• Journal of Korean Neurosurgical Society
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• 제57권6호
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• pp.408-414
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• 2015

Moyamoya-like vasculopathy develops in association with various systemic diseases and conditions, which is termed moyamoya syndrome. Relatively common diseases and conditions are related to moyamoya syndrome, including neurofibromatosis type 1, Down syndrome, thyroid disease, and cranial irradiation. Moyamoya syndrome shares phenotypical characteristics with idiopathic moyamoya disease. However, they differ in other details, including clinical presentations, natural history, and treatment considerations. The study of moyamoya syndrome can provide clinicians and researchers with valuable knowledge and insight. Although it is infrequently encountered in clinical practice, moyamoya-like vasculopathy can severely complicate outcomes for patients with various underlying diseases when the clinician fails to expect or diagnose moyamoya syndrome development. Furthermore, moyamoya syndrome could be used as a doorway to more enigmatic moyamoya disease in research. More comprehensive survey and investigation are required to uncover the secrets of all the moyamoya-like phenomena.

• Journal of Korean Neurosurgical Society
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• 제57권6호
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• pp.440-444
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• 2015

Quality of life is the current trend and issue for the most of human diseases. In moyamoya disease (MMD), surgical revascularization has been recognized as the possible assistance to reduce the neurological insult. However, the progressive nature of the disease has been invincible so far. To improve the quality of life of MMD patients not only the protection from the neurological insult but also the maintenance or improvement of cognitive function is inevitable. For pediatric MMD patients, younger age or longer duration of disease is the key factor among the prognostic factors for bad neurological outcomes. Hence, 'the earlier, the better' is the most precious rule for treatment. Protection from neurological insult is very critical and foremost important to improve cognitive outcome. Clinicians need to know the neuropsychological profile of MMD patients for the care of whole person and make an effort to protect the patients from neurological insults to maintain or improve it.

• Journal of Korean Neurosurgical Society
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• 제57권6호
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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.

• Child Health Nursing Research
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• 제21권2호
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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.

• Journal of Korean Neurosurgical Society
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• 제37권3호
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• pp.207-212
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• 2005

Objective: Classically, single photon emission tomography is known to be the reference standard for evaluating the hemodynamic status of patients with moyamoya disease. Recently, T2-weighted perfusion magnetic resonance(MR) imaging has been found to be effective in estimating cerebral hemodynamics in moyamoya disease. We aim to assess the utility of perfusion-weighted MR imaging for evaluating hemodynamic status of moyamoya disease. Methods: The subjects were fourteen moyamoya patients(mean age: 7.21 yrs) who were admitted at our hospital between Sep. 2001 to Sep 2003. Four normal children were used for control group. Perfusion MR imaging was performed before any treatment by using a T2-weighted contrast material-enhanced technique. Relative cerebral blood volume(rCBV) and time to peak enhancement(TTP) maps were calculated. Relative ratios of rCBV and TTP in the anterior cerebral artery(ACA), middle cerebral artery(MCA) and basal ganglia were measured and compared with those of the posterior cerebral artery(PCA) in each cerebral hemispheres. Using this data, we analysed the hemodynamic aspect of pediatric moyamoya disease patients in regarding to the age, Suzuki stage, signal change in FLAIR MR imaging, and hemispheres inducing symptoms. Results: The mean rCBV ratio of ACA, MCA did not differ between normal children and moyamoya patients. However the significant TTP delay was observed at ACA, MCA territories (mean = 2.3071 sec, 1.2089 see, respectively, p < 0.0001). As the Suzuki stage of patients is advanced, rCBV ratio is decreased and TTP differences increased. Conclusion: Perfusion MR can be applied for evaluating preoperative cerebral hemodynamic status of moyamoya patients. Furthermore, perfusion MR imaging can be used for determine which hemisphere should be treated, first.

• Clinical and Experimental Pediatrics
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• 제57권6호
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• pp.287-291
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• 2014

Moyamoya disease is a cerebrovascular condition that results in the narrowing of the vessels of the circle of Willis and collateral vessel formation at the base of the brain. Although relationships between Graves' disease and cerebrovascular accidents in Moyamoya disease are obscure, the coexistence of the two diseases is noteworthy. Moyamoya disease has been rarely reported in adolescent patients with thyrotoxicosis. Recently, we encountered two adolescent Korean patients with Moyamoya disease associated with Graves' disease who presented with episodic right-sided hemiparesis and syncope. These two girls who had Graves' disease had no history of other diseases or head trauma. A thyroid function test revealed a euthyroid state and a high thyroid-stimulating hormone (TSH) receptor antibody titer at that time. The patients were diagnosed with Moyamoya disease based on brain magnetic resonance angiography and cerebral four-vessel angiography. The patients underwent cranial revascularization by encephalo-duroarterio-synangiosis as soon as a diagnosis was made, which resulted in successful symptom resolution. They fared well and had no additional neurological symptoms as of their last follow-up visits. Here, we report these two cases of confirmed Moyamoya disease complicated by Graves' disease with a review of the literature, and discuss the possible association between the two diseases. To our knowledge, this is the first report in South Korea on Moyamoya disease associated with Graves' disease in adolescents with a euthyroid.

• 대한장애인치과학회지
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• 제13권2호
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• pp.108-113
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• 2017

MMD 환자는 심한 울음이나 운동 등으로 유발되는 과호흡성 뇌혈류량 감소에 주의해야 하며, 특히 소아 환자의 경우 낯선 환경에서 쉽게 울음을 터뜨리는 경향이 있고, 뇌의 산소요구량이 많으며, 뇌혈류량 변화에 민감하므로 치과 진료 시에 더욱 세심한 관리가 필요하다. 본 증례는 발달 지연을 동반하여 일상의 구강 위생 관리 및 치과 검진에 비협조적이고, 다수 치아에 광범위한 우식증을 보이는 발달장애가 있는 MMD 소아 환자를 전신 마취 하에 안전하고 효과적으로 치료한 사항을 보고하는 바이다. 환아는 이전에 수차례의 뇌경색 증상을 보여 뇌혈관 문합술을 받은 바 있고, 발달 지연 외에도 지적 장애, 언어장애, 편마비, 연하장애, 사시증을 동반하였다. 환아의 기저 질환과 협조도, 우식의 심도를 고려하여 세보플루레인을 이용한 전신 마취를 행동조절 요법으로 선택하였고, 술 전 정맥로 확보 시 환아의 불안을 조절하기 위해 미다졸람 경구 투여를 시행하였다. 모든 생징후를 안전하게 감시하며 성공적으로 치과 치료를 마친 후, 적극적인 통증 조절을 위해 아세트아미노펜 경구 투여를 시행하였다. 전신마취 이후에는 정기 검진 및 불소 도포를 통해 환자의 구강 건강 증진에 기여할 수 있었다.

• Clinical and Experimental Pediatrics
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• 제55권11호
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• pp.408-413
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• 2012

Moyamoya disease (MMD) is the most common pediatric cerebrovascular disease in Far Eastern countries. In children, MMD frequently manifests as ischemic symptomatology. Cerebral perfusion gradually decreases as the disease progresses, which often leads to cerebral infarction. The benefits of revascularization surgery, whether direct or indirect, have been well established in MMD patients with ischemic symptoms. In adults, the increase in cerebral blood flow achieved with indirect revascularization is often unsatisfactory, and direct revascularization is usually feasible. In children, however, direct revascularization is frequently technically not feasible, whereas the response to indirect revascularization is excellent, although 1 or 2 weeks are required for stabilization of symptoms. The authors describe surgical procedures and perioperative care in indirect revascularization for MMD. In addition, special considerations with regard to very young patients, patients with recent cerebral infarction, and patients with hyperthyroidism are discussed.

• Journal of Korean Neurosurgical Society
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• 제57권6호
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• pp.436-439
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• 2015

The majority of clinical studies on moyamoya disease (MMD) have focused on anterior circulation. The disease involvement of posterior circulation in MMD, mainly in the posterior cerebral artery (PCA), has been mentioned since the early 1980s, and it has been repeatedly emphasized as one of the most important factors related to poor prognosis in MMD. However, its clinical features and outcome have only been elucidated during the last few years. In this review, the angiographic definition of PCA stenosis is summarized. The clinical features are elucidated as being either early-onset or delayed-onset, according to the time of PCA stenosis diagnosis in reference to the anterior circulation revascularization surgeries. The surgical strategy and hypothesis on the mechanism of PCA stenosis is also briefly mentioned. It appears that some MMD patients may show PCA stenosis during the early or late course of the disease and that the presenting symptoms may vary. Because the hemodynamic compromise caused by PCA stenosis may respond well to surgical treatment, clinicians should be aware of the condition, especially during follow-up of MMD patients.

• 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.