Dynamic analysis of muscles and the internal structure of the peripheral nerve as biomarkers of amyotrophic lateral sclerosis: A pilot study with ultrasound imaging.

INTRODUCTION: The aim of this study was to determine the behaviour of ultrasound biomarkers of fascicle density and muscle strength in patients with amyotrophic lateral sclerosis (ALS). METHODS: We conducted an observational, cross-sectional pilot study of 14 patients with ALS (28.6% women) and 14 controls. Bilateral cross-sectional ultrasound scans were performed in the abductor pollicis brevis (APB) and tibialis anterior (TA) muscles, with recording of muscle thickness (MT) at rest and in contraction, and the difference in thickness. In the median, sciatic, and common peroneal nerves, we analysed the cross-sectional area (CSA), number of fascicles (NF) and fascicle density (FD). Analyses were nested by laterality. RESULTS: Intra- and interrater agreement regarding NF was very good, with a minimum detectable error of < 0.7%. In patients with ALS, MT was lower in the APB both at rest (P = .003; g-Hedges = 1.03) and in contraction (P = .017; g-Hedges = 0.78) and in TA at rest (P = .002; g-Hedges = 0.15) and in contraction (P = .001; g-Hedges = 0.46), with lower thickening capacity. In the nerves, patients displayed lower CSA, with lower NF and higher FD. Significant correlations were found between MT of the ABP and Medical Research Council (MRC) scores for muscle strength (r = 0.34; r2 = 12%; P = .011) and with revised ALS Functional Rating Scale scores (r = 0.44; r2 = 19%; P < .001). The difference in TA thickening correlated with MRC scores (r = 0.30; r2 = 15%; P = .003) and with revised ALS Functional Rating Scale scores (r = 0.26; r2 = 7%; P = .049). NF in the sciatic nerve showed a significant correlation with MRC scores (r = 0.35; r2 = 12%; P = .008). CONCLUSION: MT measurements derived from dynamic testing together with NF and FD may be useful biomarkers for monitoring patients with ALS and establishing a prognosis.

From thinking to acting: occupational self-analysis tools for use with people with intellectual disability. A pilot study.

BACKGROUND: Occupational self-analysis programmes have been developed to promote health, mainly in older adults. However, we have found no published studies dealing with this in people with intellectual disability (ID). The aim of this study was to examine the effectiveness of an occupational self-analysis programme in people with ID, focusing on the areas of subjective health perception and participation. METHODS: This study explored the benefits of the 'Occupational Self-Analysis Programme' in people with ID. The intervention group comprised 12 participants and the control group comprised 13 participants. Outcomes were measured using the SF-36 Health Survey and the Role Checklist. The contents of participants' journals and a focus group discussion were also analysed. RESULTS: Compared with the control group, the intervention group showed statistically significant improvements in role limitations because of physical health and role limitations because of emotional problems, social functioning and the cumulative scores of the mental component in the SF-36 Health Survey. The qualitative analysis revealed that participants in the intervention group increased their participation in activities related with daily living, leisure, employment seeking and acquisition, and social participation. These participants also became more aware of issues that lead to a greater engagement in meaningful occupations. CONCLUSION: This programme allowed participants to engage in meaningful activities with other people and to increase their perceived health status by raising awareness of the strengths and difficulties of participation.

New insights into the pathophysiology of fasciculations in amyotrophic lateral sclerosis: An ultrasound study.

OBJECTIVE: To describe the fasciculation pattern in ALS and to analyse its clinical and pathophysiological significance. METHODS: Ultrasound of 19 muscles was performed in 44 patients with a recent diagnosis (<90 days) of ALS. The number of fasciculations was recorded in each muscle and the muscle thickness and strength were additionally measured in limb muscles. A subgroup of patients were electromyographically assessed. RESULTS: US was performed in 835 muscles and EMG was available in 263 muscles. US detected fasciculations more frequently than EMG. Fasciculations were widespread, especially in upper limbs onset patients and in the cervical region. Fasciculations' number inversely associated with ALSFR-R and body mass index (BMI) and directly with BMI loss and upper motor neuron (UMN) impairment. Our statistical model suggest that fasciculations increase with the initial lower motor neuron (LMN) degeneration, reach their peak when the muscle became mildly to moderately weak, decreasing afterwards with increasing muscle weakness and atrophy. CONCLUSIONS: Our study suggests that both UMN and LMN degeneration trigger fasciculations causing BMI loss. The degree of LMN impairment could account for differences in fasciculations' rates within and between muscles. SIGNIFICANCE: In ALS, fasciculations could explain the link between hyperexcitability and BMI loss.

Outpatients' perceptions of their experiences in musculoskeletal rehabilitation care.

BACKGROUND: While patients' satisfaction with rehabilitation care is often measured, the knowledge of their experiences during delivery of care and of which characteristics may lead them to experience problems remains largely undocumented. AIM: To assess the quality of patients' experiences receiving post-acute care for musculoskeletal disorders in outpatient rehabilitation settings. To describe the extent to which variations in reported quality of patients' experiences were related to their characteristics. DESIGN: A cross sectional, self-reported survey. SETTING: 3 outpatient rehabilitation units. POPULATION: Four hundred sixty-five outpatients. Mean age 39.4 (SD=11.9). METHODS: A problem score (from 0 to 100%) of self-reported experiences was calculated on 7 specific aspects of care (belonging to organizational environment and professionals' attitudes and behaviors) and compared among participants' subgroups. RESULTS: Mean scores of the organizational environment area showed higher amount of problems (43.3%) than the professionals' attitudes and behaviours area (34.7%). Two multivariate linear regression models (with adjusted R(2) 9.3% and 4.9%) indicated that older patients and those with high global rating change were less likely to experience problems in the two areas. CONCLUSION: Problems on aspects of care were commonly experienced by patients with musculoskeletal disorders in outpatient rehabilitation settings. The age and health of patients were associated to these problems, but explained only a small variation in them. CLINICAL REHABILITATION IMPACT: Surveys measuring patients' experiences can be used to monitor the actual performance of rehabilitation settings and to pinpoint the exact issues needing to be addressed to improve quality as well as to make comparison among centres.