TY  - JOUR
AU  - Iskandar, Audrey
AU  - Buchholz, Maresa
AU  - Blotenberg, Iris
AU  - Schmitz-Hübsch, Tanja
AU  - Faber, Jennifer
AU  - Jacobi, Heike
AU  - Xie, Feng
AU  - Grobe-Einsler, Marcus
AU  - Klockgether, Thomas
AU  - Michalowsky, Bernhard
AU  - ESMI
TI  - Longitudinal description of health-related quality of life and depressive symptoms in polyQ spinocerebellar ataxia patients.
JO  - Journal of neurology
VL  - 272
IS  - 5
SN  - 0367-004X
CY  - [Darmstadt]
PB  - Steinkopff
M1  - DZNE-2025-00508
SP  - 323
PY  - 2025
AB  - 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.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
KW  - Humans
KW  - Female
KW  - Male
KW  - Quality of Life: psychology
KW  - Middle Aged
KW  - Spinocerebellar Ataxias: psychology
KW  - Spinocerebellar Ataxias: complications
KW  - Spinocerebellar Ataxias: genetics
KW  - Longitudinal Studies
KW  - Adult
KW  - Depression: etiology
KW  - Depression: psychology
KW  - Aged
KW  - Disease Progression
KW  - Severity of Illness Index
KW  - Registries
KW  - Peptides: genetics
KW  - Ataxia (Other)
KW  - Genetic disorder (Other)
KW  - Health-related quality of life (Other)
KW  - Patient-centered care (Other)
KW  - Psychologic well-being (Other)
KW  - Rare disease (Other)
KW  - polyglutamine (NLM Chemicals)
KW  - Peptides (NLM Chemicals)
LB  - PUB:(DE-HGF)16
C2  - pmc:PMC11982069
C6  - pmid:40202598
DO  - DOI:10.1007/s00415-025-13024-0
UR  - https://pub.dzne.de/record/277976
ER  -