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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., (© 2024. The Author(s).)

Objectives: To quantify health fluctuations, identify affected health-related quality of life (HRQoL) dimensions, and evaluate if fluctuations affect the HRQoL instruments recall period adherence in people living with dementia (PlwD)., Methods: Caregivers of PlwD completed a daily diary for 14 days, documenting if PlwD's health was better or worse than the day before and the affected HRQoL dimensions. Health fluctuation was categorized into low (0-4 fluctuations in 14 days), moderate (5-8), and high (9-14). Also, caregivers and PlwD completed the EQ-5D-5L (proxy- and self-reported) on days 1, 7, and 14. Subsequently, caregivers were interviewed to determine whether recurrent fluctuations were considered in the EQ-5D-5L assessment of today's health (recall period adherence)., Results: Fluctuations were reported for 96% of PlwD, on average, for 7 of the 14 days. Dimensions most frequently triggering fluctuations included memory, mobility, concentration, sleep, pain, and usual activities. Fluctuations were associated with higher EQ-5D-5L health-states variation and nonadherence to the EQ-5D-5L recall period "today." PlwD with moderate to high fluctuation had the highest EQ-5D-5L utility change between day 1 and 14 (0.157 and 0.134) and recall period nonadherence (31% and 26%) compared with PlwD with low fluctuation (0.010; 17%). Recall period nonadherence was higher in PlwD with improved compared with those with deteriorated health in the diary (37% vs 9%)., Conclusions: Health fluctuations frequently occur in dementia and strongly affect HRQoL assessments. Further research is needed to evaluate if more extended recall periods and multiple, consecutive assessments could capture health fluctuations more appropriately in dementia., Competing Interests: Author Disclosures Author disclosure forms can be accessed below in the Supplemental Material section., (Copyright © 2024. Published by Elsevier Inc.)

Although health-related quality of life (HRQoL) has developed into a crucial outcome parameter in clinical research, evidence of the EQ-5D-3L validation performance is lacking in patients with spinocerebellar ataxia (SCA) types 1, 2, 3, and 6. The objective of this study is to assess the acceptability, validity, reliability, and responsiveness of the EQ-5D-3L. For n = 842 predominantly European SCA patients of two longitudinal cohort studies, the EQ-5D-3L, PHQ-9 (Patient Health Questionnaire), and ataxia-specific clinical assessments (SARA: Scale for Assessment and Rating of Ataxia; ADL: activities of daily living as part of Friedreich's Ataxia Rating Scale; INAS: Inventory of Non-Ataxia Signs) were assessed at baseline and multiple annual follow-ups. The EQ-5D-3L was evaluated regarding acceptability, distribution properties, convergent and known-groups validity, test-retest reliability, and effect size measures to analyze health changes. The non-item response was low (EQ-5D-3L index: 0.8%; EQ-VAS: 3.4%). Ceiling effects occurred in 9.9% (EQ-5D-3L) and 3.0% (EQ-VAS) with a mean EQ-5D-3L index of 0.65 ± 0.21. In total, convergent validity showed moderate to strong Spearman's rho (r s > 0.3) coefficients comparing EQ-5D-3L and EQ-VAS with PHQ-9, SARA, ADL, and INAS. EQ-5D-3L could discriminate between groups of age, SARA, ADL, and INAS. Intra-class correlation coefficients (EQ-5D-3L ICC : 0.95/EQ-VAS ICC : 0.88) and Kappa statistics (range 0.44 to 0.93 for EQ-5D-3L items) indicated tolerable reliability. EQ-5D-3L shows small (effect size < 0.3) to moderate (effect size 0.3-0.59) health changes regarding ataxia severity. The analysis confirms an acceptable, reliable, valid, and responsive recommended EQ-5D-3L in SCA patients, measuring the HRQoL adequately, besides well-established clinical instruments., (© 2023. The Author(s).)

Introduction: The EQ-5D is a widely used health-related quality of life (HRQoL) instrument. The recall period "today" may miss out on recurrent health fluctuations often observed in people with dementia (PlwD). Thus, this study aims to assess the frequency of health fluctuations, affected HRQoL dimensions and the impact of the health fluctuations on the assessment of health today using the EQ-5D-5L., Methods and Analysis: This mixed-methods study will base on n=50 patient and caregiver dyads and four main study phases: (1) Baseline assessment of patients' socio-demographic and clinical characteristics; (2) caregivers self-completion of a daily diary for 14 days, documenting patient's today's health compared to yesterday, the affected HRQoL dimensions, and events that could have caused the fluctuations; (3) administration of the EQ-5D-5L as self- and proxy-rating at baseline, day seven and day 14; (4) interviewing caregivers on patient's health fluctuation, the consideration of past fluctuations in the assessment of health today using the EQ-5D-5L, and the appropriateness of recall periods to capture health fluctuations on day 14. Qualitative semi-structured interview data will be analyzed thematically. Quantitative analyses will be used to describe the frequency and intensity of health fluctuations, affected dimensions, and the association between health fluctuation and its consideration in the assessment of health today., Discussion: This study aims to reveal insights into the health fluctuation in dementia, the affected dimensions, and underlying health events, as well as whether individuals adhere to the recall period of health today using the EQ-5D-5L. This study will also provide information about more appropriate recall periods that could better capture health fluctuations., Trial Registration: This study is registered in the German Clinical Trials Register (DRKS00027956)., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Weber, Xie, Kohlmann, Mohr, Platen, Rädke, Kilimann, Engel and Michalowsky.)

Background: During a health survey in a remote area in southwest Colombia, it became apparent that a high percentage of the population suffered from chronic pruritus in association with high numbers of ticks and tickbites., Objective: To determine the clinical features and severity of tickbite-associated pruritus., Method: At twotime points - 8 weeks apart to account for seasonal effects - a cross-sectional study was conducted encompassing physical examination of the population, histological analysis of skin biopsies, and determining serum for antibodies against spotted fever (SFG) rickettsiae and typhus group (TG) rickettsiae. Ticks were identified using morphological criteria, and infection by rickettsiae was determined by PCR., Results: About 94.5% of the population (95% CI 92-97%) showed clinical signs of a pruritic arthropod reaction and of chronic pruritus with lichenoid papules and hyper- and hypopigmented nodules on otherwise noninflamed skin. Pruritus markedly impaired the quality of life in terms of sleeping disturbances. No signs for other diseases were observed. Chronic pruritus appeared to be because of repeated tickbites and scratching, but not because of other dermatological or medical conditions. Antibodies against SFG and TG-rickettsiae were detected at 79.0% (95% CI 73-86) and 3.6% (95% CI 0.7-6), respectively. Ticks were identified as Amblyomma cajennense., Conclusion: Remarkably high exposure to tick bites caused an unusually high rate of acute and chronic pruritus and markedly impaired quality of life of the investigated rural community. This underlines the necessity of public health measures and surveillance of rickettsial disease., (© 2020 The Authors. International Journal of Dermatology published by Wiley Periodicals LLC on behalf of the International Society of Dermatology.)

INTRODUCTION: The aim of this study was to analyze discrepancies between self‐ and proxy‐rated health‐related quality of life (HRQoL), measured with the EuroQol 5 Dimension 5 Level survey (EQ‐5D‐5L), in people living with dementia (PlwD) and their caregivers on an individual response level. METHODS: EQ‐5D‐5L, sociodemographic and clinical data were obtained from baseline data of n = 174 dyads of a cluster‐randomized, controlled intervention trial. Self‐ and proxy‐rated EQ‐5D‐5L health profiles were evaluated in terms of response distribution and agreement (weighted Kappa), and discrepancies in individual dimension level were analyzed using the Paretian Classification of Health Change (PCHC) as well as the presence and degree of inconsistencies between ratings. RESULTS: PlwD had a mean age of 80.1, nearly the half were female and 82.3% were mildly to moderately cognitively impaired. PlwD reported a higher utility index than caregiver proxies (mean 0.75 vs. 0.68, 83% of PlwD > 0.5). According to the PCHC and inconsistency approach, 95% of PlwD rated their health differently compared to proxies; 66% with divergent responses in at least three EQ‐5D‐5L dimensions. Nine dyads (5%) showed identical ratings. Discrepancies of one higher or lower EQ‐5D‐5L response represented the most frequent discrepancy (35.4%). Caregivers were two times more likely to report "moderate problems," representing the middle of the 5‐point Likert scale. Usual activities had the lowest agreement between ratings (weighted kappa = 0.23). In PlwD reporting no or some problems in EQ‐5D‐5L‐dimensions, proxies were more likely to report more problems and vice versa, especially in the more observable dimension usual activities and less likely in the less observable domains pain/discomfort and anxiety/depression. DISCUSSION: The central tendency bias observed in proxy‐ratings could be associated with assessment uncertainties, resulting in an underestimation (overestimation) in PlwD reporting better (worse) health. This diverging trend extends the knowledge from previous studies and underlines the need for more methodological research in this area. Highlights: People living with dementia (PlwD) rate their health differently than proxies.Proxy‐ratings over‐ or underestimate PlwD health when self‐ratings are low or high.Proxies indicate a possible central tendency bias.Further research is needed to understand influencing factors. [ABSTRACT FROM AUTHOR]