Kondo S, Tanaka M, Osawa T, Okamoto K, Hirai S
Department of Neurology, Gunma University School of Medicine.
Rinsho Shinkeigaku. 1993 Oct;33(10):1033-8.
In order to study the cerebral circulation and metabolism in 6 patients with mitochondrial myopathies and 6 normal control subjects, positron emission tomography with oxygen-15 labelled tracers was performed. Four patients had predominantly central nervous system involvement, whereas 2 had myopathy alone. In comparison with normal control subjects, patients with mitochondrial myopathies showed decreased cerebral blood flow (CBF), decreased cerebral oxygen metabolic rate (CMRO2), and low oxygen extraction fraction (OEF) value due to an uncoupling of CBF and CMRO2. The reduction of OEF was remarkable in the 2 patients having predominantly central nervous disease and 1 having myopathy alone. We suppose that these findings are greatly related to mitochondrial myopathies and that they reflect the disturbance of mitochondrial oxidative metabolism in the brain. The patients whose central nervous symptoms progressed rapidly tended to show the reduction of OEF, but the degree of the reduction of OEF was not necessarily related to the severity of the central nervous symptoms. These findings suggest that the disturbance of cerebral metabolism is not uniform in patients with the same central nervous disease.
为研究6例线粒体肌病患者及6名正常对照者的脑循环和代谢情况，采用氧-15标记示踪剂进行正电子发射断层扫描。4例患者主要累及中枢神经系统，2例仅患肌病。与正常对照者相比，线粒体肌病患者脑血流量（CBF）降低、脑氧代谢率（CMRO2）降低，且由于CBF与CMRO2解偶联导致氧摄取分数（OEF）值较低。在2例主要患有中枢神经疾病的患者和1例仅患肌病的患者中，OEF的降低较为显著。我们推测这些发现与线粒体肌病密切相关，且反映了大脑线粒体氧化代谢的紊乱。中枢神经症状进展迅速的患者往往表现出OEF降低，但OEF降低的程度不一定与中枢神经症状的严重程度相关。这些发现表明，患有相同中枢神经疾病的患者脑代谢紊乱并不一致。
