AI Article Synopsis
- Health-related quality of life (HRQoL) in spinocerebellar ataxia (SCA) patients has been found to decline significantly over a three-year period, influenced by factors such as ataxia severity and mental health issues.
- The study involved longitudinal data from 310 SCA patients in one study and 525 in another, utilizing standardized clinical assessments to identify correlations and predictors of HRQoL decline.
- Key findings suggest that males and patients with earlier onset experience a more pronounced decrease in HRQoL, emphasizing the need for a focus on mental health and body weight management in their treatment plans.
Article 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). 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 = - 0.589; r = - 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.
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| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11269494 | PMC |
| http://dx.doi.org/10.1007/s12311-024-01657-2 | DOI Listing |
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