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The metabolite ratios of lactate (lac)\/Cr, N\u2010acetyl aspartate (<jats:styled-content style=\"fixed-case\">NAA<\/jats:styled-content>)\/creatine (Cr), total choline (<jats:styled-content style=\"fixed-case\">tC<\/jats:styled-content>ho)\/Cr, and myoinositol (<jats:styled-content style=\"fixed-case\">mI<\/jats:styled-content>)\/Cr are measured in the infarct\u2010like lesions by <jats:sup>1<\/jats:sup>H\u2010<jats:styled-content style=\"fixed-case\">MRS<\/jats:styled-content> and may reveal metabolic changes associated with <jats:styled-content style=\"fixed-case\">ME<\/jats:styled-content>. However, the application of this molecular imaging technique in the investigation of the pathology of <jats:styled-content style=\"fixed-case\">ME<\/jats:styled-content> subtypes is unknown.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>In this study, cerebral metabolic features of pathologically diagnosed <jats:styled-content style=\"fixed-case\">ME<\/jats:styled-content> cases, that is, 19 mitochondrial encephalomyopathy, lactic acidosis, and stroke\u2010like episodes (<jats:styled-content style=\"fixed-case\">MELAS<\/jats:styled-content>); nine chronic progressive external ophthalmoplegia (<jats:styled-content style=\"fixed-case\">CPEO<\/jats:styled-content>); and 23 healthy controls, were investigated using <jats:sup>1<\/jats:sup>H\u2010<jats:styled-content style=\"fixed-case\">MRS<\/jats:styled-content>. Receiver operating characteristics (<jats:styled-content style=\"fixed-case\">ROC<\/jats:styled-content>) analysis was used to evaluate the diagnostic power of the cerebral metabolites. Histochemical evaluation was carried out on muscle tissues derived from biopsy to assess the abnormal mitochondrial proliferation. The association between cerebral metabolic and mitochondrial cytopathy was examined by correlation analysis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Patients with <jats:styled-content style=\"fixed-case\">MELAS<\/jats:styled-content> or <jats:styled-content style=\"fixed-case\">CPEO<\/jats:styled-content> exhibited a significantly higher Lac\/Cr ratio and a lower <jats:styled-content style=\"fixed-case\">NAA<\/jats:styled-content>\/Cr ratio compared with controls. The <jats:styled-content style=\"fixed-case\">ROC<\/jats:styled-content> curve of Lac\/Cr ratio indicated prominent discrimination between <jats:styled-content style=\"fixed-case\">MELAS<\/jats:styled-content> or <jats:styled-content style=\"fixed-case\">CPEO<\/jats:styled-content> and healthy control subjects, whereas the <jats:styled-content style=\"fixed-case\">NAA<\/jats:styled-content>\/Cr ratio may present diagnostic power in the distinction of <jats:styled-content style=\"fixed-case\">MELAS<\/jats:styled-content> from <jats:styled-content style=\"fixed-case\">CPEO<\/jats:styled-content>. Lower <jats:styled-content style=\"fixed-case\">NAA<\/jats:styled-content>\/Cr ratio was associated with higher Lac\/Cr in <jats:styled-content style=\"fixed-case\">MELAS<\/jats:styled-content>, but not in <jats:styled-content style=\"fixed-case\">CPEO<\/jats:styled-content>. Furthermore, higher ragged\u2010red fibers (<jats:styled-content style=\"fixed-case\">RRF<\/jats:styled-content>s) percentages were associated with elevated Lac\/Cr and reduced <jats:styled-content style=\"fixed-case\">NAA<\/jats:styled-content>\/Cr ratios, notably in <jats:styled-content style=\"fixed-case\">MELAS<\/jats:styled-content>. This association was not noted in the case of <jats:styled-content style=\"fixed-case\">mI<\/jats:styled-content>\/Cr ratio.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Mitochondrial cytopathy (lactic acidosis and <jats:styled-content style=\"fixed-case\">RRF<\/jats:styled-content>s on muscle biopsy) was associated with neuronal viability but not glial proliferation, notably in <jats:styled-content style=\"fixed-case\">MELAS<\/jats:styled-content>. Mitochondrial neuronopathy and neuronal vulnerability are considered significant causes in the pathogenesis of <jats:styled-content style=\"fixed-case\">MELAS<\/jats:styled-content>, particularly with regard to stroke\u2010like episodes.<\/jats:p><\/jats:sec>","DOI":"10.1111\/cns.12714","type":"journal-article","created":{"date-parts":[[2017,7,11]],"date-time":"2017-07-11T06:42:39Z","timestamp":1499755359000},"page":"686-697","source":"Crossref","is-referenced-by-count":16,"title":["Mitochondrial dysfunction and cerebral metabolic abnormalities in patients with mitochondrial encephalomyopathy subtypes: Evidence from proton <scp>MR<\/scp> spectroscopy and muscle biopsy"],"prefix":"10.1111","volume":"23","author":[{"given":"Feng\u2010Nan","family":"Niu","sequence":"first","affiliation":[{"name":"Department of Pathology Drum Tower Hospital Medical School of Nanjing University Nanjing Jiangsu 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