辅酶Q10治疗线粒体肌病的临床与生化相关性

Clinical and biochemical correlations in mitochondrial myopathies treated with coenzyme Q10.

作者信息

Bresolin N, Bet L, Binda A, Moggio M, Comi G, Nador F, Ferrante C, Carenzi A, Scarlato G

机构信息

Istituto di Clinica Neurologica, Centro Dino Ferrari, Università degli Studi di Milano, Italy.

出版信息

Neurology. 1988 Jun;38(6):892-9. doi: 10.1212/wnl.38.6.892.

DOI:10.1212/wnl.38.6.892

PMID:3368070

Abstract

We tested the efficacy of coenzyme Q10 (ubidecarenone, CoQ10) therapy in patients with Kearns-Sayre syndrome and other mitochondrial myopathies with chronic progressive external ophthalmoplegia (CPEO). We treated seven patients for 1 year with daily oral administration of 120 mg of CoQ10. Throughout the treatment most of our patients showed a progressive reduction of serum lactate and pyruvate levels following standard muscle exercise and generally improved neurologic functions. The ECG and echocardiogram showed no significant changes in our patients. None of our patients showed any improvement in ptosis and CPEO.

摘要

我们测试了辅酶Q10（泛癸利酮，CoQ10）治疗患有卡恩斯-塞尔综合征及其他伴有慢性进行性眼外肌麻痹（CPEO）的线粒体肌病患者的疗效。我们对7例患者进行了为期1年的治疗，每日口服120毫克CoQ10。在整个治疗过程中，我们的大多数患者在标准肌肉运动后血清乳酸和丙酮酸水平逐渐降低，神经功能总体改善。心电图和超声心动图显示我们的患者无显著变化。我们的患者中没有一例上睑下垂和CPEO有任何改善。