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. 2021 Jan 23;268(6):2192–2207. doi: 10.1007/s00415-020-10390-9

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© The Author(s) 2021

Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/.

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Fig. 1 — CT and MRI brain of MELAS patients with nuclear gene mutations. Axial sections of CT and MRI of brain show shifting cortical-based signal changes that do not conform any arterial territory in patient P8 (A–F). CT brain shows an ill-defined focal hypodensity in the left parietal region in 2008 (a), Brain MRI shows focal wedge-shaped hyperintensity in the left temporal region resembling an ‘infarct’ in 2010 (b, c), focal hyperintensity in the right frontal region in 2012, while the lesion seen in the left temporal region in 2010 has resolved completely (d, e) and recurrence of focal hyperintensity in the left temporal region in 2014 (f). Axial sections of MRI of brain show shifting hyperintensities involving the cortex that do not conform an arterial territory in patient P9 (g–j). Brain MRI shows focal hyperintensity in the left parieto-occipital region in September 2012 (g), focal hyperintensity in the right temporal region in November 2012 (h), focal hyperintensity in left medial temporal region in January 2013 (i) and hyperintensity in bilateral frontal region in August 2016 (j)