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目的:总结血清肌酸磷酸激酶(creatinekinase,CK)增高的脊髓性肌萎缩(spinalmuscularatrophy,SMA)临床与肌电图的关系及临床意义。方法:收集10例CK增高的SMA病例,进行临床及肌电图分析,其中6例作了肌肉活检。结果:CK增高可见于脊髓性肌萎缩各型,升高的程度各有不同。肌电图检查除2例为混和性损害外,余均为神经源性损害。肌肉病理检查除1例为肌性改变外,余均为神经源性肌萎缩。结论:CK增高不能作为SMA与进行性肌营养不良的鉴别诊断指标,必须结合肌电图与肌肉活检。在诊断SMA时,当肌活检为肌性损害,反复多次的肌电图的结果更可靠;当肌电图结果不明确时,肌活检结果为确定诊断的最可靠依据。
OBJECTIVE: To summarize the relationship between clinicopathologic changes of spinal muscular atrophy (SMA) with serum creatine kinase (CK) and its clinical significance. Methods: Ten SMA patients with elevated CK were collected for clinical and electromyographic analysis. Six of them were biopsied. Results: Increased CK can be seen in all types of spinal muscular atrophy, elevated levels vary. Electromyography in addition to two cases of mixed damage, Yu are more than neurogenic damage. In addition to muscular pathological examination of 1 case of muscular changes, more than all of neurogenic muscle atrophy. Conclusion: Increased CK can not be used as a differential diagnosis index of SMA and progressive muscular dystrophy. EMG and muscle biopsy must be combined. In the diagnosis of SMA, when the muscle biopsy muscle damage, repeated EMG results more reliable; when the EMG results are not clear, muscle biopsy results confirm the diagnosis the most reliable basis.