Functional capacity in spinocerebellar ataxias

Abstract

Introduction: Spinocerebellar ataxia (SCA) is a degenerative autosomal dominant disorder that causes impairments in balance, coordination and gait. These changes limit the independence to perform daily activities (ADLs) and reduce the individuals participation, thus leading them to a sedentary lifestyle and reducing their cardiopulmonary fitness. Viable evaluation instruments that investigate functional capacity to perform ADLs are necessary to understand the impact of these changes on patients' functionality. The Glittre-ADL (T-Glittre) test is an easy-to-apply, low-cost option that reproduces AVDs close to reality. Objectives: The aims of this study was: (i) Assess the functional capacity of SCA patients through the T-Glittre test; (ii).
Investigate feasibility of using Glittre-ADL test in this population; (iii) Investigate the submaximal cardiorespiratory fitness presented during the T-Glittre test and; (iv) To investigate the association between measures of functional capacity and measures of disease severity, balance, gait as well as between these measures and measures of
submaximal cardiorespiratory fitness. Methods: This was a cross-sectional study that included a sample of 20 individuals diagnosed with ACS. The individuals were evaluated through the T-Glittre with a registry of the physiological responses, Berg Balance Scale (BSE), SARA, modifiedDynamicGait Index (mDGI), Inventory of Non-Ataxia Signs (INAS), Timed Up and go test (TUG) and Four Stage balance test (4Stage). Viability was assessed by recording adverse events. Results: No adverse events were recorded. The time taken by participants to perform the T-Glittre was [median (minimum - maximum)] 6.5 minutes (4.17-10.57) and the VO2 intake was 14, 46 ml/kg/min (8.29 – 22.45). There were significant correlations between the
time taken to complete T-Glittre and BSE (rho = -0.873; <.001), SARA (rho = 0.708; p <.001); mDGI (rho = -0.933, p <0.001). Conclusion: The T-Glittre test is a viable way to assess the functional capacity of SCA patients, and the time spent during its execution may reflect greater or lesser motor impairment.