Reichmann H
Department of Neurology, University of Würzburg, West Germany.
Neurology. 1988 Feb;38(2):244-9. doi: 10.1212/wnl.38.2.244.
Single-fiber analyses using a kinetic microphotometric method were performed on three patients with chronic progressive external ophthalmoplegia and proximal myopathy accompanied by a partial deficiency of cytochrome c oxidase. Two patients had subsarcolemmal accumulation of mitochondria (ragged-red fibers). Qualitative histochemical demonstration of cytochrome c oxidase showed a mosaic of fibers without detectable cytochrome c oxidase activity. Quantitative single fiber measurements in the patients' biopsies showed that the majority of the muscle fibers had decreased cytochrome c oxidase activity without selective involvement of a specific fiber type. Succinate dehydrogenase was measured and the ratio of the activities (succinate dehydrogenase/cytochrome c oxidase) was calculated. Normal muscle showed a ratio of about 2, whereas diseased muscle showed values between 10 and 20, due to a decrease in cytochrome c oxidase activity. Ragged-red fibers showed very low or undetectable cytochrome c oxidase activity.
采用动力学显微光度法对3例慢性进行性外眼肌麻痹伴近端肌病且伴有细胞色素c氧化酶部分缺乏的患者进行了单纤维分析。2例患者肌膜下有线粒体聚集（破碎红纤维）。细胞色素c氧化酶的定性组织化学显示纤维呈镶嵌状，无可检测到的细胞色素c氧化酶活性。对患者活检组织进行的定量单纤维测量表明，大多数肌纤维的细胞色素c氧化酶活性降低，且未选择性累及特定纤维类型。测量了琥珀酸脱氢酶并计算了其活性比值（琥珀酸脱氢酶/细胞色素c氧化酶）。正常肌肉的比值约为2，而患病肌肉的比值在10至20之间，这是由于细胞色素c氧化酶活性降低所致。破碎红纤维显示细胞色素c氧化酶活性非常低或无法检测到。
