摘要
目的:探讨脂质沉积性肌病(LSM)临床特点、误诊原因及诊治。方法:分析21例LSM的临床特点、神经电生理、肌肉病理资料,并复习相关文献。结果:21例均表现为四肢近端无力、肌酶升高,肌电图呈肌源性损害或合并神经源性损害,或无异常,12例被误诊为多发性肌炎、病毒性心肌炎、重症肌无力等,经病理证实为LSM。结论:LSM临床表现缺乏特异性,临床容易误诊,病理检查是诊断该病的主要依据。
Objective：To study the clinical and pathological features of misdiagnosised lipid storage myopa-thy（LSM ） ,and further discover the reasons of diagnosis error in this disease .Methods：The clinical data ,the results of neuroelectrophysiological and pathology of the 21 patients were analyzed and the related documents were reviewed .Results ：The 21 patients who presented myasthenia with proximal of extremities ,a rise of creatase in serum . The neuro-electrophysiological changes varied ,which could be neurogenetic changes combined with muscular abnormalities ,as well as the 12 patients were once mistakenly diagnosed as polymyositis ,limbgirdle muscular dystrophy and myasthenia gravis .They were confirmed to be LSM by pathology .Conclusion：The clinical manifestations of LSM are nonspecifie muscle weakness which should be differentiate with related myopathy ,the definite diagnosis is de-pendent on pathological examination .
出处
《陕西医学杂志》
CAS
2014年第7期804-806,共3页
Shaanxi Medical Journal
