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近年来有关线粒体脑肌病伴乳酸酸中毒及卒中样发作(MELAS)的临床和病理报告提出,此病脑循环及代谢的异常均为卒中样发作的表现。本研究目的是应用经颅多普勒超声(TCD)评价线粒体脑肌病(MEM)患者血液动力学及脑血管贮备力。 研究包括13例MEM患者(男8例,女5例),来自Osaka医科大学儿科门诊(年龄5岁～23岁),6例诊断为MELAS,4例为细胞色素C氧化酶缺乏(CCOD),1例为非对称红纤维肌痉挛(MERRF),1例诊断不明。对照组6例(男4例,女2例;年龄5岁～12岁)均无永久性神经功能障碍或神经影像方面异常。受试者临床
In recent years, the clinical and pathological report about mitochondrial encephalomyopathy associated with lactic acidosis and stroke-like episodes (MELAS) suggested that the abnormalities of cerebral circulation and metabolism in this disease are the manifestation of stroke-like episodes. The purpose of this study was to evaluate hemodynamics and cerebral vascular reserve in patients with mitochondrial encephalomyopathy (MEM) by transcranial Doppler ultrasound (TCD). The study included 13 patients with MEM (8 males and 5 females) from the Pediatric Outpatient Department of Osaka Medical University (age 5 to 23 years), 6 MELAS, 4 CCOD, 1 case of asymmetric red muscle spasm (MERRF), 1 case of unknown diagnosis. Six patients in the control group (4 males and 2 females; aged 5 to 12 years) had no permanent neurological dysfunction or neuroimaging abnormalities. Subjects clinically