肌酸磷酸激酶增高的脊髓性肌萎缩症患者临床与肌电图分析

Analysis of clinical manifestation and EMG in patients with spinal muscular atrophy accompanied by elevation of CK levels

目的 :观察血清肌酸磷酸激酶 (CK)增高的脊髓性肌萎缩症 (SMA)患者临床与肌电图的关系。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例为混合性损害外 ,余 8例均为神经源性损害。肌肉活检除 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。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 observe the relationship between the clinical manifestation and EMG in patients with spinal muscular atrophy (SMA) accompanied by elevation of blood creatine kinase(CK) levels.Methods:Analysis of clinical manifestation and EMG was done in ten patients with SMA accompanied by elevation of CK levels,and muscle biopsy was performed in six of them.Results:The elevation of CK levels was noted in any type of SMA,the degree of elevation was various.EMG showed mixed damages in 2 patients and neurogenic damages in 8 ones.Muscle biopsy demonstrated neurogenic changes in all patients but myogenic changes in one.Conclusion:The elevation of blood CK levels could not be a index in the differential diagnosis of SMA,if the muscle biopsy found myogenic damages, repeative EMG recording might be more reliable.However,if the EMG findings were uncertain,the result of the muscle biopsy might be the most important evidence in the diagnosis of SMA.