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目的 分析脂质沉积性肌病 (L SM)的临床表现和病理特点及误诊原因。方法 对 11例经肌肉活检证实的 L SM患者进行临床和肌肉病理分析。结果 11例均表现近端肌无力和肌疲劳现象 ,其中 3例曾误诊为多发性肌炎 ,1例误诊为重症肌无力 ,1例发病 9年未确诊。 11例的肌肉活检显示肌纤维内出现大量的脂肪滴。结论 L SM是一组生化方面十分复杂的疾病 ,临床上以不能耐受运动和近端肌无力为主 ,病程可波动 ,部分可自发缓解 ,临床易误诊 ,肌肉病理检查有助于确诊
Objective To analyze the clinical manifestations, pathological features and causes of misdiagnosis of liposarcoma myopathy (L SM). Methods Clinical and muscle pathological analysis was performed on 11 L SM patients confirmed by muscle biopsy. Results All the 11 cases showed proximal muscle weakness and muscle fatigue, of which 3 cases had been misdiagnosed as polymyositis, 1 case misdiagnosed as myasthenia gravis, and 1 case had not been diagnosed for 9 years. A muscle biopsy of 11 cases showed a large amount of fat droplets in the muscle fibers. Conclusion L SM is a group of biochemical complex diseases. In clinical practice, it can not tolerate exercise and proximal muscular weakness. The course of disease may fluctuate, some may spontaneously alleviate, and clinical misdiagnosis and muscle pathology may be helpful for the diagnosis