Quality of life in individuals with spinocerebellar ataxia type 10: a preliminary study

Santos, Laudiane Reis; Teive, Hélio Afonso Ghizoni; Neto, Francisco Diego Negrão Lopes; Macedo, Ana Carolina Brandt de; Mello, Neliana Maria de; Zonta, Marise Bueno

doi:10.1590/0004-282x20180077

Cited by 16 publications

(19 citation statements)

References 21 publications

(31 reference statements)

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“…Depression were consistently reported in several studies on SCA, and this could correlate significantly with quality of life [22,30]. As mentioned previously that mobility is the main concern, depression levels were also seen lower in those subjects with better mobility [6,25]. Even when other causes of depression may revolve around memory and learning ability disorders, most SCA subjects would have learning difficulty in limb control, which becomes a vicious cycle and a hazard for them to perform well in mobile activities of daily living [5].…”

Section: Environmental and Personal Factorsmentioning

confidence: 65%

“…A number of studies have also reported that difficulty in verbal memory, learning, and fluency are commonly seen [16,24]. These cognitive disorders would sum into a restriction in several community participation, but motor abilities still remained to inflict the most significant effect to quality of life in SCA [25]. Reports have mentioned the involvement of visuospatial and executive functioning abilities being reduced in SCA3, as it may correlate with reduced cerebellar perfusion [16].…”

Section: Activity and Participationmentioning

confidence: 99%

“…Functional test alternatives such as tandem gait for 5 meter test was suggested to be used, as it is very sensitive to changes post rehabilitation [18]. The tandem gait itself is a complex task which may not be performed well by all SCA subjects, therefore traditional balance assessments such as Berg Balance Scale (BBS) [17,25,44]. The BBS consist of 14 item list, with an ordinal scale of 0 to 4, higher number meaning better balance function.…”

Section: Functional Measurement Toolsmentioning

confidence: 99%

“…Aside from TUG test, a more comprehensive functional test tool such as functional independence measure (FIM) are commonly used in SCA studies [25]. The utilization of FIM had expanded the view on functional activities and illustrate their level of independence in those activities.…”

Section: Functional Measurement Toolsmentioning

confidence: 99%

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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: Environmental and Personal Factorsmentioning

confidence: 65%

Section: Activity and Participationmentioning

confidence: 99%

Section: Functional Measurement Toolsmentioning

confidence: 99%

Section: Functional Measurement Toolsmentioning

confidence: 99%

See 2 more Smart Citations

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

Triangto¹,

Setiono²,

Purba³

2022

Spinocerebellar Ataxia - Concepts, Particularities and Generalities

View full text Add to dashboard Cite

show abstract

“…People with ataxia have been shown to have significant static and dynamic imbalance, which can directly affect their self-care, transfers and locomotion functions (Aizawa et al, 2013). It can lead to a significant decrease in daily life activities and social participation and can increase the dependency of people (Santos et al, 2018). Basic BAT strengthens the individual's "body ego" and increases confidence in one's body (A. L. .…”

Section: S P E C I a L S E C T I O N : P H Y S I O T H E R A P Y F O mentioning

confidence: 99%

The impact of Basic Body Awareness Therapy on balance confidence and health-related quality of life in hunger strike victims with Wernicke Korsakoff Syndrome: pilot study

Yuce

Bahrilli

2020

torture

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Introduction: The aim of this study is to examine the short and long-term results of Basic Body Awareness Therapy (Basic BAT) on balance confidence and health-related quality of life in the people with Wernicke Korsakoff Syndrome (WKS) who survived hunger strike. Methods: A single case experimental design was used in the study. Basic BAT was applied to 4 people as a group therapy once a week for 12 weeks. Balance confidence was assessed with the Activities-specific Balance Con- fidence (ABC) Scale and health status was assessed with Health-related Quality of Life Questionnaire (SF-36 Short Form). Assess- ments were done before treatment, after treatment, at 3-month and 6-month follow-up. Results: 4 patients achieved clinically signifi- cant improvements on balance. All patients achieved significant change in physical functioning and role physical subcategories of SF-36. Visual analysis showed that the improvement in the patients continued after the treatment during the follow-up period. Conclusion: Basic BAT is effective as a physi- otherapy method in the treatment of cerebellar problems, balance disorders and gait incoordination seen in WKS. The Basic BAT method may provide a new perspective for the rehabilitation of survivors of hunger strikes with WKS.

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Cognitive Impairments in Spinocerebellar Ataxia Type 10 and Their Relation to Cortical Thickness

et al. 2021

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Background Spinocerebellar ataxia type 10 is a neurodegenerative disorder caused by the expansion of an ATTCT pentanucleotide repeat. Its clinical features include ataxia and, in some cases, epileptic seizures. There is, however, a dearth of information about its cognitive deficits and the neural bases underpinning them. Objectives The objectives of this study were to characterize the performance of spinocerebellar ataxia type 10 patients in 2 cognitive domains typically affected in spinocerebellar ataxias, memory and executive function, and to correlate the identified cognitive impairments with ataxia severity and cerebral/cerebellar cortical thickness, as quantified by MRI. Methods Memory and executive function tests were administered to 17 genetically confirmed Mexican spinocerebellar ataxia type 10 patients, and their results were compared with 17 healthy matched volunteers. MRI was performed in 16 patients. Results Patients showed deficits in visual and visuospatial short‐term memory, reduced storage capacity for verbal memory, and impaired monitoring, planning, and cognitive flexibility, which were ataxia independent. Patients with seizures (n = 9) and without seizures (n = 8) did not differ significantly in cognitive performance. There were significant correlations between short‐term visuospatial memory impairment and posterior cerebellar lobe cortical thickness (bilateral lobule VI, IX, and right X). Cognitive flexibility deficiencies correlated with cerebral cortical thickness in the left middle frontal, cingulate, opercular, and temporal gyri. Cerebellar cortical thickness in several bilateral regions was correlated with motor impairment. Conclusions Patients with spinocerebellar ataxia type 10 show significant memory and executive dysfunction that can be correlated with deterioration in the posterior lobe of the cerebellum and prefrontal, cingulate, and middle temporal cortices. © 2021 International Parkinson and Movement Disorder Society

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Quality of life in individuals with spinocerebellar ataxia type 10: a preliminary study

Abstract: In this sample, the greatest impairment of QoL in individuals with SCA10 was observed in "physical functioning" and "physical role".

Cited by 16 publications

References 21 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

The impact of Basic Body Awareness Therapy on balance confidence and health-related quality of life in hunger strike victims with Wernicke Korsakoff Syndrome: pilot study

Cognitive Impairments in Spinocerebellar Ataxia Type 10 and Their Relation to Cortical Thickness

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