Division of Endocrinology, Diabetology and Metabolism, Department of Internal Medicine, Molinette Hospital, University of Turin, C.so Dogliotti 14, 10126 Turin, Italy.
Eur J Endocrinol. 2011 Jun;164(6):985-93. doi: 10.1530/EJE-10-1169. Epub 2011 Mar 14.
Glucocorticoids are known to decrease protein synthesis and conduction velocity of muscle fibers. However, the degree of impairment of muscle protein synthesis and conduction slowing in patients with Cushing's disease remains poorly characterized. Our objective was to investigate whether and to what extent chronic endogenous hypercortisolism could decrease the circulating levels of muscle proteins and modify myoelectric indexes of sarcolemmal excitability and fatigability.
A total of ten patients with Cushing's disease and 30 healthy controls matched for age, sex, and body mass index were compared.
Blood sampling and electrophysiological tests on vastus lateralis, vastus medialis, and tibialis anterior muscles were performed.
Serum creatine kinase (CK) and plasma myoglobin were significantly lower in patients with respect to controls (P<0.001 and P<0.05 respectively): the mean relative difference between patients and controls was 48.9% for CK and 21.4% for myoglobin. Muscle fiber conduction velocity (MFCV) and myoelectric manifestations of fatigue were significantly decreased in all muscles of the patients with respect to controls. The mean relative difference in MFCV between patients and controls was 26.0% for vastus lateralis, 22.9% for vastus medialis, and 11.6% for tibialis anterior. These differences contrasted with the paucity of signs suggestive of myopathy that were obtained by needle electromyography in the patients.
Slowing of muscle fiber conduction and decreased levels of circulating muscle proteins are sensitive markers of impaired muscle function, which are suitable for use in combination with clinical assessment and standard electrodiagnostic tests for accurate identification and follow-up of myopathic patients.
已知糖皮质激素可降低肌纤维的蛋白质合成和传导速度。然而,库欣病患者的肌肉蛋白质合成受损程度和传导速度减慢仍知之甚少。我们的目的是研究内源性皮质醇过多是否以及在何种程度上可以降低循环肌肉蛋白水平,并改变肌细胞膜兴奋性和易疲劳性的肌电图指标。
总共比较了 10 例库欣病患者和 30 例年龄、性别和体重指数匹配的健康对照者。
对股外侧肌、股直肌和胫骨前肌进行血液采样和电生理测试。
与对照组相比,患者的血清肌酸激酶(CK)和血浆肌红蛋白明显降低(P<0.001 和 P<0.05):CK 的患者与对照组的平均相对差异为 48.9%,肌红蛋白为 21.4%。与对照组相比,患者的所有肌肉的肌纤维传导速度(MFCV)和肌电疲劳表现均明显降低。股外侧肌、股直肌和胫骨前肌的 MFCV 患者与对照组的平均相对差异分别为 26.0%、22.9%和 11.6%。这些差异与患者针电极肌电图检查获得的肌病体征稀少形成对比。
肌纤维传导速度减慢和循环肌肉蛋白水平降低是肌肉功能受损的敏感标志物,可与临床评估和标准电诊断测试结合使用,以准确识别和随访肌病患者。