4.2 Article

MR spectroscopy of the brain in Leigh syndrome

期刊

BRAIN & DEVELOPMENT
卷 30, 期 9, 页码 579-583

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ELSEVIER SCIENCE BV
DOI: 10.1016/j.braindev.2008.01.011

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Leigh syndrome; magnetic resonance spectroscopy; mitochondrial diseases

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Brain magnetic resonance spectroscopy in two patients with Leigh syndrome revealed the presence of lactate in gray and white matter brain tissue and relatively high choline levels in the white matter. The latter observation, most probably related to an ongoing demyefination process, underlines specific involvernent or white matter metabolism in Leigh syndrome even in cases without involvement of the white matter as visualized on MR1. Magnetic resonance spectroscopy might thus be of help in differentiating Leigh syndrome from a ranee of other mitochondrial diseases, such as ophthalmoplegia and Kearns-Sayre syndrome, showing lack of lactate in brain tissues appearing normal on MR1. (c) 2008 Elsevier B.V. All rights reserved.

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