Journal Article
Factors Influencing Health-Related Quality of Life of Patients with Spinocerebellar Ataxia
Niklas Weber; Maresa Buchholz; Anika Rädke; Jennifer Faber; Tanja Schmitz-Hübsch; Heike Jacobi; Thomas Klockgether; Wolfgang Hoffmann; Bernhard Michalowsky; Sophie Tezenas du Montcel; Peter Bauer; Paola Giunti; Arron Cook; Robyn Labrum; Michael H. Parkinson; Alexandra Durr; Alexis Brice; Perrine Charles; Cecilia Marelli; Caterina Mariotti; Lorenzo Nanetti; Marta Panzeri; Maria Rakowicz; Anna Sulek; Anna Sobanska; Ludger Schöls; Holger Hengel; Laszlo Baliko; Bela Melegh; Alessandro Filla; Antonella Antenora; Jon Infante; José Berciano; Bart P. van de Warrenburg; Dagmar Timmann; Sandra Szymanski; Sylvia Boesch; Jun-Suk Kang; Massimo Pandolfo; Jörg B. Schulz; Sonia Molho; Alhassane Diallo; Jeanette Hübener-Schmid; Magda Santana; Marcus Grobe-Einsler; Berkan Koyak; Mafalda Raposo; Manuela Lima; Hector Garcia-Moreno; Paola Giunti; Luís Pereira de Almeida; Bart van de Warrenburg; Judith van Gaalen; Dagmar Timmann; Andreas Thieme; Kathrin Reetz; Imis Dogan; Carlo Wilke; Ludger Schöls; Olaf Riess; Matthis Synofzik; Jeroen de Vries; Jon Infante; Oz Gulin; James Joers; Chiadikaobi Onyike; Michal Povazan; Eva-Maria Ratai; Jeremy Schmahmann
The Cerebellum · Vol. 23, Issue 4 · pp. 1466-1477 · 2024
Abstract
Background Little is known about the progression of health-related quality of life (HRQoL) and predicting factors in spinocerebellar ataxia (SCA). Such knowledge is crucial to identify modifiable factors promoting everyday life with SCA and attenuating HRQoL decline. Objectives This study is to assess HRQoL progression and identify factors affecting SCA patients’ HRQoL. Methods Longitudinal data (three-year follow-up) of 310 SCA patients of the European SCA3/Machado-Joseph-Disease Initiative (ESMI) (2016-2022) and 525 SCA patients (SCA1, SCA2, SCA3 or SCA6) of the EUROSCA natural history study cohort (2006–2015) were assessed. Both large cohort studies share standardized assessments of clinical measures, SARA, INAS, PHQ-9, and HRQoL (EQ-5D-3L). The association between HRQoL and clinical measures was assessed by Spearman Correlation (r s ). Multivariable panel regression models were performed to evaluate the impact of patients’ socio-demographics, age of onset, SCA type and body mass index (BMI), and clinical measures on HRQoL progression. Results HRQoL significantly decreased over one (− 0.014, p = 0.095), two (− 0.028, p = 0.003), and three years (− 0.032, p = 0.002). Ataxia severity and mental health strongly correlated with HRQoL (r s SARA = − 0.589; r s PHQ-9 = − 0.507). HRQoL more intensively declined in male (ß = − 0.024, p = 0.038) patients with an earlier age of onset (ß = 0.002, p = 0.058). Higher progression of ataxia severity (ß = − 0.010, p ≤ 0.001), mental health problems (ß = − 0.012, p < 0.001), and higher BMI (ß = − 0.003, p = 0.029) caused more severe decline of patients’ HRQoL over time. Discussion In absence of curative treatments, stronger focus on mental health and weight influence could help clinical evaluation and accompany treatment improving SCA patients’ HRQoL, especially in male patients with early disease onset.