Progression of brain atrophy in spinocerebellar ataxia type 2: a longitudinal tensor-based morphometry study.

Spinocerebellar ataxia type 2 (SCA2) is the second most frequent autosomal dominant inherited ataxia worldwide. We investigated the capability of magnetic resonance imaging (MRI) to track in vivo progression of brain atrophy in SCA2 by examining twice 10 SCA2 patients (mean interval 3.6 years) and 1...

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Autores principales:	Mario Mascalchi, Stefano Diciotti, Marco Giannelli, Andrea Ginestroni, Andrea Soricelli, Emanuele Nicolai, Marco Aiello, Carlo Tessa, Lucia Galli, Maria Teresa Dotti, Silvia Piacentini, Elena Salvatore, Nicola Toschi
Formato:	article
Lenguaje:	EN
Publicado:	Public Library of Science (PLoS) 2014
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/2e37afab5d904653b25abca50ec7e6f2
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spelling	oai:doaj.org-article:2e37afab5d904653b25abca50ec7e6f22021-11-18T08:31:09ZProgression of brain atrophy in spinocerebellar ataxia type 2: a longitudinal tensor-based morphometry study.1932-620310.1371/journal.pone.0089410https://doaj.org/article/2e37afab5d904653b25abca50ec7e6f22014-01-01T00:00:00Zhttps://www.ncbi.nlm.nih.gov/pmc/articles/pmid/24586758/pdf/?tool=EBIhttps://doaj.org/toc/1932-6203Spinocerebellar ataxia type 2 (SCA2) is the second most frequent autosomal dominant inherited ataxia worldwide. We investigated the capability of magnetic resonance imaging (MRI) to track in vivo progression of brain atrophy in SCA2 by examining twice 10 SCA2 patients (mean interval 3.6 years) and 16 age- and gender-matched healthy controls (mean interval 3.3 years) on the same 1.5 T MRI scanner. We used T1-weighted images and tensor-based morphometry (TBM) to investigate volume changes and the Inherited Ataxia Clinical Rating Scale to assess the clinical deficit. With respect to controls, SCA2 patients showed significant higher atrophy rates in the midbrain, including substantia nigra, basis pontis, middle cerebellar peduncles and posterior medulla corresponding to the gracilis and cuneatus tracts and nuclei, cerebellar white matter (WM) and cortical gray matter (GM) in the inferior portions of the cerebellar hemisphers. No differences in WM or GM volume loss were observed in the supratentorial compartment. TBM findings did not correlate with modifications of the neurological deficit. In conclusion, MRI volumetry using TBM is capable of demonstrating the progression of pontocerebellar atrophy in SCA2, supporting a possible role of MRI as biomarker in future trials.Mario MascalchiStefano DiciottiMarco GiannelliAndrea GinestroniAndrea SoricelliEmanuele NicolaiMarco AielloCarlo TessaLucia GalliMaria Teresa DottiSilvia PiacentiniElena SalvatoreNicola ToschiPublic Library of Science (PLoS)articleMedicineRScienceQENPLoS ONE, Vol 9, Iss 2, p e89410 (2014)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q Mario Mascalchi Stefano Diciotti Marco Giannelli Andrea Ginestroni Andrea Soricelli Emanuele Nicolai Marco Aiello Carlo Tessa Lucia Galli Maria Teresa Dotti Silvia Piacentini Elena Salvatore Nicola Toschi Progression of brain atrophy in spinocerebellar ataxia type 2: a longitudinal tensor-based morphometry study.
description	Spinocerebellar ataxia type 2 (SCA2) is the second most frequent autosomal dominant inherited ataxia worldwide. We investigated the capability of magnetic resonance imaging (MRI) to track in vivo progression of brain atrophy in SCA2 by examining twice 10 SCA2 patients (mean interval 3.6 years) and 16 age- and gender-matched healthy controls (mean interval 3.3 years) on the same 1.5 T MRI scanner. We used T1-weighted images and tensor-based morphometry (TBM) to investigate volume changes and the Inherited Ataxia Clinical Rating Scale to assess the clinical deficit. With respect to controls, SCA2 patients showed significant higher atrophy rates in the midbrain, including substantia nigra, basis pontis, middle cerebellar peduncles and posterior medulla corresponding to the gracilis and cuneatus tracts and nuclei, cerebellar white matter (WM) and cortical gray matter (GM) in the inferior portions of the cerebellar hemisphers. No differences in WM or GM volume loss were observed in the supratentorial compartment. TBM findings did not correlate with modifications of the neurological deficit. In conclusion, MRI volumetry using TBM is capable of demonstrating the progression of pontocerebellar atrophy in SCA2, supporting a possible role of MRI as biomarker in future trials.
format	article
author	Mario Mascalchi Stefano Diciotti Marco Giannelli Andrea Ginestroni Andrea Soricelli Emanuele Nicolai Marco Aiello Carlo Tessa Lucia Galli Maria Teresa Dotti Silvia Piacentini Elena Salvatore Nicola Toschi
author_facet	Mario Mascalchi Stefano Diciotti Marco Giannelli Andrea Ginestroni Andrea Soricelli Emanuele Nicolai Marco Aiello Carlo Tessa Lucia Galli Maria Teresa Dotti Silvia Piacentini Elena Salvatore Nicola Toschi
author_sort	Mario Mascalchi
title	Progression of brain atrophy in spinocerebellar ataxia type 2: a longitudinal tensor-based morphometry study.
title_short	Progression of brain atrophy in spinocerebellar ataxia type 2: a longitudinal tensor-based morphometry study.
title_full	Progression of brain atrophy in spinocerebellar ataxia type 2: a longitudinal tensor-based morphometry study.
title_fullStr	Progression of brain atrophy in spinocerebellar ataxia type 2: a longitudinal tensor-based morphometry study.
title_full_unstemmed	Progression of brain atrophy in spinocerebellar ataxia type 2: a longitudinal tensor-based morphometry study.
title_sort	progression of brain atrophy in spinocerebellar ataxia type 2: a longitudinal tensor-based morphometry study.
publisher	Public Library of Science (PLoS)
publishDate	2014
url	https://doaj.org/article/2e37afab5d904653b25abca50ec7e6f2
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Progression of brain atrophy in spinocerebellar ataxia type 2: a longitudinal tensor-based morphometry study.

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