Deep white matter in Huntington's disease.

White matter (WM) abnormalities have already been shown in presymptomatic (Pre-HD) and symptomatic HD subjects using Magnetic Resonance Imaging (MRI). In the present study, we examined the microstructure of the long-range large deep WM tracts by applying two different MRI approaches: Diffusion Tenso...

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Autores principales: Owen Phillips, Ferdinando Squitieri, Cristina Sanchez-Castaneda, Francesca Elifani, Carlo Caltagirone, Umberto Sabatini, Margherita Di Paola
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Publicado: Public Library of Science (PLoS) 2014
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spelling oai:doaj.org-article:9d4de6da340b4816bd5c1de732d518da2021-11-25T05:55:26ZDeep white matter in Huntington's disease.1932-620310.1371/journal.pone.0109676https://doaj.org/article/9d4de6da340b4816bd5c1de732d518da2014-01-01T00:00:00Zhttps://doi.org/10.1371/journal.pone.0109676https://doaj.org/toc/1932-6203White matter (WM) abnormalities have already been shown in presymptomatic (Pre-HD) and symptomatic HD subjects using Magnetic Resonance Imaging (MRI). In the present study, we examined the microstructure of the long-range large deep WM tracts by applying two different MRI approaches: Diffusion Tensor Imaging (DTI) -based tractography, and T2*weighted (iron sensitive) imaging. We collected Pre-HD subjects (n = 25), HD patients (n = 25) and healthy control subjects (n = 50). Results revealed increased axial (AD) and radial diffusivity (RD) and iron levels in Pre-HD subjects compared to controls. Fractional anisotropy decreased between the Pre-HD and HD phase and AD/RD increased and although impairment was pervasive in HD, degeneration occurred in a pattern in Pre-HD. Furthermore, iron levels dropped for HD patients. As increased iron levels are associated with remyelination, the data suggests that Pre-HD subjects attempt to repair damaged deep WM years before symptoms occur but this process fails with disease progression.Owen PhillipsFerdinando SquitieriCristina Sanchez-CastanedaFrancesca ElifaniCarlo CaltagironeUmberto SabatiniMargherita Di PaolaPublic Library of Science (PLoS)articleMedicineRScienceQENPLoS ONE, Vol 9, Iss 10, p e109676 (2014)
institution DOAJ
collection DOAJ
language EN
topic Medicine
R
Science
Q
spellingShingle Medicine
R
Science
Q
Owen Phillips
Ferdinando Squitieri
Cristina Sanchez-Castaneda
Francesca Elifani
Carlo Caltagirone
Umberto Sabatini
Margherita Di Paola
Deep white matter in Huntington's disease.
description White matter (WM) abnormalities have already been shown in presymptomatic (Pre-HD) and symptomatic HD subjects using Magnetic Resonance Imaging (MRI). In the present study, we examined the microstructure of the long-range large deep WM tracts by applying two different MRI approaches: Diffusion Tensor Imaging (DTI) -based tractography, and T2*weighted (iron sensitive) imaging. We collected Pre-HD subjects (n = 25), HD patients (n = 25) and healthy control subjects (n = 50). Results revealed increased axial (AD) and radial diffusivity (RD) and iron levels in Pre-HD subjects compared to controls. Fractional anisotropy decreased between the Pre-HD and HD phase and AD/RD increased and although impairment was pervasive in HD, degeneration occurred in a pattern in Pre-HD. Furthermore, iron levels dropped for HD patients. As increased iron levels are associated with remyelination, the data suggests that Pre-HD subjects attempt to repair damaged deep WM years before symptoms occur but this process fails with disease progression.
format article
author Owen Phillips
Ferdinando Squitieri
Cristina Sanchez-Castaneda
Francesca Elifani
Carlo Caltagirone
Umberto Sabatini
Margherita Di Paola
author_facet Owen Phillips
Ferdinando Squitieri
Cristina Sanchez-Castaneda
Francesca Elifani
Carlo Caltagirone
Umberto Sabatini
Margherita Di Paola
author_sort Owen Phillips
title Deep white matter in Huntington's disease.
title_short Deep white matter in Huntington's disease.
title_full Deep white matter in Huntington's disease.
title_fullStr Deep white matter in Huntington's disease.
title_full_unstemmed Deep white matter in Huntington's disease.
title_sort deep white matter in huntington's disease.
publisher Public Library of Science (PLoS)
publishDate 2014
url https://doaj.org/article/9d4de6da340b4816bd5c1de732d518da
work_keys_str_mv AT owenphillips deepwhitematterinhuntingtonsdisease
AT ferdinandosquitieri deepwhitematterinhuntingtonsdisease
AT cristinasanchezcastaneda deepwhitematterinhuntingtonsdisease
AT francescaelifani deepwhitematterinhuntingtonsdisease
AT carlocaltagirone deepwhitematterinhuntingtonsdisease
AT umbertosabatini deepwhitematterinhuntingtonsdisease
AT margheritadipaola deepwhitematterinhuntingtonsdisease
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