• Journal of Chest Surgery
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• v.35 no.3
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• pp.231-234
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• 2002

Moyamoya disease is an unusual cerebrovascular disorder characterized by occlusive intimal dysplasia of the distal internal carotid and proximal cerebral arteries as well as other collateral arteries. However, moyamoya diseases are recently being reported as a systemic process. We experienced one case of coronary artery occlusive disease affected by moyamoya disease. The patient was a 35-year-old female, experiencing intermittent NYHA class ll dyspnea and exertional chest pain for 6 months and right paresthesia for 1 month before admission. Cerebral artery angiogram showed abnormal cerebrovascular systems and confirmed moyamoya disease with cerebral infarction of the left frontal lobe. In coronary artery angiogram, left coronary artery was not visualized due to total occlusion of the left main ostium and left coronary blood flow was supplied from normal right coronary artery. CABG was performed with OPCAB. Both internal mammary arteries were used for LAD and LCx. Intraoperative coronary artery findings showed intimal hyperplasia and no definite thrombi, and nondiseased coronary arteries were good and patent. We concluded that this patient's coronary artery disease was affected by moyamoya disease, and moyamoya disease should be evaluated in the extracerebral cardiovascular system.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.4 no.1
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• pp.1-4
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• 2004

저자들은 4년 전 모야모야병으로 진단되어 수술 받았던 16세의 여아에서 지속적으로 고지질혈증이 있어 검사를 시행한 결과 가족 중 여동생에게서도 고지질혈증이 있음이 확인되었고 대사성산증, 고요산혈증, 젖산혈증 및 간비대가 지속되어 시행했던 염기 서열 분석에서 당원병 type Ia로 진단된 1례를 경험하였기에 보고하는 바이다.

• Childhood Kidney Diseases
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• v.11 no.2
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• pp.294-298
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• 2007

Moyamoya disease is a progressive cerebrovascular disorder with stenosis or occlusion of the bilateral internal carotid arteries with abnormal vascular networks at the base of the brain. Previous reports have shown that there are extracranial vascular involvements in Moyamoya disease, especially in the renal artery. We report a 7-year-old patient with Moyamoya disease associated with renovascular hyper tension, who presented in infancy with seizures and hemiparesis. Renal angiography showed multiple stenoses of the right renal artery. Although renal artery stenosis in Moyamoya disease has been effectively treated with balloon angioplasty, stent implantation, or surgery, bat-loon angioplasty could not be done in this patient due to multiple stenoses. His blood pressure was successfully controlled with medical treatment, and remained normotensive during the follow up period of 6 months.

• Journal of Korean Neurosurgical Society
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• v.29 no.8
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• pp.1136-1139
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• 2000

• 대한핵의학회:학술대회논문집
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• 1996.05a
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• pp.180-180
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• 1996

• 대한핵의학회:학술대회논문집
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• 1998.11a
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• pp.33.1-33.1
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• 1998

• Childhood Kidney Diseases
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• v.17 no.2
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• pp.143-148
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• 2013

Neurofibromatosis type 1 (NF-1) is an autosomal dominant neurocutaneous disorder, which can affect different organs or systems of the body, including the cardiovascular system. One of the more serious aspects of the disease relates to arterial involvement. In particular, renal artery stenosis is one of the most common vascular abnormalities in patients with NF-1, and the manifestations vary, ranging from no symptoms to end-stage renal failure. Treatment usually consists of antihypertensive drugs, percutaneous transluminal angioplasty, or surgery. Other causes of hypertension should be ruled out and the patient followed up for close monitoring and proper management. We report a case of bilateral renal artery stenosis and hypertension in a patient with moyamoya disease associated with neurofibromatosis type 1. This report discusses the literature available on the current subject, its clinical features, diagnosis, and treatment.

• Journal of radiological science and technology
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• v.30 no.4
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• pp.427-433
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• 2007

This study was conducted to investigate the usefulness of PASL image technique through visual and quantitative assessment by dividing CBF image, conventional perfusion magnetic resonance image, anterior cerebral artery, middle cerebral artery and posterior cerebral artery into 6 territories both right and left in moyamoya disease. In visual assessment, the scope of decreased perfusion in the PASL CBF image and conventional perfusion MR CBF image agreed with the position of deficiency in the MR image. The quantitative assessment, showed that the scope and position of decreased perfusion accord with both in the PASL CBF image and the existing conventional perfusion MR CBF image but the assessment of measuring the quantity of perfusion according to signal intensity showed a little difference.

• 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.

• 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.