{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,28]],"date-time":"2026-09-28T14:14:56Z","timestamp":1790604896014,"version":"4.1.0"},"reference-count":30,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2015,7,14]],"date-time":"2015-07-14T00:00:00Z","timestamp":1436832000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Journal of Neuroimaging"],"published-print":{"date-parts":[[2016,3]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>BACKGROUND AND PURPOSE<\/jats:title><jats:p>Imaging biomarkers of disease progression are desirable in inherited ataxias. MRI has demonstrated brain damage in Friedreich ataxia (FRDA) in form of regional atrophy of the medulla, peridentate cerebellar white matter (WM) and superior cerebellar peduncles (visible in T1\u2010weighted images) and of change of <jats:italic>microstructural<\/jats:italic> characteristics of WM tracts of the brainstem, cerebellar peduncles, cerebellum, and supratentorial structures (visible through diffusion\u2010weighted imaging). We explored the potential of brain MR morphometry and diffusion tensor imaging (DTI) to track the progression of neurodegeneration in FRDA.<\/jats:p><\/jats:sec><jats:sec><jats:title>METHODS<\/jats:title><jats:p>Eight patients (5F, 3M; age 13.4\u201041.2 years) and 8 healthy controls (2F, 6M; age 26.2\u201048.3 years) underwent 2 MRI examinations (mean 3.9 and 4.1 years apart, respectively) on the same 1.5T scanner. The protocol included 3D T1\u2010weighted images and axial diffusion\u2010weighted images (b\u2010value 1,000 s\/mm<jats:sup>2<\/jats:sup>) for calculating maps of fractional anisotropy, mean, axial and radial diffusivity, and mode of anisotropy. Tensor\u2010based morphometry was used to investigate regional volume changes and tract\u2010based spatial statistics was used to investigate <jats:italic>microstructural<\/jats:italic> changes in WM tracts.<\/jats:p><\/jats:sec><jats:sec><jats:title>RESULTS<\/jats:title><jats:p>Longitudinal analyses showed no differences in regional volume changes but a significant difference in axial diffusivity changes in cerebral and corpus callosum WM of patients as compared to controls (mean longitudinal rate of change for axial diffusivity: \u2212.02 \u00d7 10<jats:sup>\u20103<\/jats:sup> mm<jats:sup>2<\/jats:sup>\/s\/year in patients vs. .01 \u00d7 10<jats:sup>\u20103<\/jats:sup> mm<jats:sup>2<\/jats:sup>\/s\/year in controls). No correlation with number of triplets, disease duration, and worsening of the clinical deficit was observed.<\/jats:p><\/jats:sec><jats:sec><jats:title>CONCLUSION<\/jats:title><jats:p>DTI can track brain <jats:italic>microstructural<\/jats:italic> changes in FRDA and can be considered a potential biomarker of disease progression.<\/jats:p><\/jats:sec>","DOI":"10.1111\/jon.12270","type":"journal-article","created":{"date-parts":[[2015,7,14]],"date-time":"2015-07-14T22:49:15Z","timestamp":1436914155000},"page":"197-200","source":"Crossref","is-referenced-by-count":23,"title":["Regional Cerebral Disease Progression in Friedreich's Ataxia: A Longitudinal Diffusion Tensor Imaging Study"],"prefix":"10.1111","volume":"26","author":[{"given":"Mario","family":"Mascalchi","sequence":"first","affiliation":[{"name":"Quantitative and Functional Neuroradiology Research Unit at Meyer Children and Careggi Hospitals of Florence  Florence Italy"},{"name":"\u201cMario Serio\u201d Department of Experimental and Clinical Biomedical Sciences University of Florence  Florence Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicola","family":"Toschi","sequence":"additional","affiliation":[{"name":"Medical Physics Section, Department of Biomedicine and Prevention University of Rome \u201cTor Vergata,\u201d  Rome Italy"},{"name":"Department of Radiology Athinoula A. Martinos Center for Biomedical Imaging  Boston MA"},{"name":"Harvard Medical School  Boston MA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco","family":"Giannelli","sequence":"additional","affiliation":[{"name":"Unit of Medical Physics Pisa University Hospital \u201cAzienda Ospedaliero\u2010Universitaria Pisana,\u201d  Pisa Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Ginestroni","sequence":"additional","affiliation":[{"name":"Neuroradiology Unit Careggi General Hospital  Florence Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Riccardo","family":"Della Nave","sequence":"additional","affiliation":[{"name":"\u201cSan Giuseppe\u201d Hospital  Azienda USL 11 Empoli Empoli (Fi) Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlo","family":"Tessa","sequence":"additional","affiliation":[{"name":"Unit of Radiology Versilia Hospital  Azienda USL 12 Viareggio Lido di Camaiore (Lu) Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silvia","family":"Piacentini","sequence":"additional","affiliation":[{"name":"NEUROFARBA Department University of Florence  Florence Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Teresa","family":"Dotti","sequence":"additional","affiliation":[{"name":"Department of Neurological Sciences University of Siena  Siena Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco","family":"Aiello","sequence":"additional","affiliation":[{"name":"IRCSS SDN Foundation  Naples Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emanuele","family":"Nicolai","sequence":"additional","affiliation":[{"name":"IRCSS SDN Foundation  Naples Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Soricelli","sequence":"additional","affiliation":[{"name":"IRCSS SDN Foundation  Naples Italy"},{"name":"University of Naples Parthenope  Naples 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