Evolving on Wheels: One-Year of Mobility Evolution in Adults with Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay

Evolving on Wheels: One-Year of Mobility Evolution in Adults with Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay | medRxiv /* */ /* */ <!-- <!-- /*! * yepnope1.5.4 * (c) WTFPL, GPLv2 */ (function(a,b,c){function d(a){return"[object Function]"==o.call(a)}function e(a){return"string"==typeof a}function f(){}function g(a){return!a||"loaded"==a||"complete"==a||"uninitialized"==a}function h(){var a=p.shift();q=1,a?a.t?m(function(){("c"==a.t?B.injectCss:B.injectJs)(a.s,0,a.a,a.x,a.e,1)},0):(a(),h()):q=0}function i(a,c,d,e,f,i,j){function k(b){if(!o&&g(l.readyState)&&(u.r=o=1,!q&&h(),l.onload=l.onreadystatechange=null,b)){"img"!=a&&m(function(){t.removeChild(l)},50);for(var d in y[c])y[c].hasOwnProperty(d)&&y[c][d].onload()}}var j=j||B.errorTimeout,l=b.createElement(a),o=0,r=0,u={t:d,s:c,e:f,a:i,x:j};1===y[c]&&(r=1,y[c]=[]),"object"==a?l.data=c:(l.src=c,l.type=a),l.width=l.height="0",l.onerror=l.onload=l.onreadystatechange=function(){k.call(this,r)},p.splice(e,0,u),"img"!=a&&(r||2===y[c]?(t.insertBefore(l,s?null:n),m(k,j)):y[c].push(l))}function j(a,b,c,d,f){return q=0,b=b||"j",e(a)?i("c"==b?v:u,a,b,this.i++,c,d,f):(p.splice(this.i++,0,a),1==p.length&&h()),this}function k(){var a=B;return a.loader={load:j,i:0},a}var l=b.documentElement,m=a.setTimeout,n=b.getElementsByTagName("script")[0],o={}.toString,p=[],q=0,r="MozAppearance"in l.style,s=r&&!!b.createRange().compareNode,t=s?l:n.parentNode,l=a.opera&&"[object Opera]"==o.call(a.opera),l=!!b.attachEvent&&!l,u=r?"object":l?"script":"img",v=l?"script":u,w=Array.isArray||function(a){return"[object Array]"==o.call(a)},x=[],y={},z={timeout:function(a,b){return b.length&&(a.timeout=b[0]),a}},A,B;B=function(a){function b(a){var a=a.split("!"),b=x.length,c=a.pop(),d=a.length,c={url:c,origUrl:c,prefixes:a},e,f,g;for(f=0;f<d;f++)g=a[f].split("="),(e=z[g.shift()])&&(c=e(c,g));for(f=0;f<b;f++)c=x[f](c);return c}function g(a,e,f,g,h){var i=b(a),j=i.autoCallback;i.url.split(".").pop().split("?").shift(),i.bypass||(e&&(e=d(e)?e:e[a]||e[g]||e[a.split("/").pop().split("?")[0]]),i.instead?i.instead(a,e,f,g,h):(y[i.url]?i.noexec=!0:y[i.url]=1,f.load(i.url,i.forceCSS||!i.forceJS&&"css"==i.url.split(".").pop().split("?").shift()?"c":c,i.noexec,i.attrs,i.timeout),(d(e)||d(j))&&f.load(function(){k(),e&&e(i.origUrl,h,g),j&&j(i.origUrl,h,g),y[i.url]=2})))}function h(a,b){function c(a,c){if(a){if(e(a))c||(j=function(){var a=[].slice.call(arguments);k.apply(this,a),l()}),g(a,j,b,0,h);else if(Object(a)===a)for(n in m=function(){var b=0,c;for(c in a)a.hasOwnProperty(c)&&b++;return b}(),a)a.hasOwnProperty(n)&&(!c&&!--m&&(d(j)?j=function(){var a=[].slice.call(arguments);k.apply(this,a),l()}:j[n]=function(a){return function(){var b=[].slice.call(arguments);a&&a.apply(this,b),l()}}(k[n])),g(a[n],j,b,n,h))}else!c&&l()}var h=!!a.test,i=a.load||a.both,j=a.callback||f,k=j,l=a.complete||f,m,n;c(h?a.yep:a.nope,!!i),i&&c(i)}var i,j,l=this.yepnope.loader;if(e(a))g(a,0,l,0);else if(w(a))for(i=0;i (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0];var j=d.createElement(s);var dl=l!='dataLayer'?'&l='+l:'';j.src='//www.googletagmanager.com/gtm.js?id='+i+dl;j.type='text/javascript';j.async=true;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-P4HH5NV'); Skip to main content Home About Submit ALERTS / RSS Search for this keyword Advanced Search Evolving on Wheels: One-Year of Mobility Evolution in Adults with Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay View ORCID Profile François Routhier , View ORCID Profile Krista L. Best , View ORCID Profile Rose Gagnon , Caroline Rahn , View ORCID Profile Cynthia Gagnon , View ORCID Profile Luc J. Hébert , View ORCID Profile Isabelle Lessard , View ORCID Profile Xavier Rodrigue doi: https://doi.org/10.1101/2025.09.16.25335887 François Routhier a School of Rehabilitation Sciences, Faculty of Medicine, Université Laval, 1050 Avenue de la Médecine , Quebec City, QC, G1V 0A6, Canada b Centre for Interdisciplinary Research in Rehabilitation and Social Integration (Cirris), Centre intégré universitaire de santé et de services sociaux (CIUSSS) de la Capitale-Nationale , 525 Wilfrid-Hamel Boulevard, Quebec City, QC, G1M 2S8, Canada PEng, PhD Find this author on Google Scholar Find this author on PubMed Search for this author on this site ORCID record for François Routhier For correspondence: francois.Routhier{at}rea.ulaval.ca Krista L. Best a School of Rehabilitation Sciences, Faculty of Medicine, Université Laval, 1050 Avenue de la Médecine , Quebec City, QC, G1V 0A6, Canada b Centre for Interdisciplinary Research in Rehabilitation and Social Integration (Cirris), Centre intégré universitaire de santé et de services sociaux (CIUSSS) de la Capitale-Nationale , 525 Wilfrid-Hamel Boulevard, Quebec City, QC, G1M 2S8, Canada PhD Find this author on Google Scholar Find this author on PubMed Search for this author on this site ORCID record for Krista L. Best Rose Gagnon a School of Rehabilitation Sciences, Faculty of Medicine, Université Laval, 1050 Avenue de la Médecine , Quebec City, QC, G1V 0A6, Canada b Centre for Interdisciplinary Research in Rehabilitation and Social Integration (Cirris), Centre intégré universitaire de santé et de services sociaux (CIUSSS) de la Capitale-Nationale , 525 Wilfrid-Hamel Boulevard, Quebec City, QC, G1M 2S8, Canada c Population Health and Optimal Health Practices Axis, CHU de Québec – Université Laval Research Centre , 2705 Laurier Boulevard, Quebec City, QC, G1V 4G2, Canada MPT, MSc, PhD(c) Find this author on Google Scholar Find this author on PubMed Search for this author on this site ORCID record for Rose Gagnon Caroline Rahn d Institut de réadaptation en déficience physique de Québec (IRDPQ), Centre intégré universitaire de santé et de services sociaux (CIUSSS) de la Capitale-Nationale , 525 Wilfrid-Hamel Boulevard, Quebec City, QC, G1M 2S8, Canada PT Find this author on Google Scholar Find this author on PubMed Search for this author on this site Cynthia Gagnon e School of Rehabilitation, Faculty of Medicine and Health Sciences, Université de Sherbrooke , 3001 12th Avenue North, Sherbrooke, QC, J1H 5N4, Canada f Interdisciplinary Research Group on Neuromuscular Disorders, Hôpital de Jonquière, Centre intégré universitaire de santé et de services sociaux (CIUSSS) du Saguenay-Lac-Saint-Jean, 2230 de l’Hôpital , Jonquière, QC, G7X 7X2, Canada OT, PhD Find this author on Google Scholar Find this author on PubMed Search for this author on this site ORCID record for Cynthia Gagnon Luc J. Hébert a School of Rehabilitation Sciences, Faculty of Medicine, Université Laval, 1050 Avenue de la Médecine , Quebec City, QC, G1V 0A6, Canada b Centre for Interdisciplinary Research in Rehabilitation and Social Integration (Cirris), Centre intégré universitaire de santé et de services sociaux (CIUSSS) de la Capitale-Nationale , 525 Wilfrid-Hamel Boulevard, Quebec City, QC, G1M 2S8, Canada g Department of Radiology and Nuclear Medicine, Faculty of Medicine, Université Laval , 1050 Avenue de la Médecine, Quebec City, QC, G1V 0A6, Canada Fellow PT, PhD, CD Find this author on Google Scholar Find this author on PubMed Search for this author on this site ORCID record for Luc J. Hébert Isabelle Lessard f Interdisciplinary Research Group on Neuromuscular Disorders, Hôpital de Jonquière, Centre intégré universitaire de santé et de services sociaux (CIUSSS) du Saguenay-Lac-Saint-Jean, 2230 de l’Hôpital , Jonquière, QC, G7X 7X2, Canada h Centre d’Étude des Conditions de vie et des Besoins de la population (ÉCOBES) – Recherche et transfert, Cégep de Jonquière , 2505 St-Hubert Street, Jonquière, QC, G7X 7W2, Canada PT, PhD Find this author on Google Scholar Find this author on PubMed Search for this author on this site ORCID record for Isabelle Lessard Xavier Rodrigue a School of Rehabilitation Sciences, Faculty of Medicine, Université Laval, 1050 Avenue de la Médecine , Quebec City, QC, G1V 0A6, Canada b Centre for Interdisciplinary Research in Rehabilitation and Social Integration (Cirris), Centre intégré universitaire de santé et de services sociaux (CIUSSS) de la Capitale-Nationale , 525 Wilfrid-Hamel Boulevard, Quebec City, QC, G1M 2S8, Canada d Institut de réadaptation en déficience physique de Québec (IRDPQ), Centre intégré universitaire de santé et de services sociaux (CIUSSS) de la Capitale-Nationale , 525 Wilfrid-Hamel Boulevard, Quebec City, QC, G1M 2S8, Canada MD, FRCPC Find this author on Google Scholar Find this author on PubMed Search for this author on this site ORCID record for Xavier Rodrigue Abstract Full Text Info/History Metrics Supplementary material Data/Code Preview PDF Abstract Introduction Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is a progressive neurological disorder that leads to significant motor impairments and eventual wheelchair use in adulthood. Although previous studies have described limited mobility and participation in adult wheelchair users with ARSACS, data remains scarce. This study aimed to describe mobility evolution over one year of adult manual wheelchair users with ARSACS. Methods Longitudinal descriptive study including manual wheelchair users with genetically confirmed ARSACS (aged 18-59) recruited from two neuromuscular clinics in the province of Quebec (Canada). Participants completed validated questionnaires assessing wheelchair skills and self-efficacy, and wore two accelerometers for seven days to measure objective mobility (self-propulsion and non-propulsion). Data were collected at baseline and one year later, and analyzed using descriptive statistics and nonparametric repeated measures ANOVA. Results Thirty-four adult manual wheelchair users with ARSACS participated in the study, with a mean age of 46.1 years (± 8.3 years) and moderate to severe disease severity at baseline. Participants reported low manual wheelchair skills, but high self-efficacy regarding acquired skills. Over one year, objective mobility measures showed a non-significant decrease in distance traveled, number of bouts and bout length. Differences in objective mobility measures according to age and sex were observed, but were non-statistically significant. Conclusion This study represents a first step towards a better understanding of wheelchair mobility of adults with ARSACS. These findings have the potential to help improve their rehabilitation process. Highlights - Manual wheelchair users reported low wheelchair skills, but high self-efficacy - Total distance traveled, number of bouts, and bout length decreased over one year - Proportion of self-propelled objective mobility decreased over one year - Differences according to age and sex were observed, but were non-significant 1.1. Introduction Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is a slowly progressive neurological disorder characterized by cerebellar, pyramidal and neuropathic impairments [ 1 , 2 ]. ARSACS was originally described and is still most prevalent in the Charlevoix and Saguenay-Lac-Saint-Jean regions in Quebec, Canada [ 1 , 3 ]. However, cases have now been reported in many other countries, leading to its recognition as one of the most common forms of recessive spastic ataxia worldwide [ 1 , 4 , 5 ]. Despite individual variability in mutations and manifestations [ 2 , 6 - 8 ], common signs include lack of coordination, distal weakness, and impaired dexterity and balance [ 8 - 11 ]. Gait abnormalities or falls are often the initial reasons for consultation during childhood (3.4 years on average) [ 9 ]. The evolution of these limitations will largely determine the timing of wheelchair acquisition, which occurs at a mean age of 39 years (range of 17-58 years) [ 8 , 12 ]. In the province of Quebec, Canada, around 45% of adults with ARSACS are permanent wheelchair users [ 2 , 8 , 13 ]. Nevertheless, literature on adult wheelchair users with ARSACS or their mobility is scarce. Preliminary evidence indicates that loss of the ability to walk is associated with decreased social participation, functional independence, and motor performance in this population [ 8 , 10 ]. Only one recent descriptive study by Bourassa et al. has specifically described the characteristics of adult wheelchair users with ARSACS [ 14 ]. Limited wheelchair skills, significantly impaired motor function, and restricted social participation that generally decreased with age were found. This study also provided the first data on wheelchair mobility and wheelchair life-space of adults with ARSACS. Participants reported relatively limited travel in mobility areas with their wheelchair compared to other populations, such as older manual wheelchair users [ 15 ]. However, further investigation is needed, as no objective measure of wheelchair displacement was taken. Therefore, objectively measuring manual wheelchair mobility in adults with ARSACS is of interest. Such empirical evidence may help us better understand the profile and clinical evolution of this understudied subpopulation, an essential step to advance trial readiness [ 16 ] and to guide their rehabilitation process. It may also confirm and offer possible explanations related to the limitations in social participation and wheelchair mobility that have been documented subjectively in previous studies [ 8 , 14 , 17 ]. To achieve this, activity monitors (e.g., accelerometers, data loggers) have become an increasingly common method to objectively measure manual wheelchair displacement [ 18 , 19 ]. Thus, the general objective of this study was to describe the mobility evolution over one year of manual wheelchair users with ARSACS. More specifically, we measured the evolution of their wheelchair skills, wheelchair self-efficacy, and objective mobility. 1.2. Material and Methods 1.2.1. Study Design and Setting This study used a longitudinal descriptive design including comparative analyses. Data were collected as part of a larger multicenter longitudinal cohort study on the natural history of adults with ARSACS. Testing took place at the Saguenay Neuromuscular clinic ( Centre intégré universitaire de santé et de services sociaux du Saguenay-Lac-Saint-Jean ) in Saguenay (Canada) and the Centre intégré universitaire de santé et de services sociaux de la Capitale-Nationale in Quebec City (Canada). Ethical approval was obtained for each site through the Centre intégré universitaire de santé et de services sociaux de la Capitale-Nationale Research Ethics Board in rehabilitation and social integration and the Centre intégré universitaire de santé et de services sociaux du Saguenay-Lac-Saint-Jean Research Ethics Board. All participants provided written informed consent. 1.2.2. Participants and Sampling Participants were recruited in Saguenay and Quebec City through the registry of patients with recessive ataxia from each clinic. Individuals who had participated in a previous phase of the longitudinal study were contacted again by the research team and additional participants were recruited. For all participants of the larger study, a random sampling strategy stratified by age group (14–17; 18–29; 30–39; 40–49; 50–59 years) and sex (men, women) was used. To be included, participants had to be between 18 and 59 years of age, be a manual wheelchair user (occasionally or regularly), and have a genetically confirmed ARSACS diagnosis. Participants were excluded if they had other medical conditions leading to significant functional limitations or multiple morbidities preventing them from completing all assessments. 1.2.3. Main Outcome Measures 1.2.3.1. Questionnaires and Rating Scales As part of the larger study, several measures (French versions) were collected, which allowed for the description of the clinical progression of the adults recruited, as well as the progression of their manual wheelchair skills and self-efficacy. A demographic questionnaire was also used to obtain data on participants’ sex, age, main occupation, level of education, and living environment. Wheelchair skills and self-efficacy Wheelchair skills and self-efficacy were quantified using the Wheelchair Skills Test Questionnaire (WST-Q-F) 5.0 and the Wheelchair Use Confidence Scale (WheelCon-F) Short Form. The WST-Q-F assesses capacity, confidence, performance, and training goals regarding 32 manual wheelchair skills. Higher percentage scores for each subscale indicate better wheelchair skills [ 20 , 21 ]. The WheelCon-F includes 21 items, divided in two subscales (mobility self-efficacy and self-management efficacy), for which the respondents rate their level of confidence on a scale of 0-10 [ 22 ]. Both questionnaires have been validated among adults with ARSACS [ 17 ]. Motor performance The severity of cerebellar ataxia was quantified using the Scale for the Assessment and Rating of Ataxia (SARA) [ 23 ]. The SARA includes eight items for a total score ranging from 0 (no ataxia) to 40 (severe ataxia). The Disease Severity Index for adults with ARSACS (DSI-ARSACS) was also used to measure disease-specific severity. The DSI-ARSACS assesses cerebellar, neuropathic and pyramidal ARSACS impairments through eight items [ 24 ]. A higher score (/38) indicates a more severe disease stage. Both assessments have been validated in adults with ARSACS [ 24 , 25 ]. 1.2.3.2. Objective Mobility Two ActiGraph wGT3X-BT accelerometers (ActiGraph Corp., Peniscola, FL) were used to measure objective mobility (self-propelled and non-propelled). The ActiGraph wGT3X-BT is a small (4.6cm x 3.3cm x 1.5cm) and lightweight (19 grams) device that can be worn on the arm and does not impede arm movements. It contains a solid state three-axis microelectromechanical system-based accelerometer with a sample rate of 30-110 Hertz and a dynamic range of 8G ( https://www.actigraphcorp.com/ ). The validity and reliability of this device for manual wheelchair users has been demonstrated [ 26 - 29 ]. Activity counts measured using actigraphs were sampled at a frequency of 30 Hz (every 1/30 th of a second). The sampling unit (epochs) was then converted to 1s to facilitate data analysis and to ensure enough sensitivity for low-intensity activities. Participants wore two ActiGraph accelerometers for seven consecutive days, except during sleep and hygiene periods. The first accelerometer was worn between the elbow and shoulder on their non-dominant arm using an armband. The non-dominant arm was selected, as wearing the ActiGraph on the dominant arm may result in an overestimation of activity due to extraneous arm movement [ 27 ]. The other accelerometer was attached close to the axis of the wheelchair rear wheel on the same side using a sturdy plastic case and tie-wraps. We made sure that the middle of the device was aligned parallel to a spoke of the wheel and that no undesired movement was allowed. Research suggests that using two devices is a particularly promising way of capturing self-propelled wheelchair driving in real-life environments [ 29 - 31 ]. More precisely, the combination of two ActiGraph GT3X-BT accelerometers has been shown to validly differentiate between self-propelled and non-propelled wheelchair driving (overall agreement of 85%) among manual wheelchair users with spinal cord injury [ 29 ]. Participants were asked to report in writing any omissions related to wearing the accelerometers (logbook), if applicable. Objective wheelchair mobility measurements taken using actigraphs were processed using a custom algorithm previously developed and validated by our research team, which allows to derive several measures of manual wheelchair physical activity (i.e., total distance traveled, number of bouts, bout length, bout speed, and maximum speed), and to discriminate between self-propulsion and non-propulsion. More details on the algorithm’s functioning can be found in a previous article by Gagnon et al. [ 32 ]. 1.2.4. Procedures Baseline outcome measures were administered over three sessions: one 1-h session by phone and two half-day sessions in clinic ( Figure 1 ). The ActiGraph accelerometers were presented and installed during the first session in clinic. They were taken back during the second session, after the 7-day wearing period. All participants who completed the baseline assessment were subsequently contacted after one year to repeat the two measurement sessions (i.e., ARSACS rating scales, wheelchair skills and motor performance questionnaires, and 7-day actigraphs wearing period). Download figure Open in new tab Figure 1. Flow diagram representing the study’s procedures and completed assessment tools during each session. MWC, manual wheelchair; DSI-ARSACS, Disease Severity Index for adults with ARSACS; SARA, Scale for the Assessment and Rating of Ataxia; WST-Q-F, Wheelchair Skills Test Questionnaire – French version; WheelCon-F, Wheelchair Use Confidence Scale – French version. 1.2.5. Data Analysis Descriptive statistics (mean, standard deviation, proportion) were used to summarize participants’ sociodemographic characteristics, wheelchair skills, wheelchair self-efficacy, motor performance, and objective mobility (self-propelled and non-propelled). Objective mobility measures measured during the 7-day wearing period were separated into four measurement types to better detail possible variations in mobility habits (i.e., three days, seven days, weekend, week). In addition to analyses including all participants, stratified analyses according to age and biological sex were also performed to explore whether these two characteristics had a differential impact on the clinical evolution observed. Participants’ age was dichotomized based on the median age of the sample (≤ 46 years and ≥ 47 years). This choice was made to obtain two groups of similar size for statistical analysis purposes. Data were analyzed using nonparametric repeated measures analysis of variance (ANOVA, nparLD package). The level of statistical significance (α) was set at .05. All analyses were performed using R statistical software (versions 4.3.2 and 4.4.2, R Foundation). 1.3. Results 1.3.1. Participants’ Characteristics Table 1 presents the sociodemographic characteristics of the 34 adult manual wheelchair users included in the study. Mean participants’ age at baseline was 46.1 years, and 58.8% were males. Mean baseline scores for the SARA and DSI-ARSACS were respectively 27.5 ± 5.2 and 23.5 ± 4.4, indicating a significant level of ataxia (SARA) and a more severe disease stage (DSI-ARSACS). More than 70% of participants used a manual wheelchair for indoor (73.5%) and outdoor mobility (76.5%) at baseline, compared to 58.8% (indoor) and 52.9% (outdoor) at the 1-year follow-up. Participants’ autonomy level ranged from autonomous (baseline: 38.2%, 1-year follow-up: 11.8%) to complete assistance (baseline: 0.0%, 1-year follow-up: 2.9%), with the majority being partially assisted at the end of follow-up (baseline: 61.8%, 1-year follow-up: 70.6%). View this table: View inline View popup Download powerpoint Table 1. Participants sociodemographic characteristics (n = 34) 1.3.2. Wheelchair Skills and Self-efficacy Table 2 presents the mean wheelchair skills scores for the entire sample and stratified according to age and sex. Mean scores for all WST-Q-F subscales were low, ranging from 31.6 to 53.5 for the capacity subtotal, 42.3 and 64.0 for the confidence subtotal, and 31.6 and 48.1 for the performance subtotal. Scores were generally lower among participants aged 47 and older and among women, but observed differences were not statistically significant. Included participants expressed a desire to train several wheelchair abilities, both at baseline and at the 1-year follow-up (Training goals, baseline: 58.0/100, 1-year follow-up: 58.8/100). Despite low WST-Q-F scores, participants expressed a high self-efficacy level in their abilities to use a manual wheelchair (WheelCon-M-F, baseline: 7.0/10, 1-year follow-up: 7.1/10). Observed self-efficacy level was lower among users aged 47 and older, but not statistically different. View this table: View inline View popup Download powerpoint Table 2. Mean wheelchair skills and self-efficacy scores and comparison between age groups and sex 1.3.3. Objective mobility Table 3 reports the various three-day objective wheelchair mobility measures obtained using the previously validated algorithm. The seven-day, weekend, and weekly objective wheelchair mobility measures can be found in Tables S1 to S3 (Supplementary Material). After one year, mean total distance traveled (daily), number of bouts and bout length all decreased (non-statistically significant differences). These same measures were generally lower among participants aged 47 and older (when compared to those aged 46 and younger) and among women (when compared to men); however, these differences were not significant. Moreover, the proportion of the mean total distance traveled and number of bouts made without self-propelling increased between the initial assessment and 1-year follow-up (p>.05). Measures of bout speed and maximum speed remained generally similar, regardless of age, sex, and objective wheelchair mobility measurement type (three days, seven days, weekend, week). View this table: View inline View popup Download powerpoint Table 3. Mean wheelchair objective mobility measures - all and propulsion only - and comparison at a given time point (three days) 1.4. Discussion Mean wheelchair skills scores obtained by participants via the WST-Q-F were low, both at baseline and at the 1-year follow-up. Although no normative data or recognized cut-offs for manual wheelchair skills exist, mean observed scores for the capacity, confidence, and performance subscales were lower than those reported in the scientific literature for manual wheelchair users presenting with a variety of neurological conditions [ 20 , 33 ]. Several studies report a significant positive association between WST-Q scores and cardiovascular health [ 34 , 35 ]. High WST-Q scores are also associated with increased participation in daily life and social activities, greater confidence, independence, and basic mobility, all of which contribute to cardiovascular health and quality of life [ 20 , 36 ]. It would thus be of interest in subsequent studies to evaluate the impact of targeted interventions aimed at improving the various skills listed in the WST-Q (e.g., gets over obstacle, ascends/descends high curb) during the rehabilitation process of manual wheelchair users with ARSACS, especially considering the degenerative nature of this neurological condition [ 8 ]. Despite low wheelchair skills scores, recruited participants presented high overall WheelCon-M scores, reflecting a high self-efficacy towards their manual wheelchair skills. These high scores could be due to the fact that adults with ARSACS have low expectations regarding the use of a manual wheelchair, thereby increasing their self-efficacy with regards to the skills they have already mastered [ 14 ]. Objective wheelchair mobility measurements showed that participants presented low levels of objective mobility at baseline, and that these levels tended to decrease after one year. Previous studies have reported that adult manual wheelchair users with a neurological condition tend to travel between 1.4 and 2.5 kilometers per day [ 37 , 38 ], which is much higher than the mean total daily distance measured at baseline (745.4 ± 550.7 meters) and follow-up (735.1 ± 621.0 meters). The proportion of bouts and total daily distance travelled by self-propulsion also tended to decrease over time, especially among participants aged 47 years and older (1-year follow-up propulsion vs. other: total distance, p<.01; number of bouts, p=.08). These results are important, in that manual wheelchair users who travel greater distance each day tend to be more physically active and engage in more intense physical activities [ 39 , 40 ]. Greater total daily distance is also associated with improved function [ 41 ], lower depression scores, and greater life satisfaction [ 42 ]. Special attention should thus be given to interventions that promote mobility in adult manual wheelchair users with ARSACS, given the higher risk of manual wheelchair users developing comorbidities when exhibiting higher levels of physical inactivity [ 43 ]. Recruited participants expressed a significant need for training, with scores of 58.0% (baseline) and 58.8% (1-year follow-up) on the WST-Q-F’s training goals subscale. This finding is consistent with other studies indicating that manual wheelchair users may not receive enough training to improve their wheelchair skills [ 44 , 45 ]. When given the opportunity to receive training, such training is often reported to be inconsistent, not tailored to individual patients’ needs, and not addressing advanced skills and environmental adaptation [ 44 , 45 ]. The training provided also tend to focus on the acquisition of short-term skills, neglecting long-term confidence, self-efficacy, and community participation [ 44 , 46 ]. Some preliminary studies report that engaging in physical activity may help slow the progression of certain neurological diseases [ 47 ], including ARSACS [ 48 ]. Greater attention should be given in the coming years to implementing and validating manual wheelchair skills programs that are consistent, tailored to the individual needs of adults with ARSACS, and which include advanced skills training. Such programs could increase the mobility level of adults living with this condition, and foster greater community participation. 1.4.1. Strengths and Limitations Several limitations must be considered when interpreting the results of this study. The analyses performed should be regarded as exploratory given the small number of participants included. Furthermore, various reasons, such as loss to follow-up, death, loss of ability to use a manual wheelchair, and incapacity to perform the actigraphy protocol, led to a relatively high rate of missing data, both during the initial data collection and 1-year follow-up. In future studies, it will be important to ensure that the actigraphy protocol can be easily performed by participants, especially considering the high fatigability of persons with ARSACS and, more broadly, of any adult manual wheelchair user with a neurological condition. It is also possible that the placement of the actigraphs on the wheel of the manual wheelchair and on the non-dominant arm did not capture movements performed using different self-propulsion patterns, such as dominant arm and one foot or two feet. The objective mobility measurements reported in this study should therefore be viewed as minimums. Despite these limitations, we believe that this study has some noteworthy strengths. It comprehensively reports numerous preliminary longitudinal objective mobility measures that may help better understand the profile and clinical evolution of this understudied population, representing an essential step toward future clinical trials and tailoring of their rehabilitation services. Actigraphy data reported in this study were derived using a previously validated conversion algorithm with excellent accuracy. Finally, use of this same algorithm allowed to explore the proportion of self-propulsion for certain objective mobility measures (i.e., total distance, number of bouts). These mobility measures pave the way for future studies, such as correlation studies focusing on the most significant factors contributing to the observed decline in wheelchair mobility. 1.5. Conclusion Results of this study indicate that adults with ARSACS present limited manual wheelchair skills, high wheelchair self-efficacy, and low levels of objective mobility. In addition, total objective mobility done while self-propelling tended to decrease after one year. This study represents a first step towards a better understanding of mobility levels of adults with ARSACS. These findings have the potential to help improve their rehabilitation process. Data Availability All data produced in the present study are available upon reasonable request to the authors. 1.7. Statements and declarations 1.7.1. Declaration of Interest Declarations of interest: none. 1.7.2. Funding This work was supported by the Canadian Institutes of Health Research (CIHR) Project Grant no. 159777; and the Fonds de recherche du Québec – Santé (FRQ-S) through Research Scholar salary awards held by François Routhier, Krista Best, and Cynthia Gagnon. Rose Gagnon received scholarships from CIHR (#491913), the FRQ-S, the Unité de soutien SSA Québec , the Ordre professionnel de la physiothérapie du Québec , the Centre interdisciplinaire de recherche en réadaptation et intégration sociale (Cirris) and Université Laval. Funders had no role in study design, in the collection, analysis and interpretation of data, in the writing of the report, and in the decision to submit the article for publication. 1.7.3. Authors’ Contributions Conceptualization: FR, KLB, CG, LJH, IL, XR; Funding acquisition: FR, KLB, CG, LJH, XR; Investigation: FR, KLB, CR, CG; Formal analysis: FR, KLB, RG; Visualization: FR, KLB, RG; Writing – original draft: FR, KLB, RG; Writing – review and editing: FR, KLB, RG, CR, CG, LJH, IL, XR. 1.6. Acknowledgements The Authors would like to thank the following persons for their contributions: all members of the research team who contributed to data collection, and Julie Bourassa, Alexandre Desgagné-Lebeuf, Valérie Moisan, Jean Leblond, and Bernard Brais for their contribution to the study’s conception, data collection and analysis. Footnotes krista.best{at}fmed.ulaval.ca rose.gagnon.1{at}ulaval.ca caroline.rahn.ciussscn{at}ssss.gouv.qc.ca Cynthia.Gagnon4{at}USherbrooke.ca Lucj.Hebert{at}fmed.ulaval.ca isabellelessard{at}cegepjonquiere.ca xavier.rodrigue.med{at}ssss.gouv.qc.ca 1.8. References [1]. ↵ S. Vermeer , B. van de Warrenburg , E. Kamsteeg , B. 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