neurosurgical Society
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• 제41권6호
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• pp.414-417
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• 2007

A 52-year-old woman with hypertension and moyamoya disease presented with chronic subdural hematoma [CSDH]. The presumed cause of bleeding was ascribed to administrated antiplatelet agents. She responded slowy and clumsily to verbal commands and had right arm weakness. After surgery, her clinical condition improved. But two days after surgery, her symptoms became aggravated and a convulsive seizure was noted within 24 hours. Brain magnetic resonance imaging showed no organic lesion except a small amount of residual CSDH. In addition, there was no laboratory evidence of metabolic brain disease. Moreover, after the administration of an antiepileptic drug [phenytoin], her manifestations disappeared. Therefore, the authors presume that her symptoms were resulted from nonconvulsive status epilepticus [NCSE], despite a lack of ictal period electroencephalographic findings. The authors were unable to find a single case report on postoperative NCSE in Korea. Therefore, the authors report this case of nonconvulsive status epilepticus after drainage of a CSDH in a patient with moyamoya disease.

• 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.

• Journal of Korean Neurosurgical Society
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• 제54권4호
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• pp.302-308
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• 2013

Objective : MR perfusion and single photon emission computerized tomography (SPECT) are well known imaging studies to evaluate hemodynamic change between prior to and following superficial temporal artery (STA)-middle cerebral artery (MCA) anastomosis in moyamoya disease. But their side effects and invasiveness make discomfort to patients. We evaluated the ivy sign on MR fluid attenuated inversion recovery (FLAIR) images in adult patients with moyamoya disease and compared it with result of SPECT and MR perfusion images. Methods : We enrolled twelve patients (thirteen cases) who were diagnosed with moyamoya disease and underwent STA-MCA anastomosis at our medical institution during a period ranging from September of 2010 to December of 2012. The presence of the ivy sign on MR FLAIR images was classified as Negative (0), Minimal (1), and Positive (2). Regions were classified into four territories: the anterior cerebral artery (ACA), the anterior MCA, the posterior MCA and the posterior cerebral artery. Results : Ivy signs on preoperative and postoperative MR FLAIR were improved (8 and 4 in the ACA regions, 13 and 4 in the anterior MCA regions and 19 and 9 in the posterior MCA regions). Like this result, the cerebrovascular reserve (CVR) on SPECT was significantly increased in the sum of CVR in same regions after STA-MCA anastomosis. Conclusion : After STA-MCA anastomosis, ivy signs were decreased in the cerebral hemisphere. As compared with conventional diagnostic modalities such as SPECT and MR perfusion images, the ivy sign on MR FLAIR is considered as a useful indicator in detecting brain hemodynamic changes between preoperatively and postoperatively in adult moyamoya patients.