Exercise Training for autoimmune myasthenia gravis: A review of safety and effectiveness based on existing literature

No abstract is required for a review article as per instruction

Gait characterization in golden retriever muscular dystrophy dogs using linear discriminant analysis

Background: Accelerometric analysis of gait abnormalities in golden retriever muscular dystrophy (GRMD) dogs isof limited sensitivity, and produces highly complex data. The use of discriminant analysis may enable simpler andmore sensitive evaluation of treatment benefits in this important preclinical model.Methods: Accelerometry was performed twice monthly between the ages of 2 and 12 months on 8 healthy and20 GRMD dogs. Seven accelerometric parameters were analysed using linear discriminant analysis (LDA). Manipulationof the dependent and independent variables produced three distinct models. The ability of each model to detect gaitalterations and their pattern change with age was tested using a leave-one-out cross-validation approach.Results: Selecting genotype (healthy or GRMD) as the dependent variable resulted in a model (Model 1) allowing agood discrimination between the gait phenotype of GRMD and healthy dogs. However, this model was not sufficientlyrepresentative of the disease progression. In Model 2, age in months was added as a supplementary dependentvariable (GRMD_2 to GRMD_12 and Healthy_2 to Healthy_9.5), resulting in a high overall misclassification rate (83.2%).To improve accuracy, a third model (Model 3) was created in which age was also included as an explanatory variable.This resulted in an overall misclassification rate lower than 12%. Model 3 was evaluated using blinded data pertainingto 81 healthy and GRMD dogs. In all but one case, the model correctly matched gait phenotype to the actualgenotype. Finally, we used Model 3 to reanalyse data from a previous study regarding the effects ofimmunosuppressive treatments on muscular dystrophy in GRMD dogs. Our model identified significant effect ofimmunosuppressive treatments on gait quality, corroborating the original findings, with the added advantages ofdirect statistical analysis with greater sensitivity and more comprehensible data representation.Conclusions: Gait analysis using LDA allows for improved analysis of accelerometry data by applying adecision-making analysis approach to the evaluation of preclinical treatment benefits in GRMD dogs

Determinants of Performance in the Timed Up-and-Go and Six-Minute Walk Tests in Young and Old Healthy Adults

The aim of this study was to assess associations between performance in the timed up-and-go (TUG) and six-minute walk distance (6MWD) with physiological characteristics in young and old healthy adults. Thereto, we determined TUG, 6MWD, normalised jump power, centre of pressure displacement during 1-leg standing, forced expiratory volume in 1 s, percentage of age-predicted maximal heart rate (HR%) and height in 419 healthy young (men: 23.5 ± 2.8 years, women: 23.2 ± 2.9 years) and old (men: 74.6 ± 3.2 years, women: 74.1 ± 3.2 years) adults. Normalised jump power explained 8% and 19% of TUG in young (p = 0.025) and older men (p < 0.001), respectively. When fat mass percentage and age were added to normalised jump power, 30% of TUG was explained in older men (R2adj = 0.30, p < 0.001 to 0.106). Appendicular lean muscle mass percentage (ALM%) and age were the best determinants of TUG for older women (R2adj = 0.16, p < 0.001 to 0.01). HR% explained 17–39% of 6MWD across all groups (R2adj = 0.17 to 39, p < 0.001). In conclusion, in men, jump power was a key determinant for TUG, while in old women only it was the ALM%. As HR% was the most important determinant of 6MWD, motivational bias needs to be considered in the interpretation of this test

Longitudinal ambulatory measurements of gait abnormality in dystrophin-deficient dogs

Chantier qualité GAInternational audienceABSTRACT: BACKGROUND: This study aimed to measure the gait abnormalities in GRMD (Golden retriever muscular dystrophy) dogs during growth and disease progression using an ambulatory gait analyzer (3D-accelerometers) as a possible tool to assess the effects of a therapeutic intervention. METHODS: Six healthy and twelve GRMD dogs were evaluated twice monthly, from the age of two to nine months. The evolution of each gait variable previously shown to be modified in control and dystrophin-deficient adults was assessed using two-ways variance analysis (age, clinical status) with repeated measurements. A principal component analysis (PCA) was applied to perfect multivariate data interpretation. RESULTS: Speed, stride length, total power and force significantly already decreased (p < 0.01) at the age of 2 months. The other gait variables (stride frequency, relative power distributions along the three axes) became modified at later stages. Using the PCA analysis, a global gait index taking into account the main gait variables was calculated, and was also consistent to detect the early changes in the GRMD gait patterns, as well as the progressive degradation of gait quality. CONCLUSION: The gait variables measured by the accelerometers were sensitive to early detect and follow the gait disorders and mirrored the heterogeneity of clinical presentations, giving sense to monitor gait in GRMD dogs during progression of the disease and pre-clinical therapeutic trials

Determining minimal clinically important differences in the North Star Ambulatory Assessment (NSAA) for patients with Duchenne muscular dystrophy

The North Star ambulatory assessment (NSAA) is a functional motor outcome measure in Duchenne muscular dystrophy (DMD), widely used in clinical trials and natural history studies, as well as in clinical practice. However, little has been reported on the minimal clinically important difference (MCID) of the NSAA. The lack of established MCID estimates for NSAA presents challenges in interpreting the significance of the results of this outcome measure in clinical trials, natural history studies and clinical practice. Combining statistical approaches and patient perspectives, this study estimated MCID for NSAA using distribution-based estimates of 1/3 standard deviation (SD) and standard error of measurement (SEM), an anchor-based approach, with six-minute walk distance (6MWD) as the anchor, and evaluation of patient and parent perception using participant-tailored questionnaires. The MCID for NSAA in boys with DMD aged 7 to 10 years based on 1/3 SD ranged from 2.3-2.9 points, and that on SEM ranged from 2.9-3.5 points. Anchored on the 6MWD, the MCID for NSAA was estimated as 3.5 points. When the impact on functional abilities was considered using participant response questionnaires, patients and parent perceived a complete loss of function in a single item or deterioration of function in one to two items of the assessment as an important change. Our study examines MCID estimates for total NSAA scores using multiple approaches, including the impact of patient and parent perspective on within scale changes in items based on complete loss of function and deterioration of function, and provides new insight on evaluation of differences in these widely used outcome measure in DMD

Circulating levels of adipokines and IGF-1 are associated with skeletal muscle strength of young and old healthy subjects

n/

Handgrip strength cannot be assumed a proxy for overall muscle strength

Objectives: Dynapenia, low muscle strength, is predictive for negative health outcomes and is usually expressed as handgrip strength (HGS). Whether HGS can be a proxy for overall muscle strength and whether this depends on age and health status is controversial. This study assessed the agreement between HGS and knee extension strength (KES) in populations differing in age and health status. Design: Data were retrieved from 5 cohorts. Setting and Participants: Community, geriatric outpatient clinics, and a hospital. Five cohorts (960 individuals, 49.8% male) encompassing healthy young and older individuals, geriatric outpatients, and older individuals post hip fracture were included. Measures: HGS and KES were measured according to the protocol of each cohort. Pearson correlation was performed to analyze the association between HGS and KES, stratified by sex. HGS and KES were standardized into sex-specific z scores. The agreement between standardized HGS and standardized KES at population level and individual level were assessed by intraclass correlation coefficients (ICC) and Bland-Altman analysis. Results: Pearson correlation coefficients were low in healthy young (male: 0.36 to 0.45, female: 0.45) and healthy older individuals (male: 0.35 to 0.37, female: 0.44), and moderate in geriatric outpatients (male and female: 0.54) and older individuals post hip fracture (male: 0.44, female: 0.57) (P<.05, except for male older individuals post hip fracture [P¼.07]). Intraclass correlation coefficient values were poor to moderate in all populations (ie, healthy young individuals [0.41, 0.45], healthy older individuals [0.37, 0.41, 0.44], geriatric outpatients [0.54], and older individuals post hip fracture [0.54]). Bland-Altman analysis showed that within the same population of age and health status, agreement between HGS and KES varied on individual level