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These elementary MRI abnormalities can be variably combined in the single patient, often beyond what can be expected based on the classically known clinical-pathological patterns. However, a normal brain MRI is also possible. 1HMRS has a diagnostic role in patients with suspected mitochondrial encephalopathy, especially in the acute phase, as it can detect within the lesions, but also in normal appearing nervous tissue or in the ventricular cerebrospinal fluid (CSF), an abnormally prominent lactate peak, reflecting failure of the respiratory chain with a shift from the Krebs cycle to anaerobic glycolysis. So far, studies correlating MRI findings with genotype in mitochondrial disease have been possible only in small samples and would greatly benefit from data pooling. MRI and 1HMRS have provided important information on the pathophysiology of CNS damage in mitochondrial diseases by enabling in vivo non-invasive assessment of tissue abnormalities, the associated changes of blood perfusion and cellular metabolic derangement. MRI and 1HMRS are expected to serve as surrogate biomarkers in trials investigating therapeutic options in mitochondrial disease.<\/jats:p>","DOI":"10.1042\/ebc20170109","type":"journal-article","created":{"date-parts":[[2018,7,20]],"date-time":"2018-07-20T10:35:16Z","timestamp":1532082916000},"page":"409-421","update-policy":"https:\/\/doi.org\/10.1042\/crossmark_policy","source":"Crossref","is-referenced-by-count":38,"title":["Neuroimaging in mitochondrial disorders"],"prefix":"10.1042","volume":"62","author":[{"given":"Mario","family":"Mascalchi","sequence":"additional","affiliation":[{"name":"Neuroradiology Research Unit, Neuroscience Department, A Meyer Children\u2019s Hospital, University of Florence, Florence, Italy"},{"name":"Department of Experimental and Clinical Biomedical Sciences \u2018Mario Serio\u2019, University of Florence, Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martino","family":"Montomoli","sequence":"additional","affiliation":[{"name":"Pediatric Neurology, Neurogenetics and Neurobiology Unit and Laboratories, Neuroscience Department, A Meyer Children\u2019s Hospital, University of Florence, Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Renzo","family":"Guerrini","sequence":"additional","affiliation":[{"name":"Pediatric Neurology, Neurogenetics and Neurobiology Unit and Laboratories, Neuroscience Department, A Meyer Children\u2019s Hospital, University of Florence, Florence, Italy"},{"name":"IRCCS Stella Maris Foundation, Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"288","published-online":{"date-parts":[[2018,7,20]]},"reference":[{"key":"2021111610261888300_B1","doi-asserted-by":"publisher","first-page":"309","DOI":"10.1016\/j.jns.2016.02.008","article-title":"FDG-PET study of patients with Leigh syndrome","volume":"362","author":"Haginoya","year":"2016","journal-title":"J. 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