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The Traumatic Grief Inventory Self-Report (TGI-SR), which aims to assess both Persistent Complex Bereavement Disorder and Prolonged Grief Disorder, has been validated in several languages. This study sought to validate the French-Canadian version. We conducted an online survey exploring the impact of the COVID-19 pandemic on grief. With data from 728 participants, the scale demonstrated high internal consistency, correlated significantly with three other scales known to measure similar concepts, and distinguished between groups known to be different. This study supports the use of the TGI-SR French-Canadian version by clinicians and researchers to assess complications of grief.

This article presents findings from a study that investigated the impacts of the COVID-19 pandemic on domestic violence shelters’ policies and practices. This study was conducted in partnership with feminist organisations in two regions in the Quebec, Canada. Qualitative data were collected from nine domestic violence shelters, using a web-based questionnaire. Thematic content analysis was conducted using NVivo. The research findings reveal that the COVID-19 pandemic has created significant challenges for shelters, as they have had to ensure women’s and children’s safety while preventing the spread of the virus. In this context, they have had to adapt their services and practices, and it has sometimes been difficult to maintain their feminist approach. Nonetheless, shelters have been creative and have developed multiple strategies to overcome these challenges and to ensure women’s and children’s access to services. The research findings contribute to our understanding of the impacts of the COVID-19 pandemic, and highlight the essential role that these organisations have played to ensure women’s and children’s safety at a time when they have been particularly vulnerable.

Study Design This study is a case series of 48 patients. Objectives This study’s primary objective was to provide a clinical description of a group of individuals with a working diagnosis of idiopathic spinal cord herniation (ISCH). The secondary objectives were to appreciate the natural history of these patients and describe their clinical evolution with conservative or surgical management. Setting The study was carried out at l’Hôpital de l’Enfant-Jésus, CHU de Québec (Québec, Canada), a tertiary care university hospital. Methods This case series study is based on routinely collected data. Forty-eight (48) cases were identified as having an ISCH on MR imaging, between 2009 and 2019. Their medical files have been searched retrospectively. Patient characteristics were described according to their asymptomatic or symptomatic status. Results The mean age of patients at the time of diagnosis was 52.5 years. Most of the patients identified were asymptomatic (69%) and followed clinically. The main neurologic presentation for the symptomatic group was Brown-Séquard-like syndrome. 20% of the symptomatic patients were rapidly treated surgically after consultation with the neurosurgeon. The mean follow-up duration was 56 months for asymptomatic patients and 51 months for symptomatic patients. Most of our patients (41 out of 45) were considered stable or unchanged at follow-up. There was no neurological progression in all asymptomatic patients. Conclusions Our study shows that ISCH and its variants are not always symptomatic and may be a fortuitous finding. As the natural history may be non-progressive, it is probably appropriate to treat some cases expectantly.

Theatre has come to be one of the main artistic ways to convey the voices of people with disability to the general audience, and this includes people with language and communication disorders. This article aims to describe and discuss the activities of Le Théâtre Aphasique, a non-profit organization located in several cities in Quebec that delivers dramatic art workshops to people with aphasia, an acquired language disorder. Our interest is to understand how this activity has become a successful phenomenon in different disciplines, with different a priori theoretic frameworks, such as disability arts as well as rehabilitation sciences. Specifically, we suggest that the complexity and means of this activity are indeed the cause of its success given that 1) it allows people with different communication abilities to participate, while 2) encouraging the presence of people with disabilities in the theatrical scene and 3) supporting the understanding and use of different communication styles, from both individual and social perspectives. We believe that a framework built upon social, artistic, and care-driven views of disability can account for the complexity of this activity and nourish further research into how different models of disability are integrated in the experience of people with aphasia.

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 (± 10.4) years with 45.7% using a walking aid. There is a significant difference between participants with and without a walking aid in terms of lower limbs coordination, balance and mobility. Although participants who walk without a walking aid perform better than the others and they are below predictive or reference values. Despite significant mobility limitations, only mild spasticity and passive range of motion limitations were observed. However, there is a significant difference between unaffected individuals and participants with ARSACS for lower limb muscle cocontraction. Conclusions Results show a high level of lower limb impairments, balance and mobility limitation in adults’ participants with ARSACS that are still walking, including people not using a walking aid. One of the most original finding is the presence of excessive cocontraction and a relatively mild level of spasticity in the lower limbs muscles. Results of this study better circumscribes the impairments and activities that should be the focus of intervention including rehabilitation in ARSACS.

Muscle weakness and atrophy are clinical hallmarks of myotonic dystrophy type 1 (DM1). Muscle stem cells, which contribute to skeletal muscle growth and repair, are also affected in this disease. However, the molecular mechanisms leading to this defective activity and the impact on the disease severity are still elusive. Here, we explored through an unbiased approach the molecular signature leading to myogenic cell defects in DM1. Single cell RNAseq data revealed the presence of a specific subset of DM1 myogenic cells expressing a senescence signature, characterized by the high expression of genes related to senescence-associated secretory phenotype (SASP). This profile was confirmed using different senescence markers in vitro and in situ. Accumulation of intranuclear RNA foci in senescent cells, suggest that RNA-mediated toxicity contribute to senescence induction. High expression of IL-6, a prominent SASP cytokine, in the serum of DM1 patients was identified as a biomarker correlating with muscle weakness and functional capacity limitations. Drug screening revealed that the BCL-XL inhibitor (A1155463), a senolytic drug, can specifically target senescent DM1 myoblasts to induce their apoptosis and reduce their SASP. Removal of senescent cells re-established the myogenic function of the non-senescent DM1 myoblasts, which displayed improved proliferation and differentiation capacity in vitro; and enhanced engraftment following transplantation in vivo. Altogether this study presents a well-defined senescent molecular signature in DM1 untangling part of the pathological mechanisms observed in the disease; additionally, we demonstrate the therapeutic potential of targeting these defective cells with senolytics to restore myogenesis.

Daytime sleepiness and fatigue are prominent symptoms of myotonic dystrophy type 1 (DM1) that can be amenable to treatment in the context of randomized controlled trials. No study has yet documented whether self-reported measures of daytime sleepiness and fatigue can detect change over time and the meaning of this change. The aim was to explore indicators of responsiveness to change and interpretability for the Daytime Sleepiness Scale and the Fatigue Severity Scale in 115 DM1 prospectively followed patients. Results suggest that these two self-reported questionnaires are sufficiently sensitive to detect changes beyond expected measurement error over time in this population.

Background The childhood phenotype of myotonic dystrophy type 1 (DM1) involves impaired cognitive functioning starting in infancy, which may compromise later on their ability to carry out instrumental activities of daily living (IADLs) necessary for living independently. The current study aims to document the ability to perform IADLs among adults with the childhood phenotype of DM1 and to explore its links to cognitive functioning. Methods This cross-sectional exploratory study was conducted among 11 individuals living with DM1. IADLs related to money management, home management & transportation and health & safety activities were assessed by the Independent Living Scale (ILS). Neuropsychological tests assessed participants’ intellectual abilities and executive functioning. Associations were investigated using Spearman’s rho correlation. Results Important difficulties were found in all three categories of IADLs, mostly in money management in which only 2/11 participants were scored as independent. 8/11 participants showed low to very low intellectual functioning and limit to impaired executive functioning. Apathy was also a common feature as 5/11 participants showed clinical level of apathy. A lower IQ was associated with greater difficulty in the home management & transportation subtest of the ILS. Conclusions Adults with the childhood phenotype of DM1 demonstrate relative dependence in regard to the following IADLs: money management and home management & transportation. Level of dependence is, at least partially, associated with cognitive impairments. The work relates to results from an exploratory study; thus, studies must be pursued to describe in more details difficulties experienced by this population.

Immunosuppressants are associated with serious and often life-threatening adverse effects. To optimize immunotherapy, a tool that measures the immune reserve is necessary. We validated that a cell-based assay that measures TNF-α production by CD14+16+ intermediate monocytes following stimulation with EBV peptides has high sensitivity for the detection of over-immunosuppression (OIS) events. To develop a sequential, two-step assay with high specificity, we used PBMCs from kidney recipients (n = 87). Patients were classified as cases or controls, according to the occurrence of opportunistic infection, recurring bacterial infections, or de novo neoplasia. Patients who tested positive in the first step were randomly allocated to a training or a testing set for the development of the second step. In the discovery phase, an assay based on the examination of early mature B (eBm5) cells was able to discriminate OIS patients from controls with a specificity of 88%. The testing set also revealed a specificity of 88%. The interassay coefficient of variability between the experiments was 6.1%. Stratified analyses showed good diagnostic accuracy across tertiles of age and time posttransplant. In the adjusted model, the risk of OIS was more than 12 times higher in patients classified as positive than in those who tested negative (adjusted hazard ratio, 12.2; 95% confidence interval: 4.3–34.6). This sequential cell-based assay, which examines the monocyte and eBm5 cell response to EBV peptides, may be useful for identifying OIS in immunosuppressed patients.

Background. Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is a progressive disorder where upper and lower extremities motor performances may bring participation restriction. Purpose. To document participation in adults with ARSAC and explore associations with motor performances. Method. Twenty-eight participants took part in the study. Participation was assessed using the LIFE-H. Motor performance was assessed using several outcomes including the SARA, LEMOCOT, Berg Balance Scale, 10-Meter Walk Test, and Finger-to-nose Test. Findings. Participation was significantly lower in the wheelchair user subgroup. Also, for 29 activities out of 77, at least 15% of participants reported severely disrupted participation. Participation was correlated with upper and lower limbs coordination, walking ability, balance, disease severity, and fine dexterity (Spearman r = .41–0.85, p

Myotonic dystrophy type 1 (DM1) is a neuromuscular disease affecting many systems and for which muscle weakness is one of the cardinal symptoms. People with DM1 also present with balance-related impairments and high fall risk. The aim of this study was to explore explanatory factors of dynamic balance impairment in the DM1 population.A secondary analysis of data collected as part of a larger study was performed. The Mini Balance Evaluation System Test (Mini-BESTest) was used to assess dynamic balance. Age, sex, and CTG repeat length in blood were retrieved from medical records and research files. The maximal isometric muscle strength of five lower limb muscle groups (hip flexors and extensors, knee flexors and extensors, and ankle dorsiflexors) was quantitatively assessed as well as fatigue. Standard multiple regression analysis was used.Fifty-two individuals (31 men) aged between 24 and 81 years were included. The final model explains 65.9% of the balance score; ankle dorsiflexor muscle strength was the strongest explanatory factor, followed by CTG repeat length, age and fatigue to a lesser extent.Dynamic balance is impaired in people with DM1. Results of this study suggest that rehabilitation interventions aimed at improving strength of the ankle dorsiflexors and managing fatigue could help to improve dynamic balance in this specific population.

Introduction In this study we aimed to document the prevalence and age of onset of motor impairments and other key symptoms in oculopharyngeal muscular dystrophy (OPMD). Methods Retrospective chart review of patients followed at the Saguenay Neuromuscular Clinic (Quebec, Canada). Results A total of 333 participants with the (GCN)13 mutation were included. Before the age of 75 years, 27% of them had walking limitations, 14% could not climb stairs independently, and 14% used a wheelchair for long distances or daily living. The median age of onset was 54 years for ptosis and dysphagia and 58 years for lower limb proximal weakness. Other frequent symptoms included fatigue, pharyngeal pooling of thickened secretions, and dysphonia. The median age at death was 77 years and the main cause was respiratory disease. Discussion This study provides important information to help anticipatory guidance for affected people and for the development of therapeutic trials in OPMD.

An online conference was organized to promote the Interprofessional Share Decision-Making model and maximize its dissemination among stakeholders (citizens, health care providers, administrative staffs, policy makers, academics) in Canada. The goal was to enhance knowledge and engagement to share decision-making in a patient-oriented approach since shared decision-making hampered by reduced face-to-face contact, fear of infection and overworked health professionals due to the COVID-19 pandemic. Therefore, we share a summary of what has been done to engage citizens on shared making and to feed the interprofessional community on active citizen engagement in online conference. This article is protected by copyright. All rights reserved.