• Journal of Korean Neurosurgical Society
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• v.50 no.6
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• pp.486-491
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• 2011

Objective : Structural genetic variation, including copy-number variation (CNV), constitutes a substantial fraction of total genetic variability, and the importance of structural variants in modulating susceptibility is increasingly being recognized. CNV can change biological function and contribute to pathophysiological conditions of human disease. Its relationship with common, complex human disease in particular is not fully understood. Here, we searched the human genome to identify copy number variants that predispose to moya-moya type cerebrovascular disease. Methods : We retrospectively analyzed patients who had unilateral or bilateral steno-occlusive lesions at the cerebral artery from March, 2007, to September, 2009. For the 20 subjects, including patients with moyamoya type pathologies and three normal healthy controls, we divided the subjects into 4 groups : typical moyamoya (n=6), unilateral moyamoya (n=9), progression unilateral to typical moyamoya (n=2) and non-moyamoya (n=3). Fragmented DNA was hybridized on Human610Quad v1.0 DNA analysis BeadChips (Illumina). Data analysis was performed with GenomeStudio v2009.1, Genotyping 1.1.9, cnvPartition_v2.3.4 software. Overall call rates were more than 99.8%. Results : In total, 1258 CNVs were identified across the whole genome. The average number of CNV was 45.55 per subject (CNV region was 45.4). The gain/loss of CNV was 52/249, having 4.7 fold higher frequencies in loss calls. The total CNV size was 904,657,868, and average size was 993,038. The largest portion of CNVs (613 calls) were 1M-10M in length. Interestingly, significant association between unilateral moyamoya disease (MMD) and progression of unilateral to typical moyamoya was observed. Conclusion : Significant association between unilateral MMD and progression of unilateral to typical moyamoya was observed. The finding was confirmed again with clustering analysis. These data demonstrate that certain CNV associate with moyamoya-type cerebrovascular disease.

• Journal of Korean Neurosurgical Society
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• v.50 no.6
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• pp.492-496
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• 2011

Objective : Direct revascularization surgery is regarded as the most effective method of treatment of adults with moyamoya disease. These patients, however, have a higher risk of perioperative ischemic complications than do patients with atherosclerotic stroke, and are at risk for ischemic complications in the hemisphere contralateral to the one operated on. We investigated the incidence and risk factors for ischemic stroke in the contralateral hemisphere after surgical treatment of adults with moyamoya disease. Methods : We retrospectively reviewed the medical records and results of neuroimaging studies on 79 hemispheres of 73 consecutive patients with adult moyamoya disease ($mean{\pm}SD$ age, $37.96{\pm}11.27$ years; range, 18-62 years) who underwent direct bypass surgery over 6 years. Results : Ischemic complications occurred in 4 of 79 (5.1%) contralateral hemispheres, one with Suzuki stage 3 and three with Suzuki stage 4. Three patients showed posterior cerebral artery (PCA) involvement by moyamoya vessels. Advanced stage of moyamoya disease (Suzuki stages 4/5/6; $p$=0.001), PCA involvement ($p$=0.001) and postoperative hypotension (mean arterial blood pressure <80% of preoperative mean arterial blood pressure) on the first ($p$<0.0001) and second ($p$=0.003) days after surgery were significantly correlated with postoperative contralateral ischemic complications. Conclusion : In patients with advanced moyamoya disease and involvement of the PCA, intentional hypotension can result in ischemic stroke in the hemisphere contralateral to the one operated on. Careful control of perioperative blood pressure is crucial for good surgical results.

• Journal of Korean Neurosurgical Society
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• v.51 no.6
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• pp.367-369
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• 2012

Terson syndrome was originally used to describe a vitreous hemorrhage arising from aneurysmal subrarachnoid hemorrhage. Terson syndrome can be caused by intracranial hemorrhage, subdural or epidural hematoma and severe brain injury but is extremely rare in intraventricular hemorrhage associated with moyamoya disease. A 41-year-old man presented with left visual disturbance. He had a history of intraventicular hemorrhage associated with moyamoya disease three months prior to admission. At that time he was in comatose mentality. Ophthalmologic examination at our hospital detected a vitreous hemorrhage in his left eye, with right eye remaining normal. Vitrectomy with epiretinal membrane removal was performed. After operation his left visual acuity was recovered. Careful ophthalmologic examination is mandatory in patients with hemorrhagic moyamoya disease.

• Journal of Korean Neurosurgical Society
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• v.57 no.6
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• pp.432-435
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• 2015

Moyamoya disease is a unique cerebrovascular disorder characterized by idiopathic progressive stenosis at the terminal portion of the internal carotid artery (ICA) and fine vascular network. The aim of this review is to present the clinical application of quantitative digital subtraction angiography (QDSA) in pediatric moyamoya disease. Using conventional angiographic data and postprocessing software, QDSA provides time-contrast intensity curves and then displays the peak time ($T_{max}$) and area under the curve (AUC). These parameters of QDSA can be used as surrogate markers for the hemodynamic evaluation of disease severity and quantification of postoperative neovascularization in moyamoya disease.

• Journal of Korean Neurosurgical Society
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• v.57 no.6
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• pp.428-431
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• 2015

Various approaches have been attempted in translational moyamoya disease research. One promising material for modeling and treating this disease is vascular progenitor cells, which can be acquired and expanded from patient peripheral blood. These cells may provide a novel experimental model and enable us to obtain insights regarding moyamoya disease pathogenesis. We briefly present the recent accomplishments in regard to the studies of vascular progenitor cells in moyamoya disease.

• Proceedings of the KSME Conference
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• 2008.11a
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• pp.1647-1650
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• 2008

The moyamoya disease is a type of cerebrovascular disease which produces thin abnormal blood vessels like haze in the brain base because the end of internal carotid artery which supplies about 80% of blood is blocked. Regarding this moyamoya disease, the shearing stress and thrombus generation are mentioned as its main causes. This study three-dimensionally implemented the ICA, ACA, and MCA parts of the cerebrovascular configuration related to the moyamoya disease, and analyzed the hydrodynamic phenomenon with the commercial program ADINA. In particular, the correlations between shearing stress and speed distribution according to the branch angle of ACA and MCA. A numerical analysis found that the greater the branch angle of ACA and MCA, the lower the shearing stress and the greater the stationary area of the flow.. Put Abstract text here.

• Journal of Physiology & Pathology in Korean Medicine
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• v.21 no.2
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• pp.578-581
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• 2007

Moyamoya disease is characterized by the angiographic findings of bilateral occlusion of the distal portion of the internal carotid arteries with a vascular network at the base of the brain. The typical findings on conventional angiography and magnetic resonance imaging usually confirm the correct diagnosis of moyamoya disease. We experienced a 11-year-old girl with moyamoya disease showing repeated transient ischemic attacks and we report on this case with a review of the literature.

• The Journal of Pediatrics of Korean Medicine
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• v.14 no.2
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• pp.141-147
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• 2000

Moyamoya is a chronic cerebrovascular disease chracterized by progressive stenosis or occlusion of the terminal parts of both internal carotid arteries with telangiectatic vascular network of collateral circuration at the base of the brain and leptomeningeal arteries. The etiology and pathophysiology of this disease are still unknown. The clinical course in those whose first symptoms occur in childhood is different from those in whom symptoms develop in adult life. The term moyamoya disease should be resserved for those cases in which the chracteristic angiographic pattern is idiopathic; moayamoya syndrome is used when the underlying condition is known. we have experienced a case of moyamoya syndrome in a 5-year-2-month-old boy who presented right-sided hemiparesis. A cerebral angiogram revealed occlusion of abnomal collateral network. Moyamoya disease is applicable to stroke of an infant from oriental medicine point of view, and The symptoms is similar to adult stroke, we have treated adult stroke patint with herb medicine and acupuncutre and physical treatment. The acute stage of stroke is applied to the external treatment(標治), and The recovery stage is applied to the basic treament(本治).

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.39 no.6
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• pp.289-291
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• 2013

Moyamoya disease is a rare neurovascular disorder that involves constriction of certain arteries in the brain. In patients with moyamoya disease, it is very important to prevent cerebral ischemic attacks and intracerebral bleeding caused by fluctuating blood pressure and increased respiration. A 40-year-old woman with moyamoya disease was scheduled for extraction of her right upper and lower impacted wisdom teeth. Her lower impacted wisdom tooth was situated close to the inferior alveolar nerve. We decided to continue her oral antiplatelet therapy and planned intravenous sedation with analgesic agents administered approximately five minutes prior to extraction of the root of the mandibular wisdom tooth. Oral analgesic medications were regularly administered postoperatively to alleviate pain and anxiety. During the perioperative period, no cerebrovascular event occurred, and the wisdom teeth were successfully extracted as per the planned procedure. It is thought that the perioperative risks of wisdom tooth extraction in patients with moyamoya disease can be minimized with the use of our protocols.

• Child Health Nursing Research
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• v.21 no.2
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• pp.123-130
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• 2015

Purpose: The purpose of this study was to understand the intellectual and psychological features of children with Moyamoya disease who were patients in the Department of Pediatric Neurosurgery of Severance Hospital. Methods: From December 2010 through December 2012, 63 patients with diagnoses of Moyamoya disease and 59 children in a normal group were enrolled. This study was conducted using the Korean-Wechsler Intelligence Scale for Children, Rey-Kim Memory Test for children, Korean Child Behavior Checklist and Pediatric Quality of Life Inventory$^{TM}$4.0. Results: The results showed that the intellectual and psychological profiles of children with Moyamoya disease were lower than the average of the normal control group. The tested patients showed significantly lower scores for Performance Intelligence Quotient cognition level. Also, in terms of quality of life, children with Moyamoya disease had lower levels of physical and school functionality. The results were in line with those of previous studies involving psychological tests of children with chronic diseases. Conclusion: Considering the intellectual and psychological characteristics of children with Moyamoya disease, integrated psychological intervention plans including elements such as supportive therapy for patients and programs for parental education are required.