{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,21]],"date-time":"2026-03-21T13:16:28Z","timestamp":1774098988768,"version":"3.50.1"},"reference-count":42,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2018,4,14]],"date-time":"2018-04-14T00:00:00Z","timestamp":1523664000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Ann Clin Transl Neurol"],"published-print":{"date-parts":[[2018,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objective<\/jats:title><jats:p>The aim of this study was to perform the first resting\u2010state functional <jats:styled-content style=\"fixed-case\">MRI<\/jats:styled-content> (<jats:styled-content style=\"fixed-case\">RS<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">fMRI<\/jats:styled-content>) analysis in Friedreich's ataxia (<jats:styled-content style=\"fixed-case\">FRDA<\/jats:styled-content>) patients to assess possible brain functional connectivity (<jats:styled-content style=\"fixed-case\">FC<\/jats:styled-content>) differences in these patients, and test their correlations with neuropsychological performances.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>In total, 24 <jats:styled-content style=\"fixed-case\">FRDA<\/jats:styled-content> patients (M\/F: 15\/9, mean age 31.3 \u00b1 15.0) and 24 healthy controls (<jats:styled-content style=\"fixed-case\">HC<\/jats:styled-content>; M\/F: 15\/9, mean age 30.7 \u00b1 15.5) were enrolled in this cross\u2010sectional study. All patients underwent a thorough neuropsychological battery, investigating different cognitive domains. <jats:styled-content style=\"fixed-case\">RS<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">fMRI<\/jats:styled-content> data were analyzed using a seed\u2010based approach, probing the <jats:styled-content style=\"fixed-case\">FC<\/jats:styled-content> of cortical areas potentially referable to specific executive and cognitive functions compromised in <jats:styled-content style=\"fixed-case\">FRDA<\/jats:styled-content>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Compared to <jats:styled-content style=\"fixed-case\">HC<\/jats:styled-content>,<jats:styled-content style=\"fixed-case\"> FRDA<\/jats:styled-content> patients showed overall worse neuropsychological scores in several domains, including global cognitive assessment, spatial memory, visuoperception and visuospatial functions, and executive functions. Analysis of <jats:styled-content style=\"fixed-case\">RS<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">fMRI<\/jats:styled-content> data showed a higher <jats:styled-content style=\"fixed-case\">FC<\/jats:styled-content> in <jats:styled-content style=\"fixed-case\">FRDA<\/jats:styled-content> patients compared to <jats:styled-content style=\"fixed-case\">HC<\/jats:styled-content> between paracingulate gyri and the medial frontal gryrus, between the superior frontal gyrus and bilateral angular gyri, and between the middle temporal gyrus and the cingulate gyrus, with a reduced <jats:styled-content style=\"fixed-case\">FC<\/jats:styled-content> between the medial frontal gryrus and the cerebellum.<\/jats:p><\/jats:sec><jats:sec><jats:title>Interpretation<\/jats:title><jats:p>We found a reduction in <jats:styled-content style=\"fixed-case\">FC<\/jats:styled-content> between frontal areas and the contralateral cerebellar cortex in <jats:styled-content style=\"fixed-case\">FRDA<\/jats:styled-content>, in line with the known alteration in cerebello\u2010cortical pathway in this condition. On the other hand, a higher <jats:styled-content style=\"fixed-case\">FC<\/jats:styled-content> between different cortical areas was shown, possibly reflecting a compensatory phenomenon. These results, in conjunction with clinical findings, may shed new light on the pattern of supratentorial and infratentorial involvement, and on dynamics of brain plasticity in this disease.<\/jats:p><\/jats:sec>","DOI":"10.1002\/acn3.555","type":"journal-article","created":{"date-parts":[[2018,4,14]],"date-time":"2018-04-14T13:04:12Z","timestamp":1523711052000},"page":"677-686","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":36,"title":["Cognitive and functional connectivity alterations in Friedreich's ataxia"],"prefix":"10.1002","volume":"5","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0300-5160","authenticated-orcid":false,"given":"Sirio","family":"Cocozza","sequence":"first","affiliation":[{"name":"Department of Advanced Biomedical Sciences University \u201cFederico II\u201d Naples 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