关键词: 线粒体肌病; 体层摄影术; X线计算机; 病理学

Abstract: Objective To explore the role of vasculopathy and ischemic events in the pathomechanism of mitochondrial encephalomyopathies.Methods Computed tomography perfusion imaging has been applied to the evaluation of brain perfusion during the symptomatic period of mitochondrial encephalomyopathies, and mitochondria structures of the blood vessels wall within muscle fibers were observed by pathological technique.Results 7 cases of different clinical types of mitochondrial encephalomyopathies were characterized by recurrent stroke-like episodes and seizures. Neuroimaging studies demonstrated uni- and bilaterallesions predominantly in the occipital and temporal-parietal lobes, in which areas cerebral blood flow and cerebral blood volume decreased, mean transit time and the time to peak were delayed on computed tomography perfusion imaging. Muscle pathology showed incontinuous or dark staining onvessel wall by succinic acid dehydrogenase staining. Electron microscopy of blood vessels has shown swelling and a striking increase in the number of mitochondria in the smooth muscle and endothelialcells.C onclusion Mitochondrial encephalomyopathies is a systemic vascular disease. Cerebral hypoperfusion and metabolic dysfunction may represent an important feature of the pathogenesis of the strokelike episodes.

Key words: 【Key Words】 Mitochondrial myopathies; Tomography; X-ray; Pathology