莫合塔尔.吐尔逊 袁云 陈清棠 吴丽娟

    100034 北京医科大学第一医院神经内科(莫合塔尔.吐尔逊，现在新疆维吾尔自治区人民医院神经内科) 中华神经科杂志 1999 0 32 1


    关键词：脂质累积病；肌疾病；线粒体肌病；肉碱 期刊 zhsjkzz 0 论著 fur -->


    

【摘要】 目的 探讨脂质代谢性肌病的病理，讨论此病与线粒体细胞病的关系。方法 对16例经肌肉活检证实的脂质代谢性肌病进行临床和肌肉病理检查，对油红O染色片进行形态计量分析。结果 16例病人的肌肉活检显示肌纤维内出现大量的脂肪滴，5例出现不整红边纤维。脂肪滴所占肌纤维的平均面积为(17±6)%，肌纤维内脂肪滴颗粒平均数为237±37。电镜下脂肪滴在肌纤维内和间质细胞内也明显增多。在8例病人可见线粒体内类结晶样包涵体。结论 脂质代谢性肌病为全身系统性疾病。

    Clinical and pathological analysis of myopathy with disorder of lipid metabolismin 16 cases TUERXUN Mohetaer，YUAN Yun, CHEN Qingtang, et al. Departmentof Neurology, First Affiliated Hospital, Beijing Medical University, Beijing 100034

    【Abstract】 Objective To study the clinical andmyopathological features in the patients with myopathy associated with disorder of lipidmetabolism (DLM). Methods Muscle biopsies in 16 cases of DLM wereanalyzed quantitatively. Results All these cases of DLM withprogressive or fluctuating weakness and fatigability, in which 8 cases showed exerciserelated muscle pain. EMG presented myogenic features in 15 cases and it was normal in onlyone case. Abnormal ECG or signs of fatty livers on sonography occurred in 6 cases. Musclebiopsies in all cases showed much lipid storage in the muscle fibers composing (17±6)% ofthe mean muscle fiber area [the mean normal area being (0.75±0.31)%]. The mean numberof lipid granules in the muscle fibers was 237±37 on the cross sections of muscle fibers(the mean normal number being 90±54). Moreover, lipid storage was also found in theendothelial cells of the small vessels and fibroblasts were seen in a few cases.Paracrystaline inclusions in the mitochondria were detected in 8 cases. Five cases showedragged-red fibers. After steroid and L-carnitine therapy the clinical symptoms in all thecases improved. Conclusions Our results suggested that myopathy withthe disorder of lipid metabolism might be a systemic disease.

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