{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,17]],"date-time":"2026-01-17T21:01:39Z","timestamp":1768683699758,"version":"3.49.0"},"reference-count":63,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2018,9,6]],"date-time":"2018-09-06T00:00:00Z","timestamp":1536192000000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Neurol."],"DOI":"10.3389\/fneur.2018.00747","type":"journal-article","created":{"date-parts":[[2018,9,6]],"date-time":"2018-09-06T04:47:51Z","timestamp":1536209271000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":36,"title":["Functional and Structural Brain Damage in Friedreich's Ataxia"],"prefix":"10.3389","volume":"9","author":[{"given":"Marinela","family":"Vavla","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Filippo","family":"Arrigoni","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andrea","family":"Nordio","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alberto","family":"De Luca","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Silvia","family":"Pizzighello","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elisa","family":"Petacchi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gabriella","family":"Paparella","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maria Grazia","family":"D'Angelo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Erika","family":"Brighina","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Emanuela","family":"Russo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marianna","family":"Fantin","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paola","family":"Colombo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andrea","family":"Martinuzzi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2018,9,6]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"1423","DOI":"10.1126\/science.271.5254.1423","article-title":"Friedreich's ataxia: autosomal recessive disease caused by an intronic GAA triplet repeat expansion","volume":"271","author":"Campuzano","year":"1996","journal-title":"Science"},{"key":"B2","doi-asserted-by":"publisher","first-page":"11","DOI":"10.1111\/jnc.12215","article-title":"Prevalence gradients of Friedreich's ataxia and R1b haplotype in Europe co-localize, suggesting a common Palaeolithic origin in the Franco-Cantabrian ice age refuge","volume":"126","author":"Vankan","year":"2013","journal-title":"J Neurochem."},{"key":"B3","doi-asserted-by":"publisher","first-page":"589","DOI":"10.1093\/brain\/104.3.589","article-title":"Friedreich's ataxia: a clinical and genetic study of 90 families with an analysis of early diagnostic criteria and intrafamilial clustering of clinical features","volume":"104","author":"Harding","year":"1981","journal-title":"Brain"},{"key":"B4","doi-asserted-by":"publisher","first-page":"1151","DOI":"10.1016\/S0140-6736(83)92879-9","article-title":"Classification of the hereditary ataxias and paraplegias","volume":"21","author":"Harding","year":"1983","journal-title":"Lancet"},{"key":"B5","doi-asserted-by":"publisher","first-page":"675","DOI":"10.1002\/ana.410410518","article-title":"Phenotypic variability in Friedreich ataxia: role of the associated GAA triplet repeat expansion","volume":"41","author":"Montermini","year":"1997","journal-title":"Ann Neurol."},{"key":"B6","doi-asserted-by":"publisher","first-page":"70","DOI":"10.1002\/mds.26436","article-title":"Longitudinal magnetic resonance imaging study shows progressive pyramidal and callosal damage in Friedreich's ataxia","volume":"31","author":"Rezende","year":"2016","journal-title":"Mov Disord"},{"key":"B7","doi-asserted-by":"publisher","first-page":"19","DOI":"10.1016\/j.neuroimage.2007.11.050","article-title":"Brain white matter tracts degeneration in Friedreich ataxia. An in vivo MRI study using tract-based spatial statistics and voxel-based morphometry","volume":"40","author":"Della Nave","year":"2008","journal-title":"Neuroimage"},{"key":"B8","doi-asserted-by":"publisher","first-page":"1131","DOI":"10.1111\/ene.12448","article-title":"Dentate nuclei T2 relaxometry is a reliable neuroimaging marker in Friedreich's ataxia","volume":"21","author":"Bonilha da Silva","year":"2014","journal-title":"Eur J Neurol."},{"key":"B9","doi-asserted-by":"publisher","first-page":"2215","DOI":"10.1007\/s00415-016-8252-7","article-title":"Cerebral and cerebellar grey matter atrophy in Friedreich ataxia: the IMAGE-FRDA study","volume":"263","author":"Selvadurai","year":"2016","journal-title":"J Neurol."},{"key":"B10","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2018-318422","article-title":"Structural characteristics of the central nervous system in Friedreich ataxia: an in vivo spinal cord and brain MRI study","author":"Dogan","year":"2018","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"B11","doi-asserted-by":"publisher","first-page":"82","DOI":"10.1136\/jnnp.2007.124297","article-title":"Brain structural damage in Friedreich's ataxia","volume":"79","author":"Della Nave","year":"2008","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"B12","doi-asserted-by":"publisher","first-page":"367","DOI":"10.1007\/s00234-010-0807-1","article-title":"Axial diffusivity is increased in the degenerating superior cerebellar peduncles of Friedreich's ataxia","volume":"53","author":"Della Nave","year":"2011","journal-title":"Neuroradiology"},{"key":"B13","doi-asserted-by":"publisher","first-page":"705","DOI":"10.1002\/mds.23518","article-title":"Brain diffusion-weighted imaging in Friedreich's ataxia","volume":"26","author":"Rizzo","year":"2011","journal-title":"Mov Disord."},{"key":"B14","doi-asserted-by":"publisher","first-page":"504","DOI":"10.1016\/j.parkreldis.2015.02.021","article-title":"Diffusion tensor imaging and tract-based spatial statistics analysis in Friedreich's ataxia patients","volume":"21","author":"Vieira Karuta","year":"2015","journal-title":"Parkinsonism Relat Disord."},{"key":"B15","doi-asserted-by":"publisher","first-page":"572","DOI":"10.1002\/acn3.315","article-title":"Cognition in Friedreich's ataxia: a behavioral and multimodal imaging study","volume":"3","author":"Dogan","year":"2016","journal-title":"Ann Clin Transl Neurol."},{"key":"B16","doi-asserted-by":"publisher","first-page":"1459","DOI":"10.1007\/s10072-013-1402-0","article-title":"Radial diffusivity in the cerebellar peduncles correlates with clinical severity in Friedreich ataxia","volume":"34","author":"Clemm von Hohenberg","year":"2013","journal-title":"Neurol Sci."},{"key":"B17","doi-asserted-by":"publisher","first-page":"81","DOI":"10.1007\/s12311-010-0232-3","article-title":"Superior cerebellar peduncle atrophy in Friedreich's ataxia correlates with disease symptoms","volume":"10","author":"Akhlaghi","year":"2011","journal-title":"Cerebellum"},{"key":"B18","doi-asserted-by":"publisher","first-page":"116","DOI":"10.1093\/brain\/awn269","article-title":"Visual system involvement in patients with Friedreich's ataxia","volume":"132","author":"Fortuna","year":"2009","journal-title":"Brain"},{"key":"B19","doi-asserted-by":"publisher","first-page":"827","DOI":"10.1111\/j.1468-1331.2006.01363.x","article-title":"Exploring mental status in Friedreich's ataxia: a combined neuropsychological, behavioral and neuroimaging study","volume":"13","author":"Mantovan","year":"2006","journal-title":"Eur J Neurol."},{"key":"B20","doi-asserted-by":"publisher","first-page":"138","DOI":"10.1016\/j.brainres.2012.06.035","article-title":"A functional MRI study of motor dysfunction in Friedreich's ataxia","volume":"1471","author":"Akhlaghi","year":"2012","journal-title":"Brain Res."},{"key":"B21","doi-asserted-by":"publisher","first-page":"1780","DOI":"10.1002\/hbm.21319","article-title":"Neurodegeneration in friedreich's ataxia is associated with a mixed activation pattern of the brain. A fMRI study","volume":"33","author":"Ginestroni","year":"2012","journal-title":"Hum Brain Mapp."},{"key":"B22","doi-asserted-by":"publisher","first-page":"1182","DOI":"10.1093\/brain\/awv064","article-title":"Structural and functional MRI abnormalities of cerebellar cortex and nuclei in SCA3, SCA6 and Friedreich's ataxia","volume":"138","author":"Stefanescu","year":"2015","journal-title":"Brain"},{"key":"B23","doi-asserted-by":"publisher","first-page":"1221","DOI":"10.1002\/mds.27023","article-title":"Cerebral compensation during motor function in Friedreich ataxia: The IMAGE-FRDA study","volume":"32","author":"Harding","year":"2017","journal-title":"Mov Disord."},{"key":"B24","doi-asserted-by":"publisher","first-page":"200","DOI":"10.1016\/j.bandc.2012.02.011","article-title":"Decreased functional brain activation in Friedreich ataxia using the Simon effect task","volume":"79","author":"Georgiou-Karistianis","year":"2012","journal-title":"Brain Cogn."},{"key":"B25","doi-asserted-by":"publisher","first-page":"338","DOI":"10.1002\/hbm.23034","article-title":"Fronto-cerebellar dysfunction and dysconnectivity underlying cognition in friedreich ataxia: The IMAGE-FRDA study","volume":"37","author":"Harding","year":"2016","journal-title":"Hum Brain Mapp."},{"key":"B26","doi-asserted-by":"publisher","first-page":"205","DOI":"10.1016\/S0022-510X(96)00231-6","article-title":"International Cooperative Ataxia Rating Scale for pharmacological assessment of the cerebellar syndrome. The Ataxia Neuropharmacology Committee of the World Federation of Neurology","volume":"145","author":"Trouillas","year":"1997","journal-title":"J Neurol Sci."},{"key":"B27","doi-asserted-by":"publisher","first-page":"1261","DOI":"10.1212\/01.WNL.0000156802.15466.79","article-title":"Measuring Friedreich ataxia: Interrater reliability of a neurologic rating scale","volume":"64","author":"Subramony","year":"2005","journal-title":"Neurology"},{"key":"B28","doi-asserted-by":"publisher","first-page":"1717","DOI":"10.1212\/01.wnl.0000219042.60538.92","article-title":"Scale for the assessment and rating of ataxia: development of a new clinical scale","volume":"66","author":"Schmitz-H\u00fcbsch","year":"2006","journal-title":"Neurology"},{"key":"B29","doi-asserted-by":"publisher","first-page":"684","DOI":"10.1002\/acn3.332","article-title":"Progression of Friedreich ataxia: quantitative characterization over 5 years","volume":"3","author":"Patel","year":"2016","journal-title":"Ann Clin Transl Neurol."},{"key":"B30","doi-asserted-by":"publisher","first-page":"1346","DOI":"10.1016\/S1474-4422(16)30287-3","article-title":"Progression characteristics of the European Friedreich's Ataxia Consortium for Translational Studies (EFACTS), a 2 year cohort study","volume":"15","author":"Reetz","year":"2016","journal-title":"Lancet Neurol."},{"key":"B31","article-title":"WISC-III","volume-title":"Contributo Alla Taratura Italiana","author":"Orsini","year":"2006"},{"key":"B32","volume-title":"Wechsler Adult Intelligence Scale\u20143rd ed.","author":"Wechsler","year":"1997"},{"key":"B33","doi-asserted-by":"publisher","first-page":"805","DOI":"10.1006\/nimg.2000.0582","article-title":"Voxel-based morphometry-the methods","volume":"11","author":"Ashburner","year":"2000","journal-title":"NeuroImage"},{"key":"B34","doi-asserted-by":"publisher","first-page":"S139","DOI":"10.1016\/j.neuroimage.2004.07.010","article-title":"Geodesic estimation for large deformation anatomical shape averaging and interpolation","volume":"23","author":"Avants","year":"2004","journal-title":"Neuroimage"},{"key":"B35","doi-asserted-by":"publisher","first-page":"1310","DOI":"10.1109\/TMI.2010.2046908","article-title":"N4ITK: improved N3 bias correction","volume":"29","author":"Tustison","year":"2010","journal-title":"IEEE Trans Med Imaging"},{"key":"B36","doi-asserted-by":"publisher","first-page":"45","DOI":"10.1109\/42.906424","article-title":"Segmentation of brain MR images through a hidden Markov random field model and the expectation-maximization algorithm","volume":"20","author":"Zhang","year":"2001","journal-title":"IEEE Trans Med Imaging"},{"key":"B37","doi-asserted-by":"publisher","first-page":"143","DOI":"10.1002\/hbm.10062","article-title":"Fast robust automated brain extraction","volume":"17","author":"Smith","year":"2002","journal-title":"Hum Brain Mapp."},{"key":"B38","doi-asserted-by":"publisher","first-page":"782","DOI":"10.1016\/j.neuroimage.2011.09.015","article-title":"FSL","volume":"62","author":"Jenkinson","year":"2012","journal-title":"NeuroImage"},{"key":"B39","doi-asserted-by":"publisher","first-page":"26","DOI":"10.1016\/j.media.2007.06.004","article-title":"Symmetric diffeomorphic image registration with cross-correlation: evaluating automated labeling of elderly and neurodegenerative brain","volume":"12","author":"Avants","year":"2008","journal-title":"Med Image Anal."},{"key":"B40","doi-asserted-by":"publisher","DOI":"10.3389\/fninf.2014.00044","article-title":"The Insight ToolKit image registration framework","author":"Avants","year":"2014","journal-title":"Front Neuroinformatics"},{"key":"B41","doi-asserted-by":"publisher","first-page":"381","DOI":"10.1016\/j.neuroimage.2014.01.060","article-title":"Permutation inference for the general linear model","volume":"92","author":"Winkler","year":"2014","journal-title":"NeuroImage"},{"key":"B42","doi-asserted-by":"publisher","first-page":"83","DOI":"10.1016\/j.neuroimage.2008.03.061","article-title":"Threshold-free cluster enhancement: addressing problems of smoothing, threshold dependence and localisation in cluster inference","volume":"44","author":"Smith","year":"2009","journal-title":"NeuroImage"},{"key":"B43","article-title":"TORTOISE: an integrated software package for processing of diffusion MRI data","volume-title":"ISMRM 18th Annual Meeting","author":"Pierpaoli","year":"2010"},{"key":"B44","doi-asserted-by":"publisher","first-page":"209","DOI":"10.1006\/jmrb.1996.0086","article-title":"Microstructural and physiological features of tissues elucidated by quantitative-diffusion-tensor MRI","volume":"111","author":"Basser","year":"1996","journal-title":"J Mag Reson Series B"},{"key":"B45","doi-asserted-by":"publisher","first-page":"115","DOI":"10.1016\/j.jmr.2006.06.020","article-title":"A unifying theoretical and algorithmic framework for least squares methods of estimation in diffusion tensor imaging","volume":"182","author":"Koay","year":"2006","journal-title":"J Magn Reson"},{"key":"B46","doi-asserted-by":"publisher","first-page":"1585","DOI":"10.1109\/TMI.2007.906784","article-title":"High-dimensional spatial normalization of diffusion tensor images improves the detection of white matter differences: an example study using amyotrophic lateral sclerosis","volume":"26","author":"Zhang","year":"2007","journal-title":"IEEE Trans Med Imaging"},{"key":"B47","unstructured":"Unbiased white matter atlas construction using diffusion Tensor Images ZhangH YushkevichPA RueckertD GeeJC 18044571Medical Image Computing and Computer-Assisted Intervention : MICCAI International Conference on Medical Image Computing and Computer-Assisted Intervention2007"},{"key":"B48","author":"Mori","year":"2005","journal-title":"MRI Atlas of Human White Matter"},{"key":"B49","doi-asserted-by":"crossref","DOI":"10.1016\/B978-012372560-8\/50002-4","volume-title":"Statistical Parametric Mapping: The Analysis of Functional Brain Images","author":"Friston","year":"2007"},{"key":"B50","article-title":"Use of T2-Weighted 3D Acquisition for Correction of EPI-Induced Distortion in FMRI","volume-title":"Proceedings of the 24th Annual Meeting of the International Society for Magnetic Resonance in Medicine","author":"Nordio","year":"2016"},{"key":"B51","article-title":"Detection and Repair of Transient Artifacts in fMRI Data","volume-title":"Paper Presented at the Meeting of the Society for Human Brain Mapping","author":"Mazaika","year":"2005"},{"key":"B52","doi-asserted-by":"publisher","first-page":"289","DOI":"10.1007\/s10048-007-0101-5","article-title":"Frataxin gene point mutations in Italian Friedreich ataxia patients","volume":"8","author":"Gellera","year":"2007","journal-title":"Neurogenetics"},{"key":"B53","doi-asserted-by":"publisher","first-page":"1114","DOI":"10.1007\/s00415-009-5079-5","article-title":"A combined voxel-based morphometry and 1H-MRS study in patients with Friedreich's ataxia","volume":"256","author":"Fran\u00e7a","year":"2009","journal-title":"J Neurol."},{"key":"B54","doi-asserted-by":"publisher","first-page":"93","DOI":"10.1016\/j.nicl.2014.08.018","article-title":"Cerebellar pathology in Friedreich's ataxia: atrophied dentate nuclei with normal iron content","volume":"6","author":"Solbach","year":"2014","journal-title":"Neuroimage Clin."},{"key":"B55","doi-asserted-by":"crossref","DOI":"10.1007\/978-3-319-24551-5_51","article-title":"Functional Topography of the Human Cerebellum","volume-title":"Essentials of Cerebellum and Cerebellar Disorders: A Primer For Graduate Students","author":"Stoodley","year":"2016"},{"key":"B56","doi-asserted-by":"publisher","first-page":"53","DOI":"10.3389\/fneur.2011.00053","article-title":"Cerebellum and ocular motor control","volume":"2","author":"Kheradmand","year":"2011","journal-title":"Front Neurol."},{"key":"B57","doi-asserted-by":"publisher","first-page":"430","DOI":"10.1196\/annals.1325.040","article-title":"Relative atrophy of the flocculus and ocular motor dysfunction in SCA2 and SCA6","volume":"1039","author":"Ying","year":"2005","journal-title":"Ann N Y Acad Sci."},{"key":"B58","doi-asserted-by":"publisher","first-page":"187","DOI":"10.1007\/s12311-013-0525-4","article-title":"Cognitive deficits in Friedreich ataxia correlate with micro-structural changes in dentatorubral tract","volume":"13","author":"Akhlaghi","year":"2014","journal-title":"Cerebellum"},{"key":"B59","doi-asserted-by":"publisher","first-page":"197","DOI":"10.1111\/jon.12270","article-title":"Regional cerebral disease progression in Friedreich's Ataxia: a longitudinal diffusion tensor imaging study","volume":"26","author":"Mascalchi","year":"2016","journal-title":"J Neuroimaging"},{"key":"B60","doi-asserted-by":"publisher","first-page":"504","DOI":"10.1111\/jon.12050","article-title":"White matter changes in patients with friedreich ataxia after treatment with erythropoietin","volume":"24","author":"Egger","year":"2014","journal-title":"J Neuroimaging"},{"key":"B61","doi-asserted-by":"publisher","first-page":"95","DOI":"10.1016\/S0304-3940(99)00433-4","article-title":"Bilateral representation of sequential finger movements in human cortical areas","volume":"269","author":"Baraldi","year":"2005","journal-title":"Neurosci Lett."},{"key":"B62","doi-asserted-by":"crossref","first-page":"43","DOI":"10.55782\/ane-2007-1631","article-title":"Brain correlates of right-handedness","volume":"67","author":"Gut","year":"2007","journal-title":"Acta Neurobiol Exp."},{"key":"B63","doi-asserted-by":"publisher","first-page":"2047981614531573","DOI":"10.1177\/2047981614531573","article-title":"A longitudinal VBM study monitoring treatment with erythropoietin in patients with Friedreich ataxia","volume":"12","author":"Santner","year":"2014","journal-title":"Acta Radiol Short Rep."}],"container-title":["Frontiers in Neurology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/article\/10.3389\/fneur.2018.00747\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,7,10]],"date-time":"2024-07-10T00:27:01Z","timestamp":1720571221000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/article\/10.3389\/fneur.2018.00747\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,9,6]]},"references-count":63,"alternative-id":["10.3389\/fneur.2018.00747"],"URL":"https:\/\/doi.org\/10.3389\/fneur.2018.00747","relation":{},"ISSN":["1664-2295"],"issn-type":[{"value":"1664-2295","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,9,6]]},"article-number":"747"}}