Cross-cultural adaptation of the SWAL-QOL and the Sydney Swallow Questionnaire (SSQ) into French-Canadian and preliminary assessment for their use in an oculopharyngeal muscular dystrophy (OPMD) population.

PURPOSE: Patient-reported outcomes (PRO) assessing dysphagia are an essential component of clinical trials to consider how patients feel and function in response to treatments. The selected PRO needs to be translated in several languages using a systematic process ensuring its validity and equivalence for use in multicenter clinical trials. The main objectives were to translate the SWAL-QOL and the Sydney Swallow Questionnaire (SSQ) into French Canadian (SWAL-QOL-FC and SSQ-FC) and to assess their appropriateness for patients with oculopharyngeal muscular dystrophy (OPMD). METHODS: A forward- and back-translation process was followed including independent mother-tongue translators followed by committee review. Known-groups validity was assessed by comparing scores of OPMD French-Canadian participants (n = 21) known to differ in dysphagia severity according to the 80-ml drinking test score. A Mann-Whitney test was used to compare the mean scores. Cognitive interviews were conducted later on to ensure a posteriori cultural equivalence among French-Canadian participants (n = 28). RESULTS: Evidences of adequate known-groups validity was shown for the SSQ-FC. Only two domains out of 10 of the SWAL-QOL-FC (burden and eating duration) showed adequate known-groups validity. In addition, the difference in the mean composite score was not significant. Several conceptual equivalence issues were found in both questionnaires as a result of the cognitive interviews. CONCLUSION: This study provided evidence that the SSQ-FC presents better validity than the SWAL-QOL-FC in a small sample size of OPMD participants. The findings of the cognitive interviews suggest the need to develop an OPMD-specific questionnaire to capture better the whole spectrum of disease severity.

Nutritional Risk in Oculopharyngeal Muscular Dystrophy: Beyond Dysphagia.

Purpose: To document the nutritional risk in adults with oculopharyngeal muscular dystrophy (OPMD) and its association with oropharyngeal dysphagia.Methods: In this cross-sectional study, 33 adults with molecular confirmation of OPMD between 50 and 75 years old were recruited from the registry of a university-affiliated neuromuscular clinic. Nutritional risk was assessed with the French version of Seniors in the Community: Risk Evaluation for Eating and Nutrition II (SCREEN II), whereas the severity of dysphagia was assessed using the French-Canadian version of the Sydney Swallow Questionnaire. Anthropometric measurements were performed with standardized procedures.Results: SCREEN II scores showed high nutritional risk for 81.8% of OPMD participants with 6 factors contributing to nutritional risk in at least 50% of the sample. Pearson's correlational analysis showed a significant moderate relationship between dysphagia and nutritional risk (r = -0.470; P = 0.006).Conclusion: To our knowledge, this study is the first to investigate the nutritional risk of adults with OPMD. Our results indicate that individuals with OPMD may be at high nutritional risk mostly associated with swallowing difficulty, in the absence of a low body mass index. The present study highlights the need for dietary counseling in OPMD.

Do classical and computerized cognitive tests have equal intrarater reliability in myotonic dystrophy type 1?

Myotonic dystrophy type 1 (DM1) is a multisystemic inherited neuromuscular disease leading to central nervous system symptoms, including cognitive impairments, among multiple other symptoms. However, information is presently lacking regarding the psychometric properties of neuropsychological tests and promising computerized cognitive tests, such as the Cambridge Neuropsychological Test Automated Battery (CANTABⓇ). This type of information is critical to improve clinical trial readiness and provide knowledge of DM1 natural history. The aims of the present study were (1) to document the intrarater reliability of classic paper-pencil tests assessing visuospatial working memory, cognitive flexibility, attention, episodic memory and apathy, and (2) to compare these findings with their equivalent computerized automated tests from the CANTABⓇ. Thirty participants were seen twice at four-week intervals. Results showed that the Stroop Color and Word Test (ICC = 0.741-0.869) and the Ruff 2 & 7 (ICC = 0.703-0.871) appear to be reliable paper-and-pencil tests in the DM1 population. For the CANTABⓇ, a similar observation was made for the Multitasking test (ICC = 0.588-0.792). Further studies should explore the applicability and concurrent validity of the CANTAB® and classic neuropsychological assessments in additional cohorts of DM1 patients.

Potential Factors Influencing Adoption of a Primary Care Pathway to Prevent Functional Decline in Older Adults.

Introduction: To help recognize and care for community-dwelling older adults living with frailty, we plan to implement a primary care pathway consisting of frailty screening, shared decision-making to select a preventive intervention, and facilitated referral to community-based services. In this study, we examined the potential factors influencing adoption of this pathway. Methods: In this qualitative, descriptive study, we conducted semi-structured interviews and focus groups with patients aged 70 years and older, health professionals (HPs), and managers from four primary care practices in the province of Quebec, representatives of community-based services and geriatric clinics located near the practices. Two researchers conducted an inductive/deductive thematic analysis, by first drawing on the Consolidated Framework for Implementation Research and then adding emergent subthemes. Results: We recruited 28 patients, 29 HPs, and 8 managers from four primary care practices, 16 representatives from community-based services, and 10 representatives from geriatric clinics. Participants identified several factors that could influence adoption of the pathway: the availability of electronic and printed versions of the decision aids; the complexity of including a screening form in the electronic health record; public policies that limit the capacity of community-based services; HPs' positive attitudes toward shared decision-making and their work overload; and lack of funding. Conclusions: These findings will inform the implementation of the care pathway, so that it meets the needs of key stakeholders and can be scaled up.

Event-related potentials of episodic encoding after traumatic brain injury in older adults.

Episodic memory and attention impairments are frequently observed following a traumatic brain injury (TBI). Older adults are more affected than young adults after a TBI, partly because of the age-related neural and memory changes. Neural mechanisms underlying episodic memory deficits in older adults with chronic TBI remain to be investigated. The current study aimed to investigate the impact of TBI in older adults on the neural mechanisms of episodic encoding. Event-related potentials were recorded while 13 participants with mild-to-severe TBI and 14 matched controls were performing an episodic memory task in which the level of organizational strategy was manipulated through three encoding conditions. Participants were explicitly instructed to memorize words without any semantic relationship (Unrelated condition), words semantically related without any given strategies (Spontaneous condition) and words semantically related with provided category labels and organizational strategy (Guided condition). Behavioral performances indicated that older individuals with a TBI were impaired compared to matched controls whatever the condition. The electrophysiological findings showed a reduction of the P200 and LPC components amplitude in the TBI group relative to control group. Moreover, control participants without any neurological history showed a right frontal sustained activity only in the Spontaneous condition, whereas a right frontal asymmetry was observed in participants with chronic TBI whatever the encoding conditions. This was mainly the result of negative left frontal activity. These findings evidence neural dysfunctions underlying attentional and associative processes involved in memory strategies after a TBI sustained at an older age that are consistent with executive functions impairments.

Accomplishment of instrumental activities of daily living and its relationship with cognitive functions in adults with myotonic dystrophy type 1 childhood phenotype: an exploratory study.

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.

Acquisition of plasmin activity and induction of arachidonic acid release by Streptococcus suis in contact with human brain microvascular endothelial cells.

Infections caused by Streptococcus suis, a major swine pathogen, include meningitis, arthritis, pneumonia and septicaemia. In this study, we investigated interactions that may occur between human brain microvascular endothelial cells (HBMEC), the main constituent of the blood-brain barrier, and S. suis. We show that S. suis acquires plasmin activity in a time-dependent manner when in contact with cultured HBMEC. Cell-associated plasmin activity reached a plateau following a 48h co-incubation period. Zymography analysis revealed that HBMEC produce urokinase, which is probably involved in activation of plasminogen bound to S. suis. We also show that a S. suis culture supernatant which possesses both phospholipase C and haemolysin (suilysin) activities was able to induce the release of arachidonic acid from the membrane of HBMEC. Evidence suggests that the action of suilysin on HBMEC may be a prerequisite for the action of additional molecules such as phospholipase C. These new biological effects associated with S. suis may play an important role in the migration of S. suis through the blood-brain barrier and in the modulation of local inflammation.