脂肪累积肌肉病的病理和肌电图改变之间的关系

目的探讨脂肪累积性肌肉病和线粒体脂肪累积性肌肉病的肌肉活检病理改变和肌电图改变之间的关系。方法对33例肌活检确诊患者的肌肉病理改变分为单纯脂肪累积性肌肉病和线粒体-脂肪累积性肌肉病两组,对比两者肌纤维内脂肪沉积的程度以及肌电图改变的差异。结果33.3%为线粒体-脂肪累积性肌肉病,66.7%为单纯脂肪累积性肌肉病。前者肌纤维内脂肪沉积程度显著,7例出现小组样分布的小角状萎缩的肌纤维,后者脂肪沉积的程度相对轻,只有3例患者出现小角状萎缩的肌纤维。在前者81.8%的患者肌电图为肌源性损害、18.2%为混合性损害、45.5%合并周围神经损害。在后者59.1%的患者出现肌源性损害、13.6%出现混合或神经源损害、27.3%肌电图正常、22.7%合并周围神经损害。结论和单纯脂肪累积性肌肉病比较,线粒体-脂肪累积性肌肉病的脂肪滴沉积程度更显著以及更多的患者出现肌纤维萎缩,更易导致骨骼肌的电生理改变以及合并周围神经损害。

The relation of myopathological and electromyographical changes in lipid storage myopathy

Objective Lipid storage myopathy is not a rare neuromuscular disorders characterized by lipid accumulation in muscle fibers.Upon the appearance of ragged red fiber,the disease divided into pure lipid storage myopathy and mitochondrial-lipid storage myopathy.Here we compared the EMG and pathological changes in the two group.Methods This study retained 33 cases,morphologically diagnosed as lipid storage myopathy,divided into pure lipid storage myopathy and mitochondrial-lipid storage myopathy,in which we study the relation between the degree of lipid accumulation in ORO staining and EMG changes.Results 33.3% of all patients were mitochondrial-lipid storage myopathy,which have much more lipid in muscle fibers and angular fiber atrophy than the pure lipid storage myopathy.Another 66.7% were pure lipid storage myopathy with less lipid storage in fibers.In mitochondrial-lipid storage myopathy,myogenic changes was noted in 81.8% cases,mixed lesion in 18.2%,with peripheral nerve lesion in 45.5%.In pure lipid storage myopathy,myogenic change appeared in 59.1% cases,normal in 27.3%,neurogenic or mixed lesion in 13.6%,with peripheral nerve lesion in 22.7%.Conclusions Compared with pure lipid storage myopathy.Mitochondrial-lipid storage myopathy manifests more lipid storage in muscle,and more easy to cause EMG changes and peripheral nerve injury.