{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,5,19]],"date-time":"2025-05-19T13:10:01Z","timestamp":1747660201892,"version":"3.40.5"},"reference-count":60,"publisher":"Springer Science and Business Media LLC","issue":"5","license":[{"start":{"date-parts":[[2025,4,9]],"date-time":"2025-04-09T00:00:00Z","timestamp":1744156800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2025,4,9]],"date-time":"2025-04-09T00:00:00Z","timestamp":1744156800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"Deutsches Zentrum f\u00fcr Neurodegenerative Erkrankungen e.V. (DZNE) in der Helmholtz-Gemeinschaft"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J Neurol"],"published-print":{"date-parts":[[2025,5]]},"abstract":"<jats:title>Abstract<\/jats:title>\n          <jats:sec>\n            <jats:title>Introduction<\/jats:title>\n            <jats:p>Due to limited treatment options, managing symptoms has dominated care for Spinocerebellar Ataxia (SCA). Little attention has been given to health-related quality of life (HRQoL) and depressive symptoms experienced by patients across disease duration.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Objective<\/jats:title>\n            <jats:p>To investigate the course of HRQoL and the severity of depressive symptoms in SCA from disease onset to 26\u00a0years after onset and identify influencing factors.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Methods<\/jats:title>\n            <jats:p>We analyzed data from two longitudinal SCA cohorts, the EUROSCA (European Spinocerebellar Ataxia Registry) and ESMI study (European Spinocerebellar Ataxia Type 3\/Machado-Joseph Disease Initiative). Multilevel mixed-effects models were employed to demonstrate the course of HRQoL and depressive symptoms severity to investigate the role of disease progression with disease duration as a predictor of interest, along with time-varying clinical variables and time-fixed covariates.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Results<\/jats:title>\n            <jats:p>Seven hundred seventy four participants (M<jats:sub>age<\/jats:sub>\u2009=\u200950.8\u2009\u00b1\u200913.4; 48.6% female) were included. HRQoL consistently decreased throughout disease duration\u00a0across all SCA subtypes, but the decline was smallest in SCA6. The decrease in HRQoL was explained by ataxia and depression severity and driven by increasing problems with self-care, usual activities and mobility. Depressive symptoms significantly increased in SCA2 and 3 only, with a trend toward slight improvement in SCA6.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Conclusions<\/jats:title>\n            <jats:p>The trend direction of HRQoL and its significant association with the severity of ataxia symptoms align with the literature. The rapid worsening of self-care problems, the differential associations between depression and HRQoL sub-dimensions in different SCA subtypes, and the unexplainable resilience may warrant a deeper look at patient-specific intra- and interpersonal factors.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1007\/s00415-025-13024-0","type":"journal-article","created":{"date-parts":[[2025,4,9]],"date-time":"2025-04-09T13:21:45Z","timestamp":1744204905000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Longitudinal description of health-related quality of life and depressive symptoms in polyQ spinocerebellar ataxia 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The authors declare no competing interests. MB reports no disclosures. NW reports no disclosures. AR reports no disclosures. JF was funded as a fellow of the Cluster for Excellence in Clinical Neuroscience of the Hertie Foundation and received funding of the National Ataxia Foundation (NAF). TS-H receives research grants from Celgene\/bms and research grant from Hexal AG. HJ reports no disclosures. FX reports no disclosures. TK is receiving research support from the Bundesministerium f\u00fcr Bildung und Forschung (BMBF), the National Institutes of Health (NIH) and Servier. Within the last 24\u00a0months, he has received consulting fees from Biogen, UCB, and Vico Therapeutics. JF was funded within the Advanced Clinician Scientist Programme (ACCENT,\u00a0funded by the German Federal Ministry of Education and Research (BMBF);\u00a0funding code 01EO2107) and as a PI of the\u00a0iBehave Network, sponsored by the Ministry of Culture and Science of the State of North Rhine-Westphalia. JF received funding\u00a0from the National Ataxia Foundation (NAF) and\u00a0has received consultancy honoraria from Vico therapeutics, unrelated to the present manuscript. BvdW receives research support from ZonMw, Dutch Scientific Organization, Hersenstichting, and Christina Foundation; has served on scientific advisory boards and\/or did paid consultancy for Servier, Biohaven, Vico therapeutics, and Biogen; and receives royalties from BSL\/Springer-Nature. AT\u00a0received research grants\u00a0from\u00a0the\u00a0German Heredo-Ataxia Society (Deutsche Heredo-Ataxie-Gesellschaft e.V.), \u201cFreunde und F\u00f6rderer der Neurologie der Universit\u00e4tsmedizin Essen\u201d, travel grants from the German Academic\u00a0Exchange Service (Deutscher Akademischer Austauschdienst,\u00a0DAAD) and\u00a0MERCUR\/UA (Mercator Research Center Ruhr\/ Universit\u00e4tsallianz Ruhr), and\u00a0a scholarship of UMEA\/ DFG (University Medicine Essen Clinician Scientist Academy\u2014a program funded by the\u00a0German Research Foundation,\u00a0Deutsche Forschungsgemeinschaft; FU356\/12\u20131). MMS and LA were funded by the European Regional Development Fund (ERDF), through the Centro 2020 Regional Operational Programme under project CENTRO-01\u20130145-FEDER-181240 and through the COMPETE 2020\u2014Operational Programme for Competitiveness and Internationalisation and Portuguese national funds via FCT\u2014Funda\u00e7\u00e3o para a Ci\u00eancia e a Tecnologia, under projects 2022.06118.PTDC, UIDB\/04539\/2020, UIDP\/04539\/2020 and LA\/P\/0058\/2020, and European funds (GeneT GA 101059981) and by National Ataxia Foundation (NAF). PS was funded by FCT under the grant SFRH\/BD\/148451\/2019. PG is supported by the National Institute for Health Research University College London Hospitals Biomedical Research Centre UCLH. PG receives also support from the North Thames CRN. PG and HGM, work at University College London Hospitals\/ University College London, which receives a proportion of funding from the Department of Health\u2019s National Institute for Health Research Biomedical Research Centre\u2019s funding scheme. PG received funding from CureSCA3 in support of HGM work. PG has received grants and honoraria for advisory board from Vico Therapeutics, honoraria for advisory board from Triplet Therapeutics, grants and personal fees from Reata Pharmaceutical, grants from Wave.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflicts of interest"}},{"value":"ESMI and EUROSCA were approved by the ethics committees of the participating centers and have been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments. 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