[Mitochondrial angiopathy in the cerebral blood vessels of MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis and strokelike episodes)].
[Mitochondrial angiopathy in the cerebral blood vessels of MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis and strokelike episodes)].
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[MELAS脑血管中的线粒体血管病（线粒体肌病、脑病、乳酸性酸中毒和中风样发作）]。
DOI：
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发表时间：
1988
期刊：
影响因子：
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通讯作者：
Tadashi Miyatake
中科院分区：
文献类型：
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作者：
E. Ohama;S. Ohara;F. Ikuta;Keiko Tanaka;Masatoyo Nishizawa;Tadashi Miyatake
MELAS是一种独特的综合征，表现为线粒体肌病、脑病、乳酸酸中毒和复发性卒中样发作，如癫痫发作、交替性轻偏瘫、偏盲或皮质盲。在病理学上，这种疾病的特征是多发性、孤立性或连续性坏死灶（梗死或软化），大小和分期不同，主要累及双侧大脑皮质，其次累及大脑白色物质、基底节、脑干和大脑。病变的分布与血管区域不一致，表明它们不是由于通常的血栓形成或栓塞过程。这些具有特征性分布模式的病变的确切性质和发病机制仍有待阐明。我们系统地研究了脑血管从尸检的两个MELAS患者的电子显微镜。所有的主要脑动脉，包括大脑前、中、后动脉、基底动脉和椎动脉，在大脑基底部的近端部分和在皮质表面的外周部分以及脑实质内进行了检查。我们发现平滑肌细胞和内皮细胞的细胞体中线粒体明显积聚，并且图尼卡中膜中的许多平滑肌细胞显示出散在或成簇的变性或坏死。这些异常在直径达250 μ的软脑膜小动脉和小动脉的壁中最突出，在较大的软脑膜动脉和脑内小动脉和小动脉中不太频繁和严重。这些血管变化不同于已知涉及脑血管的各种疾病中描述的任何血管变化，因此是MELAS脑血管的特征。我们认为，这些特殊的血管变化称为线粒体血管病是由血管平滑肌细胞和内皮细胞本身的原发性线粒体功能障碍引起的，在这种疾病中，骨骼肌和心肌也是如此，并且它们构成了MELAS中具有异常分布模式和性质的脑病变的致病基础。
MELAS is a distinctive syndrome manifested by mitochondrial myopathy, encephalopathy, lactic acidosis, and recurrent stroke-like episodes such as seizures, alternating hemiparesis, hemianopsia, or cortical blindness. Pathologically the disorder is characterized by multiple, solitary or continuous foci of necrosis (infarct or softening), varying in size and stage, predominantly involving the bilateral cerebral cortices and to a lesser degree cerebral white matter, basal ganglia, brainstem and cereblum. The distribution of the lesions does not correspond to vascular territories, suggesting that they are not due to usual thrombotic or embolic process. The exact nature and pathogenesis of these lesions with characteristic distribution pattern remain to be elucidated. We studied systematically cerebral blood vessels from two autopsied patients with MELAS by electron microscopy. All the main cerebral arteries including anterior, middle and posterior cerebral, basilar and vertebral arteries were examined at their proximal portions at the cerebral base and at their peripheral portions at the cortical surface as well as within brain parenchyma. We found marked accumulation of mitochondria in the cell bodies of smooth muscle cells and endothelial cells and numerous smooth muscle cells showing degeneration or necrosis, sporadically or in clusters in the tunica media. These abnormalities were most prominent in the walls of pial arterioles and small arteries up to 250 mu in diameter, and less frequent and severe in the larger pial arteries and intracerebral arterioles and small arteries. These vascular changes are different from any of those described in various disorders known to involve the cerebral blood vessels and are thus characteristic to the cerebral blood vessels of MELAS. We think that these peculiar vascular changes called mitochondrial angiopathy are caused by primary mitochondrial dysfunction in the vascular smooth muscle cells and endothelial cells themselves, as is the same in the skeletal and cardiac muscles in this disease, and that they constitute the pathogenic base of the brain lesions with unusual distribution pattern and nature in MELAS.