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  T2-Pseudonormalization and Microstructural Characterization in Advanced Stages of Late-infantile Metachromatic Leukodystrophy

Martin, P., Hagberg, G., Schultz, T., Harzer, K., Klose, U., Bender, B., et al. (2020). T2-Pseudonormalization and Microstructural Characterization in Advanced Stages of Late-infantile Metachromatic Leukodystrophy. Clinical Neuroradiology, Epub ahead. doi:10.1007/s00062-020-00975-2.

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Item Permalink: http://hdl.handle.net/21.11116/0000-0007-7570-7 Version Permalink: http://hdl.handle.net/21.11116/0000-0007-7571-6
Genre: Journal Article

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 Creators:
Martin, P, Author
Hagberg, GE1, 2, Author              
Schultz, T, Author
Harzer, K, Author
Klose, U, Author
Bender, B, Author              
Nägele, T, Author
Scheffler, K1, 2, Author              
Krägeloh-Mann , I, Author
Groeschel, S, Author
Affiliations:
1Department High-Field Magnetic Resonance, Max Planck Institute for Biological Cybernetics, Max Planck Society, ou_1497796              
2Max Planck Institute for Biological Cybernetics, Max Planck Society, ou_1497794              

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 Abstract: Purpose: T2-weighted signal hyperintensities in white matter (WM) are a diagnostic finding in brain magnetic resonance imaging (MRI) of patients with metachromatic leukodystrophy (MLD). In our systematic investigation of the evolution of T2-hyperintensities in patients with the late-infantile form, we describe and characterize T2-pseudonormalization in the advanced stage of the natural disease course. Methods: The volume of T2-hyperintensities was quantified in 34 MRIs of 27 children with late-infantile MLD (median age 2.25 years, range 0.5-5.2 years). In three children with the most advanced clinical course (age >4 years) and for whom the T2-pseudonormalization was the most pronounced, WM microstructure was investigated using a multimodal MRI protocol, including diffusion-weighted imaging, MR spectroscopy (MRS), myelin water fraction (MWF), magnetization transfer ratio (MTR), T1-mapping and quantitative susceptibility mapping. Results: T2-hyperintensities in cerebral WM returned to normal in large areas of 3 patients in the advanced disease stage. Multimodal assessment of WM microstructure in areas with T2-pseudonormalization revealed highly decreased values for NAA, neurite density, isotropic water, mean and radial kurtosis, MWF and MTR, as well as increased radial diffusivity. Conclusion: In late-infantile MLD patients, we found T2-pseudonormalization in WM tissue with highly abnormal microstructure characterizing the most advanced disease stage. Pathological hallmarks might be a loss of myelin, but also neuronal loss as well as increased tissue density due to gliosis and accumulated storage material. These results suggest that a multimodal MRI protocol using more specific microstructural parameters than T2-weighted sequences should be used when evaluating the effect of treatment trials in MLD.

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 Dates: 2020-11
 Publication Status: Published online
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 Identifiers: DOI: 10.1007/s00062-020-00975-2
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Title: Clinical Neuroradiology
Source Genre: Journal
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Pages: - Volume / Issue: Epub ahead Sequence Number: - Start / End Page: - Identifier: ISSN: 1869-1439