Quantitative muscle analysis in facioscapulohumeral muscular dystrophy using whole-body fat-referenced MRI: Protocol development, multicenter feasibility, and repeatability.

INTRODUCTION/AIMS: Functional performance tests are the gold standard to assess disease progression and treatment effects in neuromuscular disorders. These tests can be confounded by motivation, pain, fatigue, and learning effects, increasing variability and decreasing sensitivity to disease progression, limiting efficacy assessment in clinical trials with small sample sizes. We aimed to develop and validate a quantitative and objective method to measure skeletal muscle volume and fat content based on whole-body fat-referenced magnetic resonance imaging (MRI) for use in multisite clinical trials. METHODS: Subjects aged 18 to 65 years, genetically confirmed facioscapulohumeral muscular dystrophy 1 (FSHD1), clinical severity 2 to 4 (Ricci's scale, range 0-5), were enrolled at six sites and imaged twice 4-12 weeks apart with T1-weighted two-point Dixon MRI covering the torso and upper and lower extremities. Thirty-six muscles were volumetrically segmented using semi-automatic multi-atlas-based segmentation. Muscle fat fraction (MFF), muscle fat infiltration (MFI), and lean muscle volume (LMV) were quantified for each muscle using fat-referenced quantification. RESULTS: Seventeen patients (mean age ± SD, 49.4 years ±13.02; 12 men) were enrolled. Within-patient SD ranged from 1.00% to 3.51% for MFF and 0.40% to 1.48% for MFI in individual muscles. For LMV, coefficients of variation ranged from 2.7% to 11.7%. For the composite score average of all muscles, observed SDs were 0.70% and 0.32% for MFF and MFI, respectively; composite LMV coefficient of variation was 2.0%. DISCUSSION: We developed and validated a method for measuring skeletal muscle volume and fat content for use in multisite clinical trials of neuromuscular disorders.

Recovery-oriented service provision and clinical outcomes in assertive community treatment.

OBJECTIVE: While the term "recovery" is routinely referenced in clinical services and health policy, few studies have examined the relationship between recovery-oriented service provision and client outcomes. The present study was designed to examine the relationship between recovery-orientation of service provision for persons with severe mental illnesses and outcomes in Assertive Community Treatment (ACT). METHODS: Client, family, staff, and manager ratings of service recovery-orientation and outcomes across a range of service utilization and community functioning indicators were examined among 67 ACT teams in Ontario, Canada. RESULTS: Significant associations were found between ratings of recovery-oriented service provision and better outcomes in the domains of legal involvement, hospitalization days, education involvement, and employment. Results were not uniformly positive or consistent, however, across stakeholder Recovery Self-Assessment (RSA) ratings or outcomes. CONCLUSIONS AND IMPLICATIONS FOR PRACTICE: These findings provide some preliminary support for an association between recovery-oriented service delivery for persons with severe mental illnesses and better outcomes. In line with the current practice commentary, this association would suggest the importance of evaluating and cultivating recovery-oriented values and practices in ACT contexts. This is a particularly salient point given that ACT standards minimally address key domains of recovery-oriented service provision. Further study is required, however, to determine if these findings apply to the implementation of ACT in other jurisdictions or generalize to other community support programs.