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Acute Cortical Lesions in MELAS Syndrome: Anatomic Distribution, Symmetry, and Evolution

Acute Cortical Lesions in MELAS Syndrome: Anatomic Distribution, Symmetry, and Evolution ORIGINAL RESEARCH PEDIATRICS Acute Cortical Lesions in MELAS Syndrome: Anatomic Distribution, Symmetry, and Evolution K.D. Bhatia, P. Krishnan, H. Kortman, J. Klostranec, and T. Krings ABSTRACT BACKGROUND AND PURPOSE: Mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS) syndrome is a rare mitochondrial disorder affecting children and young adults. Stroke-like episodes are often associated with acute cortical lesions in the posterior cerebral cortex and are classically described as asymmetric and transient. In this study we assessed the ana- tomic distribution of acute cortical lesions, the incidence of symmetry, and the temporal evolution of lesions. MATERIALS AND METHODS: This was a retrospective cohort study of patients who had a confirmed genetic diagnosis of a patho- genic variant associated with MELAS and MR imaging performed at our center (2006–2018). Each MR imaging study was assessed for new lesions using T1, T2, FLAIR, DWI, ADC, and SWI. The anatomic location, symmetry, and temporal evolution of lesions were analyzed. RESULTS: Eight patients with the same pathogenic variant of MELAS (MT-TL1 m.3243A.G) with 31 MR imaging studies were included. Forty-one new lesions were identified in 17 of the studies (5 deep, 36 cortical). Cortical lesions most commonly affected the primary visual cortex, the middle-third http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American Journal of Neuroradiology American Journal of Neuroradiology

Acute Cortical Lesions in MELAS Syndrome: Anatomic Distribution, Symmetry, and Evolution

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Publisher
American Journal of Neuroradiology
Copyright
© 2020 by American Journal of Neuroradiology
ISSN
0195-6108
eISSN
1936-959X
DOI
10.3174/ajnr.A6325
Publisher site
See Article on Publisher Site

Abstract

ORIGINAL RESEARCH PEDIATRICS Acute Cortical Lesions in MELAS Syndrome: Anatomic Distribution, Symmetry, and Evolution K.D. Bhatia, P. Krishnan, H. Kortman, J. Klostranec, and T. Krings ABSTRACT BACKGROUND AND PURPOSE: Mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS) syndrome is a rare mitochondrial disorder affecting children and young adults. Stroke-like episodes are often associated with acute cortical lesions in the posterior cerebral cortex and are classically described as asymmetric and transient. In this study we assessed the ana- tomic distribution of acute cortical lesions, the incidence of symmetry, and the temporal evolution of lesions. MATERIALS AND METHODS: This was a retrospective cohort study of patients who had a confirmed genetic diagnosis of a patho- genic variant associated with MELAS and MR imaging performed at our center (2006–2018). Each MR imaging study was assessed for new lesions using T1, T2, FLAIR, DWI, ADC, and SWI. The anatomic location, symmetry, and temporal evolution of lesions were analyzed. RESULTS: Eight patients with the same pathogenic variant of MELAS (MT-TL1 m.3243A.G) with 31 MR imaging studies were included. Forty-one new lesions were identified in 17 of the studies (5 deep, 36 cortical). Cortical lesions most commonly affected the primary visual cortex, the middle-third

Journal

American Journal of NeuroradiologyAmerican Journal of Neuroradiology

Published: Jan 1, 2020

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