Moyamoya disease
Moyamoya disease
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DOI：
10.1046/j.1440-1789.2000.00300.x
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发表时间：
2000-09-01
期刊：
影响因子：
2.3
通讯作者：
Ikezaki, K
中科院分区：
文献类型：
--
作者：
Fukui, M;Kono, S;Ikezaki, K
Moyamoya病是1957年由日本外科医生首次报道的一种特殊的慢性脑血管闭塞性疾病。该疾病的特征是双侧颈内动脉末端部分狭窄或闭塞以及动脉闭塞附近的异常血管网络。它可能导致缺血性发作或脑梗死，这在儿童中比成人更常见。在成人中，可能发生脑出血。该疾病分布于所有年龄组，但最高峰在10岁以下的儿童。狭窄闭塞动脉的典型组织病理学特征是内膜纤维细胞增厚，含有增殖的平滑肌细胞和显著迂曲且经常重复的内弹性膜。动脉壁通常没有粥样斑块。该病的病因尚不清楚;然而，由于日本人和韩国人的发病率较高，日本人的家族发病率约为10%，因此认为可能存在多因素遗传。最近的遗传学研究表明，一些负责的遗传灶在染色体3，6和17。
Moyamoya disease is a specific chronic cerebrovascular occlusive disease first reported by Japanese surgeons in 1957. The disease is characterized by stenosis or occlusion of the terminal portions of the bilateral internal carotid arteries and abnormal vascular network in the vicinity of the arterial occlusion. It may cause ischemic attacks or cerebral infarction, which is more frequent in children than in adults. In adults, cerebral hemorrhage may occur. The disease is distributed in all age groups, but the highest peak is in childhood at less than 10 years of age. The characteristic histopathologic features of the steno-occlusive arteries are fibrocellular thickening of the intima containing proliferated smooth muscle cells and prominently tortuous and often duplicated internal elastic lamina. There is usually no atheromatous plaque in the arterial wall. Etiology of the disease is still unknown; however, multifactorial inheritance is considered possible because of a higher incidence of the disease in Japanese and Koreans and approximately 10% of familial occurrence among the Japanese. Recent genetic studies suggest some responsible genetic foci in chromosomes 3, 6 and 17.