biostudies-literatureMaas RPPWMMedical Research Council CanadaFundação para a Ciência e a TecnologiaBundesministerium für Bildung und ForschungEU Joint Programme – Neurodegenerative Disease ResearchNational Ataxia FoundationMedical Research CouncilFundo Regional para a Ciência e Tecnologia1850-1860https://www.ebi.ac.uk/biostudies/studies/S-EPMC9540189biostudies-literatureUnknown37(9)<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 markeMovement disorders : official journal of the Movement Disorder SocietyDifferential Temporal Dynamics of Axial and Appendicular Ataxia in SCA3.PMC954018901ED1602A/BKlockgether TPires PInfante JAfonso Ribeiro Jvan Gaalen JMaas RPPWMBushara KGiunti PFaber JTimmann Dvan de Warrenburg BPCOnyike CSantana MMLima MHubener-Schmid JSynofzik MGarcia-Moreno HTeerenstra SSchols LPereira de Almeida LReetz Kde Vries JJJacobi HfalseDifferential Temporal Dynamics of Axial and Appendicular Ataxia in SCA3.<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 marke2022-01-01T00:00:00Z2022 Sep2025-04-22T02:00:44.497Z2025-04-05T20:11:24.308ZS-EPMC95401893580881310.1002/mds.29135