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000277976 1001_ $$0P:(DE-2719)9003158$$aIskandar, Audrey$$b0$$eFirst author$$udzne
000277976 245__ $$aLongitudinal description of health-related quality of life and depressive symptoms in polyQ spinocerebellar ataxia patients.
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000277976 520__ $$aDue to limited treatment options, managing symptoms has dominated care for Spinocerebellar Ataxia (SCA). Little attention has been given to health-related quality of life (HRQoL) and depressive symptoms experienced by patients across disease duration.To investigate the course of HRQoL and the severity of depressive symptoms in SCA from disease onset to 26 years after onset and identify influencing factors.We analyzed data from two longitudinal SCA cohorts, the EUROSCA (European Spinocerebellar Ataxia Registry) and ESMI study (European Spinocerebellar Ataxia Type 3/Machado-Joseph Disease Initiative). Multilevel mixed-effects models were employed to demonstrate the course of HRQoL and depressive symptoms severity to investigate the role of disease progression with disease duration as a predictor of interest, along with time-varying clinical variables and time-fixed covariates.Seven hundred seventy four participants (Mage = 50.8 ± 13.4; 48.6% female) were included. HRQoL consistently decreased throughout disease duration across all SCA subtypes, but the decline was smallest in SCA6. The decrease in HRQoL was explained by ataxia and depression severity and driven by increasing problems with self-care, usual activities and mobility. Depressive symptoms significantly increased in SCA2 and 3 only, with a trend toward slight improvement in SCA6.The trend direction of HRQoL and its significant association with the severity of ataxia symptoms align with the literature. The rapid worsening of self-care problems, the differential associations between depression and HRQoL sub-dimensions in different SCA subtypes, and the unexplainable resilience may warrant a deeper look at patient-specific intra- and interpersonal factors.
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000277976 650_7 $$2Other$$aAtaxia
000277976 650_7 $$2Other$$aGenetic disorder
000277976 650_7 $$2Other$$aHealth-related quality of life
000277976 650_7 $$2Other$$aPatient-centered care
000277976 650_7 $$2Other$$aPsychologic well-being
000277976 650_7 $$2Other$$aRare disease
000277976 650_7 $$026700-71-0$$2NLM Chemicals$$apolyglutamine
000277976 650_7 $$2NLM Chemicals$$aPeptides
000277976 650_2 $$2MeSH$$aHumans
000277976 650_2 $$2MeSH$$aFemale
000277976 650_2 $$2MeSH$$aMale
000277976 650_2 $$2MeSH$$aQuality of Life: psychology
000277976 650_2 $$2MeSH$$aMiddle Aged
000277976 650_2 $$2MeSH$$aSpinocerebellar Ataxias: psychology
000277976 650_2 $$2MeSH$$aSpinocerebellar Ataxias: complications
000277976 650_2 $$2MeSH$$aSpinocerebellar Ataxias: genetics
000277976 650_2 $$2MeSH$$aLongitudinal Studies
000277976 650_2 $$2MeSH$$aAdult
000277976 650_2 $$2MeSH$$aDepression: etiology
000277976 650_2 $$2MeSH$$aDepression: psychology
000277976 650_2 $$2MeSH$$aAged
000277976 650_2 $$2MeSH$$aDisease Progression
000277976 650_2 $$2MeSH$$aSeverity of Illness Index
000277976 650_2 $$2MeSH$$aRegistries
000277976 650_2 $$2MeSH$$aPeptides: genetics
000277976 693__ $$0EXP:(DE-2719)ESMI-20140101$$5EXP:(DE-2719)ESMI-20140101$$eEuropean Spinocerebellar Ataxia Type 3/Machado-Joseph Disease Initiative$$x0
000277976 7001_ $$0P:(DE-2719)9002138$$aBuchholz, Maresa$$b1$$udzne
000277976 7001_ $$0P:(DE-2719)9001870$$aBlotenberg, Iris$$b2$$udzne
000277976 7001_ $$0P:(DE-2719)9000288$$aSchmitz-Hübsch, Tanja$$b3
000277976 7001_ $$0P:(DE-2719)2811327$$aFaber, Jennifer$$b4$$udzne
000277976 7001_ $$0P:(DE-2719)2811564$$aJacobi, Heike$$b5
000277976 7001_ $$0P:(DE-HGF)0$$aXie, Feng$$b6
000277976 7001_ $$0P:(DE-2719)9001510$$aGrobe-Einsler, Marcus$$b7$$udzne
000277976 7001_ $$0P:(DE-2719)2810314$$aKlockgether, Thomas$$b8$$udzne
000277976 7001_ $$0P:(DE-2719)2810763$$aMichalowsky, Bernhard$$b9$$eLast author$$udzne
000277976 7001_ $$aESMI$$b10
000277976 7001_ $$agroups, EUROSCA Study$$b11$$eCollaboration Author
000277976 7001_ $$aReetz, Kathrin$$b12$$eContributor
000277976 7001_ $$aRaponso, Mafalda$$b13$$eContributor
000277976 7001_ $$aGonzales, Carlos$$b14$$eContributor
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000277976 7001_ $$aRakowicz, Maria$$b47$$eContributor
000277976 7001_ $$aSulek, Anna$$b48$$eContributor
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000277976 773__ $$0PERI:(DE-600)1421299-7$$a10.1007/s00415-025-13024-0$$gVol. 272, no. 5, p. 323$$n5$$p323$$tJournal of neurology$$v272$$x0367-004X$$y2025
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