{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Maas RPPWM"],"funding":["Medical Research Council Canada","Fundação para a Ciência e a Tecnologia","Bundesministerium für Bildung und Forschung","EU Joint Programme – Neurodegenerative Disease Research","National Ataxia Foundation","Medical Research Council","Fundo Regional para a Ciência e Tecnologia"],"pagination":["1850-1860"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC9540189"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["37(9)"],"pubmed_abstract":["<h4>Background</h4>Disease severity in spinocerebellar ataxia type 3 (SCA3) is commonly defined by the Scale for the Assessment and Rating of Ataxia (SARA) sum score, but little is known about the contributions and progression patterns of individual items.<h4>Objectives</h4>To investigate the temporal dynamics of SARA item scores in SCA3 patients and evaluate if clinical and demographic factors are differentially associated with evolution of axial and appendicular ataxia.<h4>Methods</h4>In a prospective, multinational cohort study involving 11 European and 2 US sites, SARA scores were determined longitudinally in 223 SCA3 patients with a follow-up assessment after 1 year.<h4>Results</h4>An increase in SARA score from 10 to 20 points was mainly driven by axial and speech items, with a marke"],"journal":["Movement disorders : official journal of the Movement Disorder Society"],"pubmed_title":["Differential Temporal Dynamics of Axial and Appendicular Ataxia in SCA3."],"pmcid":["PMC9540189"],"funding_grant_id":["01ED1602A/B"],"pubmed_authors":["Klockgether T","Pires P","Infante J","Afonso Ribeiro J","van Gaalen J","Maas RPPWM","Bushara K","Giunti P","Faber J","Timmann D","van de Warrenburg BPC","Onyike C","Santana MM","Lima M","Hubener-Schmid J","Synofzik M","Garcia-Moreno H","Teerenstra S","Schols L","Pereira de Almeida L","Reetz K","de Vries JJ","Jacobi H"],"additional_accession":[]},"is_claimable":false,"name":"Differential Temporal Dynamics of Axial and Appendicular Ataxia in SCA3.","description":"<h4>Background</h4>Disease severity in spinocerebellar ataxia type 3 (SCA3) is commonly defined by the Scale for the Assessment and Rating of Ataxia (SARA) sum score, but little is known about the contributions and progression patterns of individual items.<h4>Objectives</h4>To investigate the temporal dynamics of SARA item scores in SCA3 patients and evaluate if clinical and demographic factors are differentially associated with evolution of axial and appendicular ataxia.<h4>Methods</h4>In a prospective, multinational cohort study involving 11 European and 2 US sites, SARA scores were determined longitudinally in 223 SCA3 patients with a follow-up assessment after 1 year.<h4>Results</h4>An increase in SARA score from 10 to 20 points was mainly driven by axial and speech items, with a marke","dates":{"release":"2022-01-01T00:00:00Z","publication":"2022 Sep","modification":"2025-04-22T02:00:44.497Z","creation":"2025-04-05T20:11:24.308Z"},"accession":"S-EPMC9540189","cross_references":{"pubmed":["35808813"],"doi":["10.1002/mds.29135"]}}