{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T11:06:28Z","timestamp":1790420788525,"version":"4.1.0"},"reference-count":53,"publisher":"Springer Science and Business Media LLC","issue":"5","license":[{"start":{"date-parts":[[2023,3,1]],"date-time":"2023-03-01T00:00:00Z","timestamp":1677628800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2023,3,1]],"date-time":"2023-03-01T00:00:00Z","timestamp":1677628800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"Australian National Health and Medical Research Council","award":["1046037"],"award-info":[{"award-number":["1046037"]}]},{"DOI":"10.13039\/100002108","name":"Friedreich's Ataxia Research Alliance","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100002108","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Friedreich Ataxia Research Association"},{"DOI":"10.13039\/501100001779","name":"Monash University","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100001779","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J Neurol"],"published-print":{"date-parts":[[2023,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Friedreich ataxia (FRDA) is a rare, inherited neurodegenerative disease characterised in most cases by progressive and debilitating motor dysfunction. Degeneration of cerebellar white matter pathways have been previously reported, alongside indications of cerebello-cerebral functional alterations. In this work, we examine resting-state functional connectivity changes within cerebello-cerebral circuits, and their associations with disease severity (Scale for the Assessment and Rating of Ataxia [SARA]), psychomotor function (speeded and paced finger tapping), and white matter integrity (diffusion tensor imaging) in 35 adults with FRDA and 45 age and sex-matched controls. Voxel-wise seed-based functional connectivity was assessed for three cerebellar cortical regions (anterior lobe, lobules I-V; superior posterior lobe, lobules VI-VIIB; inferior posterior lobe, lobules VIIIA-IX) and two dentate nucleus seeds (dorsal and ventral). Compared to controls, people with FRDA showed significantly reduced connectivity between the anterior cerebellum and bilateral pre\/postcentral gyri, and between the superior posterior cerebellum and left dorsolateral PFC. Greater disease severity correlated with lower connectivity in these circuits. Lower anterior cerebellum-motor cortex functional connectivity also correlated with slower speeded finger tapping and less fractional anisotropy in the superior cerebellar peduncles, internal capsule, and precentral white matter in the FRDA cohort. There were no significant between-group differences in inferior posterior cerebellar or dentate nucleus connectivity. This study indicates that altered cerebello-cerebral functional connectivity is associated with functional status and white matter damage in cerebellar efferent pathways in people with FRDA, particularly in motor circuits.<\/jats:p>","DOI":"10.1007\/s00415-023-11637-x","type":"journal-article","created":{"date-parts":[[2023,3,1]],"date-time":"2023-03-01T18:02:52Z","timestamp":1677693772000},"page":"2360-2369","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["Reduced cerebello-cerebral functional connectivity correlates with disease severity and impaired white matter integrity in Friedreich ataxia"],"prefix":"10.1007","volume":"270","author":[{"given":"Rebecca","family":"Kerestes","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hannah","family":"Cummins","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nellie","family":"Georgiou-Karistianis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Louisa P.","family":"Selvadurai","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Louise A.","family":"Corben","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martin B.","family":"Delatycki","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gary F.","family":"Egan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6843-9592","authenticated-orcid":false,"given":"Ian H.","family":"Harding","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2023,3,1]]},"reference":[{"key":"11637_CR1","doi-asserted-by":"publisher","first-page":"1133","DOI":"10.1177\/0883073812448230","volume":"27","author":"MB Delatycki","year":"2012","unstructured":"Delatycki MB, Corben LA (2012) Clinical features of Friedreich ataxia. J Child Neurol 27:1133\u20131137","journal-title":"J Child Neurol"},{"key":"11637_CR2","doi-asserted-by":"publisher","first-page":"570","DOI":"10.1002\/ana.26200","volume":"90","author":"IH Harding","year":"2021","unstructured":"Harding IH, Chopra S, Arrigoni F, Boesch S, Brunetti A, Cocozza S, Corben LA, Deistung A, Delatycki M, Diciotti S, Dogan I, Evangelisti S, Franca MC Jr, Goricke SL, Georgiou-Karistianis N, Gramegna LL, Henry PG, Hernandez-Castillo CR, Hutter D, Jahanshad N, Joers JM, Lenglet C, Lodi R, Manners DN, Martinez ARM, Martinuzzi A, Marzi C, Mascalchi M, Nachbauer W, Pane C, Peruzzo D, Pisharady PK, Pontillo G, Reetz K, Rezende TJR, Romanzetti S, Sacca F, Scherfler C, Schulz JB, Stefani A, Testa C, Thomopoulos SI, Timmann D, Tirelli S, Tonon C, Vavla M, Egan GF, Thompson PM (2021) Brain structure and degeneration staging in Friedreich Ataxia: magnetic resonance imaging volumetrics from the ENIGMA-Ataxia working group. Ann Neurol 90:570\u2013583","journal-title":"Ann Neurol"},{"key":"11637_CR3","doi-asserted-by":"publisher","first-page":"338","DOI":"10.1002\/hbm.23034","volume":"37","author":"IH Harding","year":"2016","unstructured":"Harding IH, Corben LA, Storey E, Egan GF, Stagnitti MR, Poudel GR, Delatycki MB, Georgiou-Karistianis N (2016) Fronto-cerebellar dysfunction and dysconnectivity underlying cognition in friedreich ataxia: the IMAGE-FRDA study. Hum Brain Mapp 37:338\u2013350","journal-title":"Hum Brain Mapp"},{"key":"11637_CR4","doi-asserted-by":"publisher","first-page":"435","DOI":"10.1007\/s12311-019-1008-z","volume":"18","author":"T Lindig","year":"2019","unstructured":"Lindig T, Bender B, Kumar VJ, Hauser TK, Grodd W, Brendel B, Just J, Synofzik M, Klose U, Scheffler K, Ernemann U, Schols L (2019) Pattern of cerebellar atrophy in Friedreich\u2019s Ataxia-using the SUIT template. Cerebellum 18:435\u2013447","journal-title":"Cerebellum"},{"key":"11637_CR5","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1016\/j.jns.2011.01.010","volume":"303","author":"AH Koeppen","year":"2011","unstructured":"Koeppen AH (2011) Friedreich\u2019s ataxia: pathology, pathogenesis, and molecular genetics. J Neurol Sci 303:1\u201312","journal-title":"J Neurol Sci"},{"key":"11637_CR6","doi-asserted-by":"publisher","first-page":"572","DOI":"10.1002\/acn3.315","volume":"3","author":"I Dogan","year":"2016","unstructured":"Dogan I, Tinnemann E, Romanzetti S, Mirzazade S, Costa AS, Werner CJ, Heim S, Fedosov K, Schulz S, Timmann D, Giordano IA, Klockgether T, Schulz JB, Reetz K (2016) Cognition in Friedreich\u2019s ataxia: a behavioral and multimodal imaging study. Ann Clin Transl Neurol 3:572\u2013587","journal-title":"Ann Clin Transl Neurol"},{"key":"11637_CR7","doi-asserted-by":"publisher","first-page":"81","DOI":"10.1007\/s12311-010-0232-3","volume":"10","author":"H Akhlaghi","year":"2011","unstructured":"Akhlaghi H, Corben L, Georgiou-Karistianis N, Bradshaw J, Storey E, Delatycki MB, Egan GF (2011) Superior cerebellar peduncle atrophy in Friedreich\u2019s ataxia correlates with disease symptoms. Cerebellum 10:81\u201387","journal-title":"Cerebellum"},{"key":"11637_CR8","doi-asserted-by":"publisher","first-page":"1459","DOI":"10.1007\/s10072-013-1402-0","volume":"34","author":"C Clemm von Hohenberg","year":"2013","unstructured":"Clemm von Hohenberg C, Schocke MF, Wigand MC, Nachbauer W, Guttmann CR, Kubicki M, Shenton ME, Boesch S, Egger K (2013) Radial diffusivity in the cerebellar peduncles correlates with clinical severity in Friedreich ataxia. Neurol Sci 34:1459\u20131462","journal-title":"Neurol Sci"},{"key":"11637_CR9","doi-asserted-by":"publisher","first-page":"138","DOI":"10.1016\/j.jns.2014.05.057","volume":"343","author":"LA Corben","year":"2014","unstructured":"Corben LA, Kashuk SR, Akhlaghi H, Jamadar S, Delatycki MB, Fielding J, Johnson B, Georgiou-Karistianis N, Egan GF (2014) Myelin paucity of the superior cerebellar peduncle in individuals with Friedreich ataxia: an MRI magnetization transfer imaging study. J Neurol Sci 343:138\u2013143","journal-title":"J Neurol Sci"},{"key":"11637_CR10","doi-asserted-by":"publisher","first-page":"367","DOI":"10.1007\/s00234-010-0807-1","volume":"53","author":"R Della Nave","year":"2011","unstructured":"Della Nave R, Ginestroni A, Diciotti S, Salvatore E, Soricelli A, Mascalchi M (2011) Axial diffusivity is increased in the degenerating superior cerebellar peduncles of Friedreich\u2019s ataxia. Neuroradiology 53:367\u2013372","journal-title":"Neuroradiology"},{"key":"11637_CR11","doi-asserted-by":"publisher","first-page":"19","DOI":"10.1016\/j.neuroimage.2007.11.050","volume":"40","author":"R Della Nave","year":"2008","unstructured":"Della Nave R, Ginestroni A, Tessa C, Salvatore E, Bartolomei I, Salvi F, Dotti MT, De Michele G, Piacentini S, Mascalchi M (2008) Brain white matter tracts degeneration in Friedreich ataxia. an in vivo MRI study using tract-based spatial statistics and voxel-based morphometry. Neuroimage 40:19\u201325","journal-title":"Neuroimage"},{"key":"11637_CR12","doi-asserted-by":"publisher","first-page":"882","DOI":"10.1148\/radiol.10091742","volume":"255","author":"E Pagani","year":"2010","unstructured":"Pagani E, Ginestroni A, Della Nave R, Agosta F, Salvi F, De Michele G, Piacentini S, Filippi M, Mascalchi M (2010) Assessment of brain white matter fiber bundle atrophy in patients with Friedreich ataxia. Radiology 255:882\u2013889","journal-title":"Radiology"},{"key":"11637_CR13","doi-asserted-by":"publisher","first-page":"705","DOI":"10.1002\/mds.23518","volume":"26","author":"G Rizzo","year":"2011","unstructured":"Rizzo G, Tonon C, Valentino ML, Manners D, Fortuna F, Gellera C, Pini A, Ghezzo A, Baruzzi A, Testa C, Malucelli E, Barbiroli B, Carelli V, Lodi R (2011) Brain diffusion-weighted imaging in Friedreich\u2019s ataxia. Mov Disord 26:705\u2013712","journal-title":"Mov Disord"},{"key":"11637_CR14","doi-asserted-by":"publisher","first-page":"504","DOI":"10.1016\/j.parkreldis.2015.02.021","volume":"21","author":"SC Vieira Karuta","year":"2015","unstructured":"Vieira Karuta SC, Raskin S, de Carvalho NA, Gasparetto EL, Doring T, Teive HA (2015) Diffusion tensor imaging and tract-based spatial statistics analysis in Friedreich\u2019s ataxia patients. Parkinsonism Relat Disord 21:504\u2013508","journal-title":"Parkinsonism Relat Disord"},{"key":"11637_CR15","doi-asserted-by":"publisher","first-page":"1920","DOI":"10.1002\/hbm.24921","volume":"41","author":"LP Selvadurai","year":"2020","unstructured":"Selvadurai LP, Corben LA, Delatycki MB, Storey E, Egan GF, Georgiou-Karistianis N, Harding IH (2020) Multiple mechanisms underpin cerebral and cerebellar white matter deficits in Friedreich ataxia: the IMAGE-FRDA study. Hum Brain Mapp 41:1920\u20131933","journal-title":"Hum Brain Mapp"},{"key":"11637_CR16","doi-asserted-by":"publisher","DOI":"10.3389\/fneur.2021.802496","volume":"12","author":"M Vavla","year":"2021","unstructured":"Vavla M, Arrigoni F, Peruzzo D, Montanaro D, Frijia F, Pizzighello S, De Luca A, Della Libera E, Tessarotto F, Guerra P, Harding IH, Martinuzzi A (2021) Functional MRI studies in Friedreich\u2019s Ataxia: a systematic review. Front Neurol 12:802496","journal-title":"Front Neurol"},{"key":"11637_CR17","doi-asserted-by":"publisher","first-page":"138","DOI":"10.1016\/j.brainres.2012.06.035","volume":"1471","author":"H Akhlaghi","year":"2012","unstructured":"Akhlaghi H, Corben L, Georgiou-Karistianis N, Bradshaw J, Delatycki MB, Storey E, Egan GF (2012) A functional MRI study of motor dysfunction in Friedreich\u2019s ataxia. Brain Res 1471:138\u2013154","journal-title":"Brain Res"},{"key":"11637_CR18","doi-asserted-by":"publisher","first-page":"200","DOI":"10.1016\/j.bandc.2012.02.011","volume":"79","author":"N Georgiou-Karistianis","year":"2012","unstructured":"Georgiou-Karistianis N, Akhlaghi H, Corben LA, Delatycki MB, Storey E, Bradshaw JL, Egan GF (2012) Decreased functional brain activation in Friedreich ataxia using the Simon effect task. Brain Cogn 79:200\u2013208","journal-title":"Brain Cogn"},{"key":"11637_CR19","doi-asserted-by":"publisher","first-page":"1780","DOI":"10.1002\/hbm.21319","volume":"33","author":"A Ginestroni","year":"2012","unstructured":"Ginestroni A, Diciotti S, Cecchi P, Pesaresi I, Tessa C, Giannelli M, Della Nave R, Salvatore E, Salvi F, Dotti MT, Piacentini S, Soricelli A, Cosottini M, De Stefano N, Mascalchi M (2012) Neurodegeneration in friedreich\u2019s ataxia is associated with a mixed activation pattern of the brain. A fMRI study Hum Brain Mapp 33:1780\u20131791","journal-title":"A fMRI study Hum Brain Mapp"},{"key":"11637_CR20","doi-asserted-by":"publisher","first-page":"809","DOI":"10.1007\/s00429-021-02413-6","volume":"227","author":"R Shishegar","year":"2022","unstructured":"Shishegar R, Harding IH, Selvadurai LP, Corben LA, Delatycki MB, Egan GF, Georgiou-Karistianis N (2022) Longitudinal investigation of brain activation during motor tasks in Friedreich ataxia: 24-month data from IMAGE-FRDA. Brain Struct Funct 227:809\u2013819","journal-title":"Brain Struct Funct"},{"key":"11637_CR21","doi-asserted-by":"publisher","first-page":"677","DOI":"10.1002\/acn3.555","volume":"5","author":"S Cocozza","year":"2018","unstructured":"Cocozza S, Costabile T, Tedeschi E, Abate F, Russo C, Liguori A, Del Vecchio W, Paciello F, Quarantelli M, Filla A, Brunetti A, Sacca F (2018) Cognitive and functional connectivity alterations in Friedreich\u2019s ataxia. Ann Clin Transl Neurol 5:677\u2013686","journal-title":"Ann Clin Transl Neurol"},{"key":"11637_CR22","doi-asserted-by":"publisher","first-page":"5334","DOI":"10.1002\/hbm.25621","volume":"42","author":"G Naeije","year":"2021","unstructured":"Naeije G, Coquelet N, Wens V, Goldman S, Pandolfo M, De Tiege X (2021) Age of onset modulates resting-state brain network dynamics in Friedreich Ataxia. Hum Brain Mapp 42:5334\u20135344","journal-title":"Hum Brain Mapp"},{"key":"11637_CR23","doi-asserted-by":"publisher","first-page":"94","DOI":"10.1002\/acn3.50966","volume":"7","author":"G Naeije","year":"2020","unstructured":"Naeije G, Wens V, Coquelet N, Sjogard M, Goldman S, Pandolfo M, De Tiege XP (2020) Age of onset determines intrinsic functional brain architecture in Friedreich ataxia. Ann Clin Transl Neurol 7:94\u2013104","journal-title":"Ann Clin Transl Neurol"},{"key":"11637_CR24","doi-asserted-by":"publisher","first-page":"8432","DOI":"10.1523\/JNEUROSCI.23-23-08432.2003","volume":"23","author":"RM Kelly","year":"2003","unstructured":"Kelly RM, Strick PL (2003) Cerebellar loops with motor cortex and prefrontal cortex of a nonhuman primate. J Neurosci 23:8432\u20138444","journal-title":"J Neurosci"},{"key":"11637_CR25","doi-asserted-by":"publisher","first-page":"489","DOI":"10.1016\/j.neuroimage.2008.08.039","volume":"44","author":"CJ Stoodley","year":"2009","unstructured":"Stoodley CJ, Schmahmann JD (2009) Functional topography in the human cerebellum: a meta-analysis of neuroimaging studies. Neuroimage 44:489\u2013501","journal-title":"Neuroimage"},{"key":"11637_CR26","doi-asserted-by":"publisher","first-page":"1178","DOI":"10.1001\/archneur.1991.00530230086029","volume":"48","author":"JD Schmahmann","year":"1991","unstructured":"Schmahmann JD (1991) An emerging concept. The cerebellar contribution to higher function. Arch Neurol 48:1178\u20131187","journal-title":"Arch Neurol"},{"key":"11637_CR27","doi-asserted-by":"publisher","first-page":"53","DOI":"10.1002\/cne.902890105","volume":"289","author":"JD Schmahmann","year":"1989","unstructured":"Schmahmann JD, Pandya DN (1989) Anatomical investigation of projections to the basis pontis from posterior parietal association cortices in rhesus monkey. J Comp Neurol 289:53\u201373","journal-title":"J Comp Neurol"},{"key":"11637_CR28","doi-asserted-by":"publisher","first-page":"1213","DOI":"10.1016\/j.neuroimage.2012.04.011","volume":"61","author":"L Sang","year":"2012","unstructured":"Sang L, Qin W, Liu Y, Han W, Zhang Y, Jiang T, Yu C (2012) Resting-state functional connectivity of the vermal and hemispheric subregions of the cerebellum with both the cerebral cortical networks and subcortical structures. Neuroimage 61:1213\u20131225","journal-title":"Neuroimage"},{"key":"11637_CR29","doi-asserted-by":"publisher","first-page":"2151","DOI":"10.1093\/cercor\/bht065","volume":"24","author":"JA Bernard","year":"2014","unstructured":"Bernard JA, Peltier SJ, Benson BL, Wiggins JL, Jaeggi SM, Buschkuehl M, Jonides J, Monk CS, Seidler RD (2014) Dissociable functional networks of the human dentate nucleus. Cereb Cortex 24:2151\u20132159","journal-title":"Cereb Cortex"},{"key":"11637_CR30","doi-asserted-by":"publisher","first-page":"413","DOI":"10.1146\/annurev.neuro.31.060407.125606","volume":"32","author":"PL Strick","year":"2009","unstructured":"Strick PL, Dum RP, Fiez JA (2009) Cerebellum and nonmotor function. Annu Rev Neurosci 32:413\u2013434","journal-title":"Annu Rev Neurosci"},{"key":"11637_CR31","doi-asserted-by":"publisher","first-page":"1221","DOI":"10.1002\/mds.27023","volume":"32","author":"IH Harding","year":"2017","unstructured":"Harding IH, Corben LA, Delatycki MB, Stagnitti MR, Storey E, Egan GF, Georgiou-Karistianis N (2017) Cerebral compensation during motor function in Friedreich ataxia: The IMAGE-FRDA study. Mov Disord 32:1221\u20131229","journal-title":"Mov Disord"},{"key":"11637_CR32","doi-asserted-by":"publisher","first-page":"218","DOI":"10.1002\/mds.28825","volume":"37","author":"W Khan","year":"2022","unstructured":"Khan W, Corben LA, Bilal H, Vivash L, Delatycki MB, Egan GF, Harding IH (2022) Neuroinflammation in the cerebellum and brainstem in Friedreich Ataxia: an [18F]-FEMPA PET study. Mov Disord 37:218\u2013224","journal-title":"Mov Disord"},{"key":"11637_CR33","doi-asserted-by":"publisher","first-page":"1717","DOI":"10.1212\/01.wnl.0000219042.60538.92","volume":"66","author":"T Schmitz-Hubsch","year":"2006","unstructured":"Schmitz-Hubsch T, du Montcel ST, Baliko L, Berciano J, Boesch S, Depondt C, Giunti P, Globas C, Infante J, Kang JS, Kremer B, Mariotti C, Melegh B, Pandolfo M, Rakowicz M, Ribai P, Rola R, Schols L, Szymanski S, van de Warrenburg BP, Durr A, Klockgether T, Fancellu R (2006) Scale for the assessment and rating of ataxia: development of a new clinical scale. Neurology 66:1717\u20131720","journal-title":"Neurology"},{"key":"11637_CR34","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuroimage.2020.116819","volume":"218","author":"S Han","year":"2020","unstructured":"Han S, Carass A, He Y, Prince JL (2020) Automatic cerebellum anatomical parcellation using U-Net with locally constrained optimization. Neuroimage 218:116819","journal-title":"Neuroimage"},{"key":"11637_CR35","doi-asserted-by":"publisher","DOI":"10.3791\/63340-v","author":"R Kerestes","year":"2022","unstructured":"Kerestes R, Han S, Balachander S, Hernandez-Castillo C, Prince JL, Diedrichsen J, Harding IH (2022) A standardized pipeline for examining human cerebellar grey matter morphometry using structural magnetic resonance imaging. J Vis Exp. https:\/\/doi.org\/10.3791\/63340-v","journal-title":"J Vis Exp"},{"key":"11637_CR36","doi-asserted-by":"publisher","first-page":"tgab050","DOI":"10.1093\/texcom\/tgab050","volume":"2","author":"JA Bernard","year":"2021","unstructured":"Bernard JA, Ballard HK, Jackson TB (2021) Cerebellar dentate connectivity across adulthood: a large-scale resting state functional connectivity investigation. Cereb Cortex Commun. 2:tgab050","journal-title":"Cereb Cortex Commun."},{"key":"11637_CR37","doi-asserted-by":"publisher","first-page":"125","DOI":"10.1089\/brain.2012.0073","volume":"2","author":"S Whitfield-Gabrieli","year":"2012","unstructured":"Whitfield-Gabrieli S, Nieto-Castanon A (2012) Conn: a functional connectivity toolbox for correlated and anticorrelated brain networks. Brain Connect 2:125\u2013141","journal-title":"Brain Connect"},{"key":"11637_CR38","doi-asserted-by":"publisher","first-page":"692","DOI":"10.1089\/brain.2019.0692","volume":"9","author":"SA Anteraper","year":"2019","unstructured":"Anteraper SA, Guell X, Taylor HP, D\u2019Mello A, Whitfield-Gabrieli S, Joshi G (2019) Intrinsic functional connectivity of dentate nuclei in autism spectrum disorder. Brain Connect 9:692\u2013702","journal-title":"Brain Connect"},{"key":"11637_CR39","doi-asserted-by":"publisher","first-page":"1487","DOI":"10.1016\/j.neuroimage.2006.02.024","volume":"31","author":"SM Smith","year":"2006","unstructured":"Smith SM, Jenkinson M, Johansen-Berg H, Rueckert D, Nichols TE, Mackay CE, Watkins KE, Ciccarelli O, Cader MZ, Matthews PM, Behrens TE (2006) Tract-based spatial statistics: voxelwise analysis of multi-subject diffusion data. Neuroimage 31:1487\u20131505","journal-title":"Neuroimage"},{"key":"11637_CR40","doi-asserted-by":"publisher","first-page":"381","DOI":"10.1016\/j.neuroimage.2014.01.060","volume":"92","author":"AM Winkler","year":"2014","unstructured":"Winkler AM, Ridgway GR, Webster MA, Smith SM, Nichols TE (2014) Permutation inference for the general linear model. Neuroimage 92:381\u2013397","journal-title":"Neuroimage"},{"key":"11637_CR41","doi-asserted-by":"publisher","first-page":"687","DOI":"10.1136\/jnnp-2011-301940","volume":"83","author":"JC Stout","year":"2012","unstructured":"Stout JC, Jones R, Labuschagne I, O\u2019Regan AM, Say MJ, Dumas EM, Queller S, Justo D, Santos RD, Coleman A, Hart EP, Durr A, Leavitt BR, Roos RA, Langbehn DR, Tabrizi SJ, Frost C (2012) Evaluation of longitudinal 12 and 24 month cognitive outcomes in premanifest and early Huntington\u2019s disease. J Neurol Neurosurg Psychiatry 83:687\u2013694","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"11637_CR42","doi-asserted-by":"publisher","first-page":"1281","DOI":"10.1002\/mds.25964","volume":"29","author":"JC Stout","year":"2014","unstructured":"Stout JC, Queller S, Baker KN, Cowlishaw S, Sampaio C, Fitzer-Attas C, Borowsky B, Investigators H-C (2014) HD-CAB: a cognitive assessment battery for clinical trials in Huntington\u2019s disease 1,2,3. Mov Disord 29:1281\u20131288","journal-title":"Mov Disord"},{"key":"11637_CR43","doi-asserted-by":"publisher","first-page":"2215","DOI":"10.1007\/s00415-016-8252-7","volume":"263","author":"LP Selvadurai","year":"2016","unstructured":"Selvadurai LP, Harding IH, Corben LA, Stagnitti MR, Storey E, Egan GF, Delatycki MB, Georgiou-Karistianis N (2016) Cerebral and cerebellar grey matter atrophy in Friedreich ataxia: the IMAGE-FRDA study. J Neurol 263:2215\u20132223","journal-title":"J Neurol"},{"key":"11637_CR44","doi-asserted-by":"publisher","first-page":"747","DOI":"10.3389\/fneur.2018.00747","volume":"9","author":"M Vavla","year":"2018","unstructured":"Vavla M, Arrigoni F, Nordio A, De Luca A, Pizzighello S, Petacchi E, Paparella G, D\u2019Angelo MG, Brighina E, Russo E, Fantin M, Colombo P, Martinuzzi A (2018) Functional and structural brain damage in Friedreich\u2019s Ataxia. Front Neurol 9:747","journal-title":"Front Neurol"},{"key":"11637_CR45","doi-asserted-by":"publisher","first-page":"483","DOI":"10.1111\/ene.13843","volume":"26","author":"TJR Rezende","year":"2019","unstructured":"Rezende TJR, Martinez ARM, Faber I, Girotto Takazaki KA, Martins MP, de Lima FD, Lopes-Cendes I, Cendes F, Franca MC Jr (2019) Developmental and neurodegenerative damage in Friedreich\u2019s ataxia. Eur J Neurol 26:483\u2013489","journal-title":"Eur J Neurol"},{"key":"11637_CR46","doi-asserted-by":"publisher","first-page":"1182","DOI":"10.1093\/brain\/awv064","volume":"138","author":"MR Stefanescu","year":"2015","unstructured":"Stefanescu MR, Dohnalek M, Maderwald S, Thurling M, Minnerop M, Beck A, Schlamann M, Diedrichsen J, Ladd ME, Timmann D (2015) Structural and functional MRI abnormalities of cerebellar cortex and nuclei in SCA3, SCA6 and Friedreich\u2019s ataxia. Brain 138:1182\u20131197","journal-title":"Brain"},{"key":"11637_CR47","doi-asserted-by":"publisher","first-page":"97","DOI":"10.1186\/s12883-022-02615-3","volume":"22","author":"G Naeije","year":"2022","unstructured":"Naeije G, Schulz JB, Corben LA (2022) The cognitive profile of Friedreich ataxia: a systematic review and meta-analysis. BMC Neurol 22:97","journal-title":"BMC Neurol"},{"key":"11637_CR48","doi-asserted-by":"publisher","first-page":"525","DOI":"10.1007\/s00429-009-0208-6","volume":"213","author":"JS Damoiseaux","year":"2009","unstructured":"Damoiseaux JS, Greicius MD (2009) Greater than the sum of its parts: a review of studies combining structural connectivity and resting-state functional connectivity. Brain Struct Funct 213:525\u2013533","journal-title":"Brain Struct Funct"},{"key":"11637_CR49","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuroimage.2020.117609","volume":"226","author":"T Sarwar","year":"2021","unstructured":"Sarwar T, Tian Y, Yeo BTT, Ramamohanarao K, Zalesky A (2021) Structure-function coupling in the human connectome: a machine learning approach. Neuroimage 226:117609","journal-title":"Neuroimage"},{"key":"11637_CR50","doi-asserted-by":"publisher","first-page":"1226","DOI":"10.1089\/hum.2020.264","volume":"31","author":"IH Harding","year":"2020","unstructured":"Harding IH, Lynch DR, Koeppen AH, Pandolfo M (2020) Central nervous system therapeutic targets in Friedreich Ataxia. Hum Gene Ther 31:1226\u20131236","journal-title":"Hum Gene Ther"},{"key":"11637_CR51","doi-asserted-by":"publisher","first-page":"78","DOI":"10.1097\/NEN.0b013e31827e5762","volume":"72","author":"AH Koeppen","year":"2013","unstructured":"Koeppen AH, Mazurkiewicz JE (2013) Friedreich ataxia: neuropathology revised. J Neuropathol Exp Neurol 72:78\u201390","journal-title":"J Neuropathol Exp Neurol"},{"key":"11637_CR52","doi-asserted-by":"publisher","first-page":"1261","DOI":"10.1136\/jnnp-2015-312665","volume":"87","author":"IH Harding","year":"2016","unstructured":"Harding IH, Raniga P, Delatycki MB, Stagnitti MR, Corben LA, Storey E, Georgiou-Karistianis N, Egan GF (2016) Tissue atrophy and elevated iron concentration in the extrapyramidal motor system in Friedreich ataxia: the IMAGE-FRDA study. J Neurol Neurosurg Psychiatry 87:1261\u20131263","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"11637_CR53","doi-asserted-by":"publisher","first-page":"335","DOI":"10.1002\/mds.27606","volume":"34","author":"PGD Ward","year":"2019","unstructured":"Ward PGD, Harding IH, Close TG, Corben LA, Delatycki MB, Storey E, Georgiou-Karistianis N, Egan GF (2019) Longitudinal evaluation of iron concentration and atrophy in the dentate nuclei in friedreich ataxia. Mov Disord 34:335\u2013343","journal-title":"Mov Disord"}],"container-title":["Journal of Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00415-023-11637-x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s00415-023-11637-x\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00415-023-11637-x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,4,25]],"date-time":"2023-04-25T13:10:02Z","timestamp":1682428202000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s00415-023-11637-x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,3,1]]},"references-count":53,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2023,5]]}},"alternative-id":["11637"],"URL":"https:\/\/doi.org\/10.1007\/s00415-023-11637-x","relation":{},"ISSN":["0340-5354","1432-1459"],"issn-type":[{"value":"0340-5354","type":"print"},{"value":"1432-1459","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,3,1]]},"assertion":[{"value":"17 November 2022","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"7 February 2023","order":2,"name":"revised","label":"Revised","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"19 February 2023","order":3,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"1 March 2023","order":4,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"The authors declare that they have no conflicts of interest.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}}]}}