Diagnosis and Treatment of Moyamoya Disease
Diagnosis and Treatment of Moyamoya Disease
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DOI：
10.12771/emj.2013.36.1.9
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发表时间：
2013-03
期刊：
影响因子：
--
通讯作者：
E. Seo
中科院分区：
文献类型：
--
作者：
E. Seo
烟雾病是一种病因不明的脑血管疾病，其特征为双侧颈内动脉终末部、大脑前动脉和/或大脑中动脉近端部狭窄或闭塞，闭塞动脉附近血管网异常。它在亚洲国家经常发生，特别是在韩国和日本，但在西方国家很少见。为了确定烟雾病的病因，人们对尸检病理学、发病机制中涉及的因素以及遗传学进行了大量研究。可发生于儿童至成年任何年龄，一般首发表现为儿童脑缺血症状，成人颅内出血症状。由于它会进展并导致中风复发，因此早期诊断和适当的治疗已得到认可。脑血管造影对于明确诊断和治疗计划至关重要。磁共振成像/磁共振血管造影对于血运重建后的诊断和随访工具很有用。单光子发射计算机断层扫描和正电子发射断层扫描评估脑血流动力学有助于诊断和评估烟雾病患者脑缺血的严重程度。手术血运重建对于表现为缺血症状的烟雾病有效，可以防止进一步缺血和梗塞。对于出血型烟雾病，可以考虑血运重建。采用直接旁路、间接联管和组合方法。对于大多数患者来说，血运重建对于预防短暂性脑缺血发作的效果非常好。 (梨花医学杂志 2013;36(1):9-17)
Moyamoya disease is a cerebrovascular disease of unknown etiology, which is characterized by bilateral stenosis or occlusion at terminal portion of internal carotid artery and at proximal portion of anterior cerebral artery and /or middle cerebral artery and abnormal vascular network in the vicinity of the arterial occlusions. It occurs frequently in Asian countries, particularly in Korea and Japan, but is rare in Western countries. To establish the etiology of moyamoya disease, much about the pathology from autopsies, factors involved in its pathogenesis, and its genetics have been studied. It may occur at any age from childhood to adulthood and in general, initial manifestation is cerebral ischemic symptoms in children and intracranial hemorrhage symptoms in adults. Because it progress and cause recurrent stroke, early diagnosis and proper management has been recognized. Cerebral angiography is essential for definitive diagnosis and treatment plan. Magnetic resonance imaging/magnetic resonance angiography is useful for diagnosis and follow-up tools after revascularization. Evaluation of the cerebral hemodynamics by single photon emission computed tomography and positron emission tomography is useful for diagnosis and assessment of the severity of cerebral ischemia in moyamoya patients. Surgical revascularization is effective for moyamoya disease manifesting as ischemic symptoms, to prevent further ischemia and infarction. In hemorrhagic type moyamoya disease, revascularization can be considered. Direct bypass, indirect synangiosis and combined methods are used. Outcomes of revascularization are excellent in preventing transient ischemic attacks in most patients. (Ewha Med J 2013;36(1):9-17)