Health-related quality of life in patients with spinocerebellar ataxia

Sánchez-López, C.R.; Perestelo-Pérez, Lilisbeth; Escobar, Antonio; López-Bastida, Julio; Serrano‐Aguilar, Pedro

doi:10.1016/j.nrleng.2015.09.002

Cited by 14 publications

(16 citation statements)

References 20 publications

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“…Various ordinal options in each questions should firstly be calculated through a formula to obtain domain scores. This finding then reveals how FIM could also be used to assess overtime changes that would complement the changes in other ataxia specific tools, in which better mobility correlates with greater quality of life [25,53].…”

Section: Functional Measurement Toolsmentioning

confidence: 85%

International Classification of Functioning, Health and Disability (ICF) Conceptual Approach towards Spinocerebellar Ataxia

Triangto¹,

Setiono²,

Purba³

2022

Spinocerebellar Ataxia - Concepts, Particularities and Generalities

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Spinocerebellar Ataxia (SCA) is an autosomal dominant disease with progressive decline towards functional capacity. Although studies had shown that there are various SCA types, physical medicine and rehabilitation approach would focus mostly on functional aspects in each individuals. Analysis through International Classification of Functioning, Disability, and Health would assist clinicians to identify activity and participation aspects of SCA, mostly revolves around mobility function. Good correlation of mobility with quality of life was also reported, and thus it is only natural that this becomes the main focus of rehabilitative intervention. Approximately one hour physical exercise session focusing on postural control and balance was proven to be effective in improving disease related measurement tool, functional capacity, and quality of life. These benefits could be improved through newer therapies such as exercise games and virtual reality, virtually creates a rapidly changing environment, thus providing training through anticipatory actions. It is speculated that neuroplasticity through self-recognition of errors are the main physiology of recovery in SCA. Finally, it could be seen that rehabilitation intervention remains to be a cornerstone in current ataxia therapy, with goals of achieving exercise gains while alleviating the natural functional decline of the disease.

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Section: Functional Measurement Toolsmentioning

confidence: 85%

International Classification of Functioning, Health and Disability (ICF) Conceptual Approach towards Spinocerebellar Ataxia

Triangto¹,

Setiono²,

Purba³

2022

Spinocerebellar Ataxia - Concepts, Particularities and Generalities

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“…SF-36 includes eight each scored between 0 and 100, with 0 indicating worst health and 100 indicating the best health status possible. Previous studies have used SF-36 amongst patients with ataxia (11)(12)(13). The Chinese version of SF-36 was used in this study.…”

Section: Methodsmentioning

confidence: 99%

Cost of Cerebellar Ataxia in Hong Kong: A Retrospective Cost-of-Illness Analysis

et al. 2020

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Background: Cerebellar ataxia affects the coordination and balance of patients. The impact of this disease increases burden in patients, caregivers and society. Costs and the burden of this disease have not been investigated in Hong Kong. Objectives: (1) To estimate the socioeconomic cost of cerebellar ataxia in Hong Kong for the base year 2019, (2) to assess the health-related quality of life (HRQoL) and severity of ataxia, and (3) to establish the correlation between the severity and cost of cerebellar ataxia and to examine the correlation between the severity of cerebellar ataxia and HRQoL. Methods: A retrospective cross-sectional study was conducted amongst 31 patients with cerebellar ataxia. Cost-related data were obtained through self-reported questionnaires. The severity of ataxia was assessed using the Scale for Assessment and Rating of Ataxia, and HRQoL was assessed using the Short Form (36) Health Survey (SF-36). Pearson correlation was used for normally distributed data, whereas Spearman correlation was used otherwise. Results: The mean severity of ataxia was 21 out of 40. The average direct and indirect costs of a patient with ataxia in 6 months were HKD 51,371 and HKD 93,855, respectively. The mean difference between the independent to minimally dependent in activities of daily living (ADL) group and the moderate to maximally dependent in ADL group for direct and indirect costs was HKD 33,829 and HKD 51,444, respectively. Significant expenditure was related to production lost (42%), caregiver salary (17%), and in-patient care (16%). The physical functioning (r = −0.58) and general health (r = −0.41) of SF-36 were negatively correlated with disease severity (p < 0.05). A significant, positive correlation was found between disease severity and direct cost (Spearman's rho = 0.39) and the cost of hiring a caregiver (Spearman's rho = 0.43). Conclusion: The mean cost for 6 months for patients with cerebellar ataxia in Hong Kong is HKD 146,832. Additional support, including employment, access to specialist consultants, informal home care and community participation, are some areas that should be addressed. Future study on a larger population with a prospective design is necessary to confirm the aforementioned claims.

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“…The importance of carer welfare was underlined by a recent study which found that the presence of a carer was one of the main determinants to self-perceived quality of life in patients with spinocerebellar ataxia 11 . The current study represents the first to have investigated the views of both people with ataxia and their friends and relatives.…”

Section: Other Challengesmentioning

confidence: 99%

“…These include studies on specific diseases both at single points in time, e.g. children and adults with FRDA [6][7][8][9][10] and patients with autosomal dominant spinocerebellar ataxias (SCA) 11 , as well as over the course of several years to capture progression of the disorder, again involving patients with FRDA 12,13 and SCAs 14 . These studies have established reductions in HRQOL, the degree of which can often be linked to the severity of the disease.…”

Section: Introductionmentioning

confidence: 99%

Symptom burden of people with progressive ataxia, and its wider impact on their friends and relatives: a cross-sectional study

et al. 2021

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Background: Progressive ataxias are complex disorders that result in a wide variety of symptoms. Whilst we currently have a relatively good understanding of the symptom patterns associated with the various types of ataxia, and how these diseases progress over time, their impact on the person with ataxia is less well understood. In addition, little is known about how carers, friends and families are affected by them. This paper aims to provide preliminary information on the presence and impact of medical symptoms and day-to-day challenges on people with ataxia and their friends and relatives. Method: Data were extracted from a survey by Ataxia UK for their members. The views of 366 people with ataxia and 52 friends and relatives are reported. Data were analysed for the entire groups, as well as for the three most common ataxia types represented in the sample, Friedreich’s ataxia, inherited ataxia (excluding Friedreich’s ataxia), and cerebellar ataxia of unknown cause. Results: The survey confirmed the symptom patterns described in previous research, but further showed that the impact of these symptoms can vary across ataxia populations. Similar findings were observed for day-to-day challenges. Friends and relatives experienced similar challenges to people with ataxia, indicating that support provided has to consider those supporting people with ataxia as well as the patient. Respondents also highlighted limitations in terms of accessing support services, and not all services were able to cater fully to their specific needs. Conclusion: This study begins to provide information that can be used in further research to explore the needs of people with ataxia and their carers, friends, and relatives. Such research will support treatment trial design, ensuring patients’ needs are considered, help to tailor support services to their needs, and ensure health care professionals have the necessary skills to fully address them.

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Health-related quality of life in patients with spinocerebellar ataxia

Cited by 14 publications

References 20 publications

International Classification of Functioning, Health and Disability (ICF) Conceptual Approach towards Spinocerebellar Ataxia

International Classification of Functioning, Health and Disability (ICF) Conceptual Approach towards Spinocerebellar Ataxia

Cost of Cerebellar Ataxia in Hong Kong: A Retrospective Cost-of-Illness Analysis

Symptom burden of people with progressive ataxia, and its wider impact on their friends and relatives: a cross-sectional study

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