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用光镜,透射与扫描电镜观察了7例已经血管造影和手术证实的Moyamoya病的脑动脉。2例取自颈内动脉末端闭塞部,管腔已被增生的结缔组织阻塞。它们的病理特征是:中膜平滑肌细胞和内弹力板广泛遭受破坏,平滑肌细胞变性、坏死,破坏残留的浓缩的细胞器和细胞成分散在于间质中,中膜变溥。内膜平滑肌细胞增生,亦遭同样破坏,说明平滑肌细胞的破坏是反复发生的。内弹力板极度屈曲,部分增厚,部分变薄、断裂、崩解。其他3例分别取自大脑中动脉M_2和M_5段,另2例取自脑底的侧支血管。它们的变化程度轻,且较局限,但病变性质相同。上述各种变化提示,Moyamoya病是一种以平滑肌细胞和内弹力板反复破坏为特征的进行性疾病。对这些变化的发生机制进行了讨论。
Seven cases of Moyamoya disease, cerebral arteries, which had been confirmed by angiography and surgery, were observed with light microscope, transmission electron microscope and scanning electron microscope. Two cases were taken from the terminal occlusion of the internal carotid artery and the lumen had been obstructed by the hyperplastic connective tissue. Their pathological features are extensive damage to the smooth muscle cells of the tunica media and the endoelastome, degeneration and necrosis of the smooth muscle cells, the destruction of residual concentrated organelles and cellular components scattered in the interstitium, the tunica media. Intimal smooth muscle cell proliferation, also suffered the same damage, indicating that the destruction of smooth muscle cells is repeated. Inner elastic plate extreme buckling, some thickening, some thinning, fracture, disintegration. The other three cases were taken from the M_2 and M_5 segments of the middle cerebral artery and the other two were from the collateral vessels in the brain. Their degree of change is light and more limited, but the nature of lesions is the same. These various changes suggest that Moyamoya disease is a progressive disease characterized by repeated destruction of smooth muscle cells and endplate. The mechanism of these changes is discussed.