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。
Objective To study
the clinical and myopathological features in the patients with myopathy associated with
disorder of lipid metabolism (DLM). Methods Muscle biopsies in 16 cases of DLM were
analyzed quantitatively. Results All these cases of DLM with progressive or fluctuating
weakness and fatigability, in which 8 cases showed exercise related muscle pain. EMG
presented myogenic features in 15 cases and it was normal in only one case. Abnormal ECG or
signs of fatty livers on sonography occurred in 6 cases. Muscle biopsies in all cases showed
much lipid storage in the muscle fibers composing (17±6)% of the mean muscle fiber area [the
mean normal area being (0.75±0.31)%]. The mean number of 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 the endothelial 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 showed ragged red fibers. After steroid and L carnitine therapy
the clinical symptoms in all the cases improved. Conclusions Our results suggested that
myopathy with the disorder of lipid metabolism might be a systemic disease.
出处
《中华神经科杂志》
CAS
CSCD
1999年第1期31-34,共4页
Chinese Journal of Neurology
关键词
脂质累积病
线粒体肌病
临床病理
Lipoidosis Muscular diseases Mitochondrial myopathies Carnitine
