Abstract
Mobility limitations, including a decrease in walking speed, are major issues for people with autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS). Improving our understanding of factors influencing walking speed in ARSACS may inform the development of future interventions for gait rehabilitation and contribute to better clinical practices. The objective of the study was to identify the factors influencing the self-selected walking speed in adults with ARSACS. The dependent variable of this cross-sectional study was the self-selected speed and the factors (independent variables) were age, sex, balance, balance confidence, knee flexion and extension cocontraction indexes, lower limb coordination, passive range of motion of ankle dorsiflexion, knee and hip extension, and global spasticity. Multiple regression models were used to assess the relationships between walking speed and each factor individually. Six factors were significantly associated with walking speed and thus included in regression models. The models explained between 42.4 and 66.5% of the total variance of the self-selected walking speed. The factors that most influence self-selected walking speed are balance and lower limb coordination. In order of importance, the other factors that also significantly influence self-selected walking speed are ankle dorsiflexion range of motion, lower limb spasticity, knee extension range of motion, and confidence in balance. Balance and lower limb coordination should be targeted in rehabilitation interventions to maintain walking ability and functional independence as long as possible. The six factors identified should also be included in future studies to deepen our understanding of walking speed.
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Data Availability
Anonymized data not published within this article will be made available by request from any qualified investigator, following approval of the data use proposal by the Ethics Review Board of the CIUSSS-SLSJ.
Abbreviations
- 10mWT:
-
10-m walk test
- ABC-simplified:
-
Activity-specific balance confidence—simplified
- ARSACS:
-
Autosomal recessive spastic ataxia of Charlevoix-Saguenay
- BBS:
-
Berg balance scale
- CI:
-
Cocontraction index
- EMG:
-
Electromyography
- ICC:
-
Intraclass correlation coefficient
- LEMOCOT:
-
Lower extremity motor coordination test
- MAS:
-
Modified Ashworth scale
- SD:
-
Standard deviation
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Acknowledgements
The authors would like to thank all patients who participated in this study as their involvement is essential to the success of the research. The authors would like to thank Djamal Berbiche for his support in the statistical analysis.
Funding
This study was supported by the European Union’s Horizon 2020 research and innovation program under the ERA-NET Cofund action no. 643578. It was also supported by the CIFR team (MDC and Ataxia of Charlevoix-Saguenay) and Ataxia of Charlevoix-Saguenay foundation (to C.G. and B.B.) and the BMBF (01GM1607), under the frame of the E-Rare-3 network PREPARE. Funding sources also include the Canadian Institutes of Health Research in partnership with the Ataxia of Charlevoix-Saguenay Foundation (Emerging Team Grant no TR2-119189). CG holds a career-grant funding from Fonds de recherche en santé du Québec (no 31011). Sponsors did not contribute to the design, other study steps, or data analysis.
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IL, BB, JM, and CG contributed to the study concept and design. IL and RSG had a major role in the data acquisition. IL, IC, LJH, BB, and CG contributed to data analysis and interpretation. The first draft of the manuscript was written by IL and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.
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The study was approved by the Ethics Review Board of the Centre intégré universitaire de santé et de services sociaux du Saguenay–Lac-Saint-Jean (#2015-002) and performed in accordance with the ethical standards as laid down in the 1964 Declaration of Helsinki and its later amendments.
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Written informed consent was obtained for all participants enrolled in the study.
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C.G. is a consultant for Vertex, Arthrex, Seelos, and Biogen, and also conducts academic work for Biogen, Ionis, and Seelos, all unrelated to the present manuscript. All other authors declare that they have no conflict of interest.
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Lessard, I., HĂ©bert, L.J., St-Gelais, R. et al. Toward a Better Understanding of Walking Speed in Ataxia of Charlevoix-Saguenay: a Factor Exploratory Study. Cerebellum (2023). https://doi.org/10.1007/s12311-023-01646-x
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DOI: https://doi.org/10.1007/s12311-023-01646-x