乳酸和丙酮酸最小运动量试验对线粒体肌病的诊断价值  

Diagnosis value of minimum exercise test of lactic acid and pyruvic acid for mitochondrial myopathy

作　　者：孙永安[1] 徐俊[2] 王燕华[1] 暴党振[1] 伍其专[1] 都爱莲[3] 

机构地区：[1]北京大学第一医院神经科,100034 [2]南京医科大学附属脑科医院神经内科 [3]浙江大学医学院附属第二医院神经内科

出　　处：《临床神经病学杂志》2009年第3期172-174,共3页Journal of Clinical Neurology

摘　　要：目的研究乳酸和丙酮酸最小运动量试验(METLP)对线粒体肌病的诊断价值。方法对肌肉病理明确诊断为线粒体肌病的21例患者、6例其他肌病患者及14例非肌病患者进行METLP检测,并对其检测结果进行分析。结果线粒体肌病组METLP 18例(85.7%)异常,12例≥2项指标异常,主要为运动后血浆乳酸/运动前丙酮酸(L2/P1)>17;其他肌病组METLP 6例(100%)异常,主要为有氧运动后血浆乳酸浓度(L2)升高,或者经休息其血浆乳酸浓度(L3)不降、甚至升高;非肌病组为3例(21.4%)异常。结论在METLP的各项评价指标中,L2/P1作为线粒体肌病与非肌病的诊断参考指标最为可靠;L3/L2可以作为鉴别线粒体肌病与其他肌病的可靠指标;但线粒体肌病的诊断应结合METLP、临床及病理综合分析。Objective To investigate the diagnosis value of minimum exercise test of lactic acid and pyruvic acid（METLP） for mitochondrial myopathy. Methods METLP were applied in 21 patients with mitochondrial myopathy, 6 patients with other myopathy and 14 patients without myopathy. All of them were diagnosed by muscle biopsy. The results of METLP were analyzed. Results The abnormal results of METLP were found in 18 （85.7%） patients with mitochondrial myopathy, including 12 cases had 2 or more abnormal indexes. The mainly abnormal index was the value of lactic acid post-exercise/ pyruvic acid pre-exercise （ L2/P1 ） 〉 17. The abnormal results of METLP were found in 6 （ 100% ） patients with other myopathy. Most of them showed a high concentration of lactic acid post-aerobic exercise（I2） ,and sustainable to after rest,or even more higher（ L3 ）. 3 （21.4%）patients without myopathy showed abnormal results of METLP. Conclusions In all the indexes of METLP, the value of L2/P1 is the reliable index for diagnosis mitochondrial myopathy or no-myopathy ; the value of L3/L2 is the most important index in differential diagnosis of mitochondrial myopathy and other myopathy. METLP plays a certain role in the diagnosis of the mitochondrial myopathy, but the clinical and pathological data ought to be combined.

关 键 词：线粒体肌病 乳酸 丙酮酸 

分 类 号：R746[医药卫生—神经病学与精神病学]