Ohama E, Ikuta F
Department of Pathology, Brain Research Institute, Niigata University, Japan.
Acta Neuropathol. 1987;75(1):1-7. doi: 10.1007/BF00686785.
Morphological study of the choroid plexuses of two patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) revealed an enormously increased number of mitochondria with structural abnormalities in almost all of the choroidal epithelial cells. The majority of the cells also showed loss of microvilli and collapsed or attenuated apical cytoplasmic processes with increased electron density and lysosome-like dense bodies. The blood vessels of the choroid plexus showed the features of mitochondrial angiopathy previously described in the pial arteries of the same patients. These findings are interpreted as the morphological expression of a primary biochemical defect of the mitochondrial function in the choroid plexus, and as the probable explanation for increased CSF lactate and pyruvate levels in this disease.
对两名患有线粒体肌病、脑病、乳酸性酸中毒和卒中样发作（MELAS）患者的脉络丛进行的形态学研究显示，几乎所有脉络丛上皮细胞中的线粒体数量大幅增加且结构异常。大多数细胞还表现出微绒毛缺失，顶端细胞质突起塌陷或变细，电子密度增加以及出现溶酶体样致密小体。脉络丛的血管呈现出先前在同一患者软脑膜动脉中所描述的线粒体血管病特征。这些发现被解释为脉络丛中线粒体功能原发性生化缺陷的形态学表现，也是该疾病中脑脊液乳酸和丙酮酸水平升高的可能原因。
