脂质沉积性肌病 (ｌｉｐｉｄｓｔｏｒａｇｅｍｙｏｐａｔｈｙ ,ＬＳＭ )是肌肉中长链脂肪酸代谢障碍 ,致脂质沉积在肌纤维中而引起的一组肌病 ,属于常染色体隐性遗传病。其病因较多 ,但以肉毒碱缺乏所致常见。 70年代初才被认识[1] 。该病较少见 ,国内报道尚少。本文对其临床表 Lipid deposition myopathy (lipidstoragemyopathy, LSM) is a group of myopathies caused by lipid metabolism of long chain fatty acids in the muscle caused by lipid deposition in muscle fibers, is an autosomal recessive disease. The etiology more, but due to the lack of carnitine common. The early 70s was recognized [1]. The disease is rare, there are still few domestic reports. This article on its clinical form