{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T19:20:56Z","timestamp":1792783256229,"version":"4.5.0"},"reference-count":58,"publisher":"Springer Science and Business Media LLC","issue":"8","license":[{"start":{"date-parts":[[2022,4,1]],"date-time":"2022-04-01T00:00:00Z","timestamp":1648771200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2022,4,1]],"date-time":"2022-04-01T00:00:00Z","timestamp":1648771200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"publisher","award":["FU356\/12-1"],"award-info":[{"award-number":["FU356\/12-1"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"publisher","award":["441409627"],"award-info":[{"award-number":["441409627"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]},{"name":"European Joint Programme on Rare Diseases","award":["825575"],"award-info":[{"award-number":["825575"]}]},{"name":"German Heredoataxia Societey","award":["-"],"award-info":[{"award-number":["-"]}]},{"name":"Freunde and F\u00f6rderer der Neurologie am Universit\u00e4tsklinikum Essen","award":["-"],"award-info":[{"award-number":["-"]}],"id":[{"id":"https:\/\/ror.org\/02na8dn90","id-type":"ROR","asserted-by":"crossref"}]},{"DOI":"10.13039\/501100008349","name":"Universit\u00e4t Duisburg-Essen","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100008349","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J Neurol"],"published-print":{"date-parts":[[2022,8]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>\n                      A brief bedside test has recently been introduced by Hoche et al. (Brain, 2018) to screen for the\n                      <jats:italic>Cerebellar Cognitive Affective Syndrome<\/jats:italic>\n                      (CCAS) in patients with cerebellar disease.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>This multicenter study tested the ability of the CCAS-Scale to diagnose CCAS in individual patients with common forms of hereditary ataxia.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>A German version of the CCAS-Scale was applied in 30 SCA3, 14 SCA6 and 20 FRDA patients, and 64 healthy participants matched for age, sex, and level of education. Based on original cut-off values, the number of failed test items was assessed, and CCAS was considered possible (one failed item), probable (two failed items) or definite (three failed items). In addition a total sum raw score was calculated.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>On a group level, failed items were significantly higher and total sum scores were significantly lower in SCA3 patients compared to matched controls. SCA6 and FRDA patients performed numerically below controls, but respective group differences failed to reach significance. The ability of the CCAS-Scale to diagnose CCAS in individual patients was limited to severe cases failing three or more items. Milder cases failing one or two items showed a great overlap with the performance of controls exhibiting a substantial number of false-positive test results. The word fluency test items differentiated best between patients and controls.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>As a group, SCA3 patients performed below the level of SCA6 and FRDA patients, possibly reflecting additional cerebral involvement. Moreover, the application of the CCAS-Scale in its present form results in a high number of false-positive test results, that is identifying controls as patients, reducing its usefulness as a screening tool for CCAS in individual patients.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1007\/s00415-022-11071-5","type":"journal-article","created":{"date-parts":[[2022,4,1]],"date-time":"2022-04-01T16:48:09Z","timestamp":1648831689000},"page":"4363-4374","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":35,"title":["The CCAS-scale in hereditary ataxias: helpful on the group level, particularly in SCA3, but limited in individual patients"],"prefix":"10.1007","volume":"269","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6221-8601","authenticated-orcid":false,"given":"Andreas","family":"Thieme","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer","family":"Faber","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patricia","family":"Sulzer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathrin","family":"Reetz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Imis","family":"Dogan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miriam","family":"Barkhoff","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Janna","family":"Krahe","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Heike","family":"Jacobi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julia-Elisabeth","family":"Aktories","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martina","family":"Minnerop","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Saskia","family":"Elben","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raquel","family":"van der Veen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Johanna","family":"M\u00fcller","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giorgi","family":"Batsikadze","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J\u00fcrgen","family":"Konczak","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthis","family":"Synofzik","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sandra","family":"Roeske","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dagmar","family":"Timmann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2022,4,1]]},"reference":[{"key":"11071_CR1","doi-asserted-by":"publisher","first-page":"433","DOI":"10.1016\/s0074-7742(08)60363-3","volume":"41","author":"JD Schmahmann","year":"1997","unstructured":"Schmahmann JD, Sherman JC (1997) Cerebellar cognitive affective syndrome. Int Rev Neurobiol 41:433\u2013440. https:\/\/doi.org\/10.1016\/s0074-7742(08)60363-3","journal-title":"Int Rev Neurobiol"},{"key":"11071_CR2","doi-asserted-by":"publisher","first-page":"561","DOI":"10.1093\/brain\/121.4.561","volume":"121","author":"JD Schmahmann","year":"1998","unstructured":"Schmahmann JD, Sherman JC (1998) The cerebellar cognitive affective syndrome. Brain 121:561\u2013579. https:\/\/doi.org\/10.1093\/brain\/121.4.561","journal-title":"Brain"},{"key":"11071_CR3","doi-asserted-by":"publisher","first-page":"248","DOI":"10.1093\/brain\/awx317","volume":"141","author":"F Hoche","year":"2018","unstructured":"Hoche F, Guell X, Vangel MG, Sherman JC, Schmahmann JD (2018) The cerebellar cognitive affective\/ Schmahmann syndrome scale. Brain 141:248\u2013270. https:\/\/doi.org\/10.1093\/brain\/awx317","journal-title":"Brain"},{"key":"11071_CR4","doi-asserted-by":"publisher","first-page":"1371","DOI":"10.1038\/s41593-019-0436-x","volume":"22","author":"M King","year":"2019","unstructured":"King M, Hernandez-Castillo CR, Poldrack RA, Ivry RB, Diedrichsen J (2019) Functional boundaries in the human cerebellum revealed by a multi-domain task battery. Nat Neurosci 22:1371\u20131378. https:\/\/doi.org\/10.1038\/s41593-019-0436-x","journal-title":"Nat Neurosci"},{"key":"11071_CR5","doi-asserted-by":"publisher","first-page":"552","DOI":"10.1007\/s12311-016-0815-8","volume":"16","author":"M Adamaszek","year":"2017","unstructured":"Adamaszek M, D\u2019Agata F, Ferrucci R, Habas C, Keulen S, Kirkby KC, Leggio M, Marien P, Molinari M, Moulton E, Orsi L, Van Overwalle F, Papadelis C, Priori A, Sacchetti B, Schutter DJ, Styliadis C, Verhoeven J (2017) Consensus paper: cerebellum and emotion. Cerebellum 16:552\u2013576. https:\/\/doi.org\/10.1007\/s12311-016-0815-8","journal-title":"Cerebellum"},{"key":"11071_CR6","doi-asserted-by":"publisher","first-page":"102","DOI":"10.1007\/s12311-019-01068-8","volume":"19","author":"GPD Argyropoulos","year":"2020","unstructured":"Argyropoulos GPD, van Dun K, Adamaszek M, Leggio M, Manto M, Masciullo M, Molinari M, Stoodley CJ, Van Overwalle F, Ivry RB, Schmahmann JD (2020) The cerebellar cognitive affective\/schmahmann syndrome: a task force paper. Cerebellum 19:102\u2013125. https:\/\/doi.org\/10.1007\/s12311-019-01068-8","journal-title":"Cerebellum"},{"key":"11071_CR7","doi-asserted-by":"publisher","first-page":"3921","DOI":"10.1007\/s00415-021-10486-w","volume":"268","author":"H Jacobi","year":"2021","unstructured":"Jacobi H, Faber J, Timmann D, Klockgether T (2021) Update cerebellum and cognition. J Neurol 268:3921\u20133925. https:\/\/doi.org\/10.1007\/s00415-021-10486-w","journal-title":"J Neurol"},{"key":"11071_CR8","doi-asserted-by":"publisher","first-page":"437","DOI":"10.1016\/j.neuroimage.2018.01.082","volume":"172","author":"X Guell","year":"2018","unstructured":"Guell X, Gabrieli JDE, Schmahmann JD (2018) Triple representation of language, working memory, social and emotion processing in the cerebellum: convergent evidence from task and seed-based resting-state fMRI analyses in a single large cohort. Neuroimage 172:437\u2013449. https:\/\/doi.org\/10.1016\/j.neuroimage.2018.01.082","journal-title":"Neuroimage"},{"key":"11071_CR9","doi-asserted-by":"publisher","first-page":"1482","DOI":"10.1007\/s00415-003-0258-2","volume":"250","author":"C Globas","year":"2003","unstructured":"Globas C, Bosch S, Zuhlke C, Daum I, Dichgans J, Burk K (2003) The cerebellum and cognition. Intellectual function in spinocerebellar ataxia type 6 (SCA6). J Neurol 250:1482\u20131487. https:\/\/doi.org\/10.1007\/s00415-003-0258-2","journal-title":"J Neurol"},{"key":"11071_CR10","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. https:\/\/doi.org\/10.1002\/acn3.315","journal-title":"Ann Clin Transl Neurol"},{"key":"11071_CR11","doi-asserted-by":"publisher","first-page":"398","DOI":"10.1007\/s00415-008-0680-6","volume":"255","author":"P Garrard","year":"2008","unstructured":"Garrard P, Martin NH, Giunti P, Cipolotti L (2008) Cognitive and social cognitive functioning in spinocerebellar ataxia\u202f: a preliminary characterization. J Neurol 255:398\u2013405. https:\/\/doi.org\/10.1007\/s00415-008-0680-6","journal-title":"J Neurol"},{"key":"11071_CR12","doi-asserted-by":"publisher","first-page":"189","DOI":"10.1016\/j.neuropsychologia.2011.11.017","volume":"50","author":"FE Cooper","year":"2012","unstructured":"Cooper FE, Grube M, Von Kriegstein K, Kumar S, English P, Kelly TP, Chinnery PF, Griffiths TD (2012) Distinct critical cerebellar subregions for components of verbal working memory. Neuropsychologia 50:189\u2013197. https:\/\/doi.org\/10.1016\/j.neuropsychologia.2011.11.017","journal-title":"Neuropsychologia"},{"key":"11071_CR13","doi-asserted-by":"publisher","first-page":"496","DOI":"10.1136\/jnnp.2007.119883","volume":"79","author":"M Suenaga","year":"2008","unstructured":"Suenaga M, Kawai Y, Watanabe H, Atsuta N, Ito M, Tanaka F, Katsuno M, Fukatsu H, Naganawa S, Sobue G (2008) Cognitive impairment in spinocerebellar ataxia type 6. J Neurol Neurosurg Psychiatry 79:496\u2013499. https:\/\/doi.org\/10.1136\/jnnp.2007.119883","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"11071_CR14","doi-asserted-by":"publisher","first-page":"257","DOI":"10.1159\/000206850","volume":"61","author":"Y Kawai","year":"2009","unstructured":"Kawai Y, Suenaga M, Watanabe H, Sobue G (2009) Cognitive impairment in spinocerebellar degeneration. Eur Neurol 61:257\u2013268. https:\/\/doi.org\/10.1159\/000206850","journal-title":"Eur Neurol"},{"key":"11071_CR15","doi-asserted-by":"publisher","first-page":"870","DOI":"10.1002\/mds.2369","volume":"26","author":"T Schmitz-Huebsch","year":"2011","unstructured":"Schmitz-Huebsch T, Coudert M, Tezenas du Montcel S, Giunti P, Labrum R, Durr A, Ribai P, Charles P, Linnemann C, Schoels L, Rakowicz M, Rola R, Zdzienicka E, Fancellu R, Mariotti C, Baliko L, Melegh B, Filla A, Salvatore E, van de Warrenburg BP, Szymanski S, Infante J, Timmann D, Boesch S, Depondt C, Kang JS, Schulz JB, Klopstock T, Lossnitzer N, Lowe B, Frick C, Rottlander D, Schlaepfer TE, Klockgether T (2011) Depression comorbidity in spinocerebellar ataxia. Mov Disord 26:870\u2013876. https:\/\/doi.org\/10.1002\/mds.2369","journal-title":"Mov Disord"},{"key":"11071_CR16","doi-asserted-by":"publisher","first-page":"834","DOI":"10.1007\/s12311-012-0363-9","volume":"11","author":"A Nieto","year":"2012","unstructured":"Nieto A, Correia R, de N\u00f3brega E, Mont\u00f3n F, Hess S, Barroso J (2012) Cognition in Friedreich ataxia. Cerebellum 11:834\u2013844. https:\/\/doi.org\/10.1007\/s12311-012-0363-9","journal-title":"Cerebellum"},{"key":"11071_CR17","doi-asserted-by":"publisher","first-page":"1004","DOI":"10.1002\/mds.10033","volume":"17","author":"TM Zawacki","year":"2002","unstructured":"Zawacki TM, Grace J, Friedman JH, Sudarsky L (2002) Executive and emotional dysfunction in Machado-Joseph disease. Mov Disord 17:1004\u20131010. https:\/\/doi.org\/10.1002\/mds.10033","journal-title":"Mov Disord"},{"key":"11071_CR18","doi-asserted-by":"publisher","first-page":"2370","DOI":"10.1007\/s00415-013-6998-8","volume":"260","author":"TM Lopes","year":"2013","unstructured":"Lopes TM, D\u2019Abreu A, Fran\u00e7a MC, Yasuda CL, Betting LE, Samara AB, Castellano G, Somazz JC, Balthazar ML, Lopes-Cendes I, Cendes F (2013) Widespread neuronal damage and cognitive dysfunction in spinocerebellar ataxia type 3. J Neurol 260:2370\u20132379. https:\/\/doi.org\/10.1007\/s00415-013-6998-8","journal-title":"J Neurol"},{"key":"11071_CR19","doi-asserted-by":"publisher","first-page":"433","DOI":"10.1007\/s12311-010-0183-8","volume":"9","author":"I Klinke","year":"2010","unstructured":"Klinke I, Minnerop M, Schmitz-H\u00fcbsch T, Hendriks M, Klockgether T, W\u00fcllner U, Helmstaedter C (2010) Neuropsychological features of patients with spinocerebellar ataxia (SCA) types 1, 2, 3, and 6. Cerebellum 9:433\u2013442. https:\/\/doi.org\/10.1007\/s12311-010-0183-8","journal-title":"Cerebellum"},{"key":"11071_CR20","first-page":"5765","volume":"7","author":"J Ma","year":"2014","unstructured":"Ma J, Wu C, Lei J, Zhang X (2014) Cognitive impairments in patients with spinocerebellar ataxia types 1, 2 and 3 are positively correlated to the clinical severity of ataxia symptoms. Int J Clin Exp Med 7:5765\u20135771","journal-title":"Int J Clin Exp Med"},{"key":"11071_CR21","doi-asserted-by":"publisher","first-page":"600","DOI":"10.1007\/s12311-011-0276-z","volume":"10","author":"F D'Agata","year":"2011","unstructured":"D\u2019Agata F, Caroppo P, Baudino B, Caglio M, Croce M, Bergui M, Tamietto M, Mortara P, Orsi L (2011) The recognition of facial emotions in spinocerebellar ataxia patients. Cerebellum 10:600\u2013610. https:\/\/doi.org\/10.1007\/s12311-011-0276-z","journal-title":"Cerebellum"},{"key":"11071_CR22","doi-asserted-by":"publisher","first-page":"18","DOI":"10.1016\/j.ijchp.2017.11.004","volume":"18","author":"A Nieto","year":"2018","unstructured":"Nieto A, Hern\u00e1ndez-Torres A, P\u00e9rez-Flores J, Mont\u00f3n F (2018) Depressive symptoms in Friedreich ataxia. Int J Clin Health Psychol 18:18\u201326. https:\/\/doi.org\/10.1016\/j.ijchp.2017.11.004","journal-title":"Int J Clin Health Psychol"},{"key":"11071_CR23","doi-asserted-by":"publisher","first-page":"1037","DOI":"10.1007\/s12311-012-0354-x","volume":"11","author":"P Braga-Neto","year":"2012","unstructured":"Braga-Neto P, Dutra LA, Pedroso JL, Fel\u00edcio AC, Alessi H, Santos-Galduroz RF, Bertolucci PH, Castiglioni ML, Bressan RA, de Garrido GE, Barsottini OG, Jackowski A (2012) Cognitive deficits in Machado-Joseph disease correlate with hypoperfusion of visual system areas. Cerebellum 11:1037\u20131044. https:\/\/doi.org\/10.1007\/s12311-012-0354-x","journal-title":"Cerebellum"},{"key":"11071_CR24","doi-asserted-by":"publisher","first-page":"1757","DOI":"10.1001\/archneur.61.11.1757","volume":"61","author":"Y Kawai","year":"2004","unstructured":"Kawai Y, Takeda A, Abe Y, Washimi Y, Tanaka F, Sobue G (2004) Cognitive impairments in Machado-Joseph disease. Arch Neurol 61:1757\u20131760. https:\/\/doi.org\/10.1001\/archneur.61.11.1757","journal-title":"Arch Neurol"},{"key":"11071_CR25","doi-asserted-by":"publisher","first-page":"350","DOI":"10.1007\/s00415-019-09582-9","volume":"267","author":"S Cocozza","year":"2020","unstructured":"Cocozza S, Costabile T, Pontillo G, Lieto M, Russo C, Radice L, Pane C, Filla A, Brunetti A, Sacc\u00e0 F (2020) Cerebellum and cognition in Friedreich ataxia: a voxel-based morphometry and volumetric MRI study. J Neurol 267:350\u2013358. https:\/\/doi.org\/10.1007\/s00415-019-09582-9","journal-title":"J Neurol"},{"key":"11071_CR26","doi-asserted-by":"publisher","first-page":"504","DOI":"10.1007\/s12311-013-0457-z","volume":"12","author":"A Nieto","year":"2013","unstructured":"Nieto A, Correia R, de N\u00f3brega E, Mont\u00f3n F, Barroso J (2013) Cognition in late-onset Friedreich ataxia. Cerebellum 12:504\u2013512. https:\/\/doi.org\/10.1007\/s12311-013-0457-z","journal-title":"Cerebellum"},{"key":"11071_CR27","doi-asserted-by":"publisher","first-page":"1435","DOI":"10.1002\/mds.25512","volume":"28","author":"S Roeske","year":"2013","unstructured":"Roeske S, Filla I, Heim S, Amunts K, Helmstaedter C, W\u00fcllner U, Wagner M, Klockgether T, Minnerop M (2013) Progressive cognitive dysfunction in spinocerebellar ataxia type 3. Mov Disord 28:1435\u20131438. https:\/\/doi.org\/10.1002\/mds.25512","journal-title":"Mov Disord"},{"key":"11071_CR28","doi-asserted-by":"publisher","first-page":"260","DOI":"10.1007\/s00415-016-8344-4","volume":"264","author":"I Tamura","year":"2017","unstructured":"Tamura I, Takei A, Hamada S, Nonaka M, Kurosaki Y, Moriwaka F (2017) Cognitive dysfunction in patients with spinocerebellar ataxia type 6. J Neurol 264:260\u2013267. https:\/\/doi.org\/10.1007\/s00415-016-8344-4","journal-title":"J Neurol"},{"key":"11071_CR29","doi-asserted-by":"publisher","first-page":"25","DOI":"10.1016\/j.neuropsychologia.2017.10.036","volume":"107","author":"Z Rentiya","year":"2017","unstructured":"Rentiya Z, Khan NS, Ergun E, Ying SH, Desmond JE (2017) Distinct cerebellar regions related to motor and cognitive performance in SCA6 patients. Neuropsychologia 107:25\u201330. https:\/\/doi.org\/10.1016\/j.neuropsychologia.2017.10.036","journal-title":"Neuropsychologia"},{"key":"11071_CR30","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. https:\/\/doi.org\/10.1007\/s00415-016-8252-7","journal-title":"J Neurol"},{"key":"11071_CR31","doi-asserted-by":"publisher","unstructured":"Yap KH, Kessels RPC, Azmin S, van de Warrenburg B, Mohamed Ibrahim N (2021) Neurocognitive changes in spinocerebellar ataxia type 3: a systematic review with a narrative design. Cerebellum: Epub ahead of print. https:\/\/doi.org\/10.1007\/s12311-021-01282-3","DOI":"10.1007\/s12311-021-01282-3"},{"key":"11071_CR32","doi-asserted-by":"publisher","first-page":"39","DOI":"10.1186\/s42466-020-00071-3","volume":"2","author":"A Thieme","year":"2020","unstructured":"Thieme A, Roeske S, Faber J, Sulzer P, Minnerop M, Elben S, Jacobi H, Reetz K, Dogan I, Barkhoff M, Konczak J, Wondzinski E, Siebler M, Mueller O, Sure U, Schmahmann JD, Klockgether T, Synofzik M, Timmann D (2020) Validation of a German version of the Cerebellar Cognitive Affective\/ Schmahmann Syndrome Scale: preliminary version and study protocol. Neurol Res Pract 2:39. https:\/\/doi.org\/10.1186\/s42466-020-00071-3","journal-title":"Neurol Res Pract"},{"key":"11071_CR33","doi-asserted-by":"publisher","unstructured":"Rodr\u00edguez-Labrada R, Batista-Izquierdo A, Gonz\u00e1lez-Melix Z, Reynado-Cejas L, V\u00e1zquez- Mojena Y, Sanz YA, Canales-Ochoa N, Gonz\u00e1lez-Zald\u00edvar Y, Dogan I, Reetz K, Vel\u00e1zquez- P\u00e9rez L (2021) Cognitive decline is closely associated with ataxia severity in Spinocerebellar Ataxia Type 2: a Validation Study of the Schmahmann Syndrome Scale. Cerebellum: Epub ahead of print. https:\/\/doi.org\/10.1007\/s12311-021-01305-z","DOI":"10.1007\/s12311-021-01305-z"},{"key":"11071_CR34","doi-asserted-by":"publisher","first-page":"3456","DOI":"10.1007\/s00415-021-10516-7","volume":"268","author":"RPPW Maas","year":"2021","unstructured":"Maas RPPW, Killaars S, van de Warrenburg BPC, Schutter DJLG (2021) The cerebellar cognitive affective syndrome scale reveals early neuropsychological deficits in SCA3 patients. J Neurol 268:3456\u20133466. https:\/\/doi.org\/10.1007\/s00415-021-10516-7","journal-title":"J Neurol"},{"key":"11071_CR35","doi-asserted-by":"publisher","first-page":"e705","DOI":"10.1212\/WNL.0000000000008959","volume":"94","author":"CD Stephen","year":"2020","unstructured":"Stephen CD, Balkwill D, James P, Haxton E, Sassower K, Schmahmann JD, Eichler F, Lewis R (2020) Quantitative oculomotor and nonmotor assessments in late-onset GM2 gangliosidosis. Neurology 94:e705\u2013e717. https:\/\/doi.org\/10.1212\/WNL.0000000000008959","journal-title":"Neurology"},{"key":"11071_CR36","doi-asserted-by":"publisher","unstructured":"Chirino-P\u00e9rez A, Marrufo-Mel\u00e9ndez OR, Mu\u00f1oz-L\u00f3pez JI, Hernandez-Castillo CR, Ramirez- Garcia G, D\u00edaz R, Nu\u00f1ez-Orozco L, Fernandez-Ruiz J (2021) Mapping the cerebellar cognitive affective syndrome in patients with chronic cerebellar strokes. Cerebellum: Epub ahead of print. https:\/\/doi.org\/10.1007\/s12311-021-01290-3","DOI":"10.1007\/s12311-021-01290-3"},{"key":"11071_CR37","doi-asserted-by":"publisher","first-page":"1050","DOI":"10.1002\/acn3.51079","volume":"7","author":"G Naeije","year":"2020","unstructured":"Naeije G, Rai M, Allaerts N, Sjogard M, De Ti\u00e8ge X, Pandolfo M (2020) Cerebellar cognitive disorder parallels cerebellar motor symptoms in Friedreich ataxia. Ann Clin Transl Neurol 7:1050\u20131054. https:\/\/doi.org\/10.1002\/acn3.51079","journal-title":"Ann Clin Transl Neurol"},{"key":"11071_CR38","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awaa41","volume":"144","author":"A Thieme","year":"2021","unstructured":"Thieme A, R\u00f6ske S, Faber J, Sulzer P, Minnerop M, Elben S, Reetz K, Dogan I, Barkhoff M, Konczak J, Wondzinski E, Siebler M, Hetze S, M\u00fcller O, Sure U, Klockgether T, Synofzik M, Timmann D (2021) Reference values for the cerebellar cognitive affective syndrome scale: age and education matter. Brain 144:e20. https:\/\/doi.org\/10.1093\/brain\/awaa41","journal-title":"Brain"},{"key":"11071_CR39","doi-asserted-by":"publisher","first-page":"1717","DOI":"10.1212\/01.wnl.0000219042.60538.92","volume":"66","author":"T Schmitz-Huebsch","year":"2006","unstructured":"Schmitz-Huebsch 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, Schoels 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. https:\/\/doi.org\/10.1212\/01.wnl.0000219042.60538.92","journal-title":"Neurology"},{"key":"11071_CR40","doi-asserted-by":"publisher","first-page":"418","DOI":"10.1007\/s12311-012-0421-3","volume":"12","author":"H Jacobi","year":"2013","unstructured":"Jacobi H, Rakowicz M, Rola R, Fancellu R, Mariotti C, Charles P, Durr A, Kueper M, Timmann D, Linnemann C, Schoels L, Kaut O, Schaub C, Filla A, Baliko L, Melegh B, Kang JS, Giunti P, van de Warrenburg BP, Fimmers R, Klockgether T (2013) Inventory of Non-Ataxia Signs (INAS): validation of a new clinical assessment instrument. Cerebellum 12:418\u2013428. https:\/\/doi.org\/10.1007\/s12311-012-0421-3","journal-title":"Cerebellum"},{"key":"11071_CR41","doi-asserted-by":"publisher","first-page":"565","DOI":"10.1038\/s41592-019-0470-3","volume":"16","author":"J Ho","year":"2019","unstructured":"Ho J, Tumkaya T, Aryal S, Choi H, Claridge-Chang A (2019) Moving beyond P values: data analysis with estimation graphics. Nat Methods 16:565\u2013566. https:\/\/doi.org\/10.1038\/s41592-019-0470-3","journal-title":"Nat Methods"},{"key":"11071_CR42","doi-asserted-by":"publisher","first-page":"506","DOI":"10.1093\/fampra\/9.4.506","volume":"9","author":"YT Van der Schouw","year":"1992","unstructured":"Van der Schouw YT, Verbeek AL, Ruijs JH (1992) ROC curves for the initial assessment of new diagnostic tests. Fam Pract 9:506\u2013511. https:\/\/doi.org\/10.1093\/fampra\/9.4.506","journal-title":"Fam Pract"},{"key":"11071_CR43","doi-asserted-by":"publisher","first-page":"11","DOI":"10.1016\/j.gheart.2013.01.001","volume":"8","author":"RB D'Agostino","year":"2013","unstructured":"D\u2019Agostino RB, Pencina MJ, Massaro JM, Coady S (2013) Cardiovascular disease risk assessment: insights from Framingham. Glob Heart 8:11\u201323. https:\/\/doi.org\/10.1016\/j.gheart.2013.01.001","journal-title":"Glob Heart"},{"key":"11071_CR44","doi-asserted-by":"publisher","first-page":"926","DOI":"10.1007\/s00261-015-0574-x","volume":"41","author":"R Faletti","year":"2016","unstructured":"Faletti R, Battisti G, Discalzi A, Grognardi ML, Martinello S, Oderda M, Gontero P, Bergamasco L, Cassinis MC, Fonio P (2016) Can DW-MRI, with its ADC values, be a reliable predictor of biopsy outcome in patients with suspected prostate cancer? Abdom Radiol (NY) 41:926\u2013933. https:\/\/doi.org\/10.1007\/s00261-015-0574-x","journal-title":"Abdom Radiol (NY)"},{"key":"11071_CR45","doi-asserted-by":"publisher","first-page":"34","DOI":"10.1016\/j.jclinepi.2006.03.012","volume":"60","author":"CB Terwee","year":"2007","unstructured":"Terwee CB, Bot SD, de Boer MR, van der Windt DA, Knol DL, Dekker J, Bouter LM, de Vet HC (2007) Quality criteria were proposed for measurement properties of health status questionnaires. J Clin Epidemiol 60:34\u201342. https:\/\/doi.org\/10.1016\/j.jclinepi.2006.03.012","journal-title":"J Clin Epidemiol"},{"key":"11071_CR46","doi-asserted-by":"publisher","first-page":"32","DOI":"10.1002\/1097-0142(1950)3:1<32:aid-cncr2820030106>3.0.co;2-3","volume":"3","author":"WJ Youden","year":"1950","unstructured":"Youden WJ (1950) Index for rating diagnostic tests. Cancer 3:32\u201335. https:\/\/doi.org\/10.1002\/1097-0142(1950)3:1%3c32:aid-cncr2820030106%3e3.0.co;2-3","journal-title":"Cancer"},{"key":"11071_CR47","doi-asserted-by":"publisher","first-page":"549","DOI":"10.1007\/s12311-011-0318-6","volume":"11","author":"P Braga-Neto","year":"2012","unstructured":"Braga-Neto P, Pedroso JL, Alessi H, Dutra LA, Felicio AC, Minett T, Weisman P, Santos-Galduroz RF, Bertolucci PH, Gabbai AA, Barsottini OG (2012) Cerebellar cognitive affective syndrome in Machado Joseph disease: core clinical features. Cerebellum 11:549\u2013556. https:\/\/doi.org\/10.1007\/s12311-011-0318-6","journal-title":"Cerebellum"},{"key":"11071_CR48","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. https:\/\/doi.org\/10.1146\/annurev.neuro.31.060407.125606","journal-title":"Annu Rev Neurosci"},{"key":"11071_CR49","doi-asserted-by":"publisher","first-page":"4348","DOI":"10.1002\/hbm.25551","volume":"42","author":"F Palesi","year":"2021","unstructured":"Palesi F, Ferrante M, Gaviraghi M, Misiti A, Savini G, Lascialfari A, D\u2019Angelo E, Gandini Wheeler-Kingshott CAM (2021) Motor and higher-order functions topography of the human dentate nuclei identified with tractography and clustering methods. Hum Brain Mapp 42:4348\u20134361. https:\/\/doi.org\/10.1002\/hbm.25551","journal-title":"Hum Brain Mapp"},{"key":"11071_CR50","doi-asserted-by":"publisher","first-page":"3672","DOI":"10.1093\/brain\/awr266","volume":"134","author":"AM Tedesco","year":"2011","unstructured":"Tedesco AM, Chiricozzi FR, Clausi S, Lupo M, Molinari M, Leggio MG (2011) The cerebellar cognitive profile. Brain 134:3672\u20133686. https:\/\/doi.org\/10.1093\/brain\/awr266","journal-title":"Brain"},{"key":"11071_CR51","doi-asserted-by":"publisher","first-page":"493","DOI":"10.1007\/s12311-013-0456-0","volume":"12","author":"AH Koeppen","year":"2013","unstructured":"Koeppen AH, Ramirez RL, Bjork ST, Bauer P, Feustel PJ (2013) The reciprocal cerebellar circuitry in human hereditary ataxia. Cerebellum 12:493\u2013503. https:\/\/doi.org\/10.1007\/s12311-013-0456-0","journal-title":"Cerebellum"},{"key":"11071_CR52","unstructured":"Bidichandani SI, Delatycki MB (1993\u20132021) Friedreich Ataxia. In: Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Stephens K, Amemiya A (eds) Gene Reviews [Internet]"},{"key":"11071_CR53","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, Th\u00fcrling 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. https:\/\/doi.org\/10.1093\/brain\/awv064","journal-title":"Brain"},{"key":"11071_CR54","doi-asserted-by":"publisher","first-page":"343","DOI":"10.1017\/S1355617720000958","volume":"27","author":"A Hern\u00e1ndez-Torres","year":"2021","unstructured":"Hern\u00e1ndez-Torres A, Mont\u00f3n F, Hess Medler S, de N\u00f3brega \u00c9, Nieto A (2021) Longitudinal study of cognitive functioning in Friedreich\u2019s Ataxia. J Int Neuropsychol Soc 27:343\u2013350. https:\/\/doi.org\/10.1017\/S1355617720000958","journal-title":"J Int Neuropsychol Soc"},{"key":"11071_CR55","doi-asserted-by":"publisher","first-page":"204","DOI":"10.1007\/s12311-017-0890-5","volume":"17","author":"S Sayah","year":"2018","unstructured":"Sayah S, Rotg\u00e9 JY, Francisque H, Gargiulo M, Czernecki V, Justo D, Lahlou-Laforet K, Hahn V, Pandolfo M, Pelissolo A, Fossati P, Durr A (2018) Personality and neuropsychological profiles in Friedreich Ataxia. Cerebellum 17:204\u2013212. https:\/\/doi.org\/10.1007\/s12311-017-0890-5","journal-title":"Cerebellum"},{"key":"11071_CR56","doi-asserted-by":"publisher","first-page":"182","DOI":"10.1080\/00207454.2017.1377198","volume":"128","author":"F Giocondo","year":"2018","unstructured":"Giocondo F, Curcio G (2018) Spinocerebellar ataxia: a critical review of cognitive and sociocognitive deficits. Int J Neurosci 128:182\u2013191. https:\/\/doi.org\/10.1080\/00207454.2017.1377198","journal-title":"Int J Neurosci"},{"key":"11071_CR57","doi-asserted-by":"publisher","first-page":"549","DOI":"10.1093\/scan\/nsz032","volume":"14","author":"F Van Overwalle","year":"2019","unstructured":"Van Overwalle F, De Coninck S, Heleven E, Perrotta G, Taib NOB, Manto M, Marien P (2019) The role of the cerebellum in reconstructing social action sequences: a pilot study. Soc Cogn Affect Neurosci 14:549\u2013558. https:\/\/doi.org\/10.1093\/scan\/nsz032","journal-title":"Soc Cogn Affect Neurosci"},{"key":"11071_CR58","doi-asserted-by":"publisher","first-page":"182","DOI":"10.1007\/s12311-019-01094-6","volume":"19","author":"R Shishegar","year":"2020","unstructured":"Shishegar R, Harding IH, Corben LA, Delatycki MB, Storey E, Egan GF, Georgiou-Karistianis N (2020) Longitudinal increases in cerebral brain activation during working memory performance in Friedreich Ataxia: 24-month data from IMAGE-FRDA. Cerebellum 19:182\u2013191. https:\/\/doi.org\/10.1007\/s12311-019-01094-6","journal-title":"Cerebellum"}],"container-title":["Journal of Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00415-022-11071-5.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s00415-022-11071-5\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00415-022-11071-5.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,7,18]],"date-time":"2022-07-18T12:31:43Z","timestamp":1658147503000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s00415-022-11071-5"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,4,1]]},"references-count":58,"journal-issue":{"issue":"8","published-print":{"date-parts":[[2022,8]]}},"alternative-id":["11071"],"URL":"https:\/\/doi.org\/10.1007\/s00415-022-11071-5","relation":{},"ISSN":["0340-5354","1432-1459"],"issn-type":[{"value":"0340-5354","type":"print"},{"value":"1432-1459","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,4,1]]},"assertion":[{"value":"11 November 2021","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"22 February 2022","order":2,"name":"revised","label":"Revised","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"23 February 2022","order":3,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"1 April 2022","order":4,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"All authors declare no financial disclosures or conflicts of interest other than the funding stated below concerning the research covered in this manuscript.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}},{"value":"The study has prospectively been registered at the German Clinical Study Register (\n                      \n                      ; DRKS-ID: DRKS00016854).","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Study registration"}},{"value":"This study was approved by the local ethics committees of the participating sites and performed in accordance with the ethical standards laid down in the Declaration of Helsinki of 1964 and its later amendments.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical approval"}},{"value":"Participants\u2019 consent was obtained prior to any study related procedures.","order":5,"name":"Ethics","group":{"name":"EthicsHeading","label":"Informed consent"}}]}}