Toward a Better Understanding of Walking Speed in Ataxia of Charlevoix-Saguenay: a Factor Exploratory Study.

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.

Natural History of Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay: a 4-Year Longitudinal Study.

Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is a neurologic disorder with generally well-known clinical manifestations. However, few studies assessed their progression rate using a longitudinal design. This study aimed to document the natural history of ARSACS over a 4-year period in terms of upper and lower limb functions, balance, walking capacity, performance in daily living activities, and disease severity.

Digital health metrics reveal upper limb impairment profiles in ARSACS.

Objective: Adults with autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) often present with reduced upper limb coordination affecting their independence in daily life. Previous studies in ARSACS identified reduced performance in clinical assessments requiring fine and gross dexterity as well as prehension. However, the kinematic and kinetic aspects underlying reduced upper limb coordination in ARSACS have not been systematically investigated yet.

A rehabilitation program to increase balance and mobility in ataxia of Charlevoix-Saguenay: An exploratory study.

Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is characterized by balance impairment and mobility limitations, which both increase the risk of falling. The objective of this study was to explore the effects of a rehabilitation program aimed at increasing trunk and lower limb motor control on balance and walking abilities, and accomplishment of activities of daily living. In this exploratory study, a group-supervised rehabilitation program was performed three times a week for 8 weeks (two sessions at a rehabilitation gym and one pool session).

French Translation and Cross-cultural Adaptation of the Scale for the Assessment and Rating of Ataxia.

The Scale for the Assessment and Rating of Ataxia (SARA) is a widely used scale for assessing the severity of ataxia in clinics, natural history studies, and treatment trials worldwide. However, no French translation with validated cross-cultural adaptation is available. This study aimed to translate and adapt the SARA into French.

Participation and Functional Independence in Adults With Recessive Spastic Ataxia of Charlevoix-Saguenay.

Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is a progressive disorder where upper and lower extremities motor performances may bring participation restriction. To document participation in adults with ARSAC and explore associations with motor performances. Twenty-eight participants took part in the study.

Sexual activity based fears during pregnancy, sexual function and dyadic adjustment in couples who are expecting their first child.

Sexual activity based fears during pregnancy are common, but very few studies have examined their association with relationship variables. Secondary analysis of associations between these fears during pregnancy and both partners' sexual function and dyadic adjustment were conducted. A sample of 67 French-Canadian first-time parenting couples living in Ontario completed online questionnaires on sexual activity based fears, sexual function, and dyadic adjustment as experienced during pregnancy.

Reliability and validity of digital health metrics for assessing arm and hand impairments in an ataxic disorder.

Objectives: Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is the second most frequent recessive ataxia and commonly features reduced upper limb coordination. Sensitive outcome measures of upper limb coordination are essential to track disease progression and the effect of interventions. However, available clinical assessments are insufficient to capture behavioral variability and detailed aspects of motor control.

How to Deal With Sexual Changes During and After Pregnancy: Results of a Brief Psycho-Educational Workshop With Future and New Parenting Couples.

After documenting parenting couples' needs regarding perinatal sexuality, this study developed and evaluated a brief psycho-educational workshop on perinatal sexuality. Participants (16 future and 17 new parents) completed five steps: (1) online questionnaire (T1) assessing needs toward the workshop and sexoperinatal knowledge, attitudes, and perceptions; (2) 2-hour online workshop on perinatal sexuality; (3) post-workshop satisfaction questionnaire; (4) 1-month (T2) and (5) 2-month (T3) post-workshop questionnaires with the same measures as in T1. One month after the workshop, knowledge, attitudes, and perceptions toward perinatal sexuality increased significantly.

Functional mobility in walking adult population with ataxia of Charlevoix-Saguenay.

Background: This study aimed to describe lower limbs impairments, balance and activity limitations related to indoor mobility in adult walkers with autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS).

Results: Twenty-five participants were recruited with a mean age of 32.2 (± 10.

Testing an Online Training Session on Couples' Perinatal Sexual Changes Among Health Care Professionals and Paraprofessionals.

Introduction: During and after pregnancy, couples have to adapt to sexual changes. Although many couples have questions and preoccupations regarding those changes, they are rarely addressed by professionals and paraprofessionals in perinatal health care, mainly because of lack of knowledge and training. An online training session that addressed couples' perinatal sexual changes tailored for health care perinatal professionals (nurses and midwives) and paraprofessionals (doulas) was developed, implemented, and evaluated.

Effects and Acceptability of an Individualized Home-Based 10-Week Training Program in Adults with Myotonic Dystrophy Type 1.

Background: Muscle weakness is a cardinal sign of myotonic dystrophy type 1, causing important functional mobility limitations and increasing the risk of falling. As a non-pharmacological, accessible and safe treatment for this population, strength training is an intervention of choice.

Objective: To document the effects and acceptability of an individualized semi-supervised home-based exercise program on functional mobility, balance and lower limb strength, and to determine if an assistive training device has a significant impact on outcomes.

Couple Adaptation to the Birth of a Child: The Roles of Attachment and Perfectionism.

This study aims to examine two explanatory factors of relationship satisfaction and parental alliance among both parents of a new child, namely romantic attachment and perfectionism. A sample of 80 couples completed individual online questionnaires. Path analyses based on the Actor-Partner Interdependence Model were carried out to explore the actor and partner effects of each explanatory variable (attachment, perfectionism) on both partners' postnatal relationship satisfaction and parental alliance.

The role of clothing on participation of persons with a physical disability: A scoping review.

Determining ways to facilitate participation of persons with a physical disability is crucial and clothing may play a central role. This review aims to synthesize and examine the role of clothing on participation of persons with a physical disability. Six research databases and grey literature were searched following Arksey & O'Malley's six steps, including multiple expert consultations.

Development and validation of a disease severity index for ataxia of Charlevoix-Saguenay.

Objective: To develop a disease-specific severity index for adults with autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) (DSI-ARSACS) that considers the 3 components (pyramidal, cerebellar, neuropathic) of the disease, and to document its content validity, internal consistency, and construct validity.

Methods: The Beta DSI-ARSACS (17 items) was developed based on literature review and expert inputs and then administered to 26 participants. Items reduction was based on Cronbach α and desirable criteria.

Inclusion of Real-Time Hand Hygiene Observation and Feedback in a Multimodal Hand Hygiene Improvement Strategy in Low-Resource Settings.

Importance: Hand hygiene adherence monitoring and feedback can reduce health care-acquired infections in hospitals. Few low-cost hand hygiene adherence monitoring tools exist in low-resource settings.

Objective: To pilot an open-source application for mobile devices and an interactive analytical dashboard for the collection and visualization of health care workers' hand hygiene adherence data.

Expanding the clinical description of autosomal recessive spastic ataxia of Charlevoix-Saguenay.

Background And Purpose: Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) diagnosis is based on the presence of three main clinical features: 1) ataxia, 2) pyramidal involvement, and 3) axonal neuropathy. This study aimed to explore, among a cohort of adults with ARSACS, the prevalence of other signs and symptoms than those commonly describe in this disease and compare their prevalence between younger (<40 years) and older (≥40 years) participants.

Methods: A clinical interview based on a standardized questionnaire was conducted.

Trauma périnéal à l'accouchement, intimité, ajustement dyadique et fonction sexuelle de nouveaux couples parentaux.

Objective: This study focuses on perineal trauma during childbirth and its relationship to postnatal marital and sexual issues as expressed by the sexual function and perceived intimacy of new parent couples in Ontario.

Methods: As part of a retrospective study on perinatal intimate and sexual experiences, 67 Canadian French-speaking couples from Ontario (heterosexual couples with their first child between 6 and 12 months of age) were recruited. They filled out online questionnaires that included questions about the severity of the perineal trauma and validated questionnaires that evaluated perceived sexual intimacy, dyadic adjustment, and sexual function.

From motor performance to participation: a quantitative descriptive study in adults with autosomal recessive spastic ataxia of Charlevoix-Saguenay.

Background: Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay (ARSACS) is a recessive neurological disorder with cerebellar, pyramidal and neuropathic features. Natural history data are urgently needed to increase trial readiness. This study aimed to describe the clinical phenotype including dexterity, coordination, strength, mobility, balance, disease severity, participation, and quality of life observed in adults with ARSACS homozygous for the c.

An exploratory natural history of ataxia of Charlevoix-Saguenay: A 2-year follow-up.

Objective: To document the decline of upper and lower limb functions, mobility, and independence in daily living activities in adults with autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) over a 2-year period.

Methods: An exploratory longitudinal design was used. Nineteen participants were assessed on 2 occasions 2 years apart.

Assessing mobility and balance in Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay population: Validity and reliability of four outcome measures.

Objective: To assess the construct validity of the 10-Meter Walk Test (10mWT), Six-Minute Walk Test (6MWT), Berg Balance Scale (BERG), and Timed Up and Go (TUG) in adults with Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay (ARSACS), in addition to the interrater reliability of the 10mWT and 6MWT.

Methods: Reliability was determined using the intraclass correlation coefficient (ICC). Validity was determined first by correlating the 10mWT, 6MWT, BERG, and TUG with participant's age, lower limb coordination, and disease severity, and then by assessing their capacity to distinguish between participants based on sex and disease stages.

The role of clothing in participation of persons with a physical disability: a scoping review protocol.

Introduction: Clothing is an important aspect of nearly all human societies from performing social and cultural functions to indicating social status, a form of protection and a way for self-expression. It can help or hinder the ability to fulfil everyday activities and social roles and with the rising industry of wearable technologies, smart textiles are adding health-monitoring functions to clothing. The influence that clothing can have on the life of someone with a physical disability is significant, and further research is needed to understand it better.

Validity and Reliability of Outcome Measures Assessing Dexterity, Coordination, and Upper Limb Strength in Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay.

Objective: To document in adults affected by autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) the intra- and interrater reliability, standard error of measurement, agreement, minimal detectable change, and construct validity of the 9-Hole Peg Test (NHPT), the Standardized Finger-to-Nose Test (SFNT), and grip strength.

Design: Metrologic study.

Setting: Neuromuscular rehabilitation clinic.