Singing and Dancing With Neuromuscular Conditions: A Mixed-Methods Study.

The purpose of this study was to assess the safety and meaningfulness of a 15-week recreational dance and singing program for people with neuromuscular conditions. Within a transformative mixed-methods design, pulmonary function tests, plethysmography through wearable technology (Hexoskin vests), individualized neuromuscular quality-of-life assessments (version 2.0), and semistructured interviews were used. The interviews were analyzed through inductive, semantic thematic analysis. Although the sample sizes were small (six people with neuromuscular conditions), the authors found no evidence of safety concerns. There was evidence of respiratory improvements and reported improvements in swallowing and speech. The most notable quality-of-life changes included improvements related to weakness, swallowing, relationships, and leisure. The participants shared that the program offered meaningful social connection and embodied skills and safe and pleasurable physical exertion. The authors learned that recreational singing and dancing programs could be a safe and deeply meaningful activity for those with neuromuscular conditions that impact respiration.

Systematic Review and Expert Consensus on the Use of Orthoses (Splints and Casts) with Adults and Children after Burn Injury to Determine Practice Guidelines.

The objective of this review was to systematically evaluate the available literature addressing the use of orthoses (splints and casts) with adult and pediatric burn survivors and determine whether practice guidelines could be proposed. This review provides evidence-based recommendations specifically for rehabilitation professionals who are responsible for burn survivor rehabilitation. A summary recommendation was made after the literature was retrieved using a systematic review and critical appraisal by multiple authors. The level of evidence of the literature was determined in accordance with the Oxford Centre for Evidence-based Medicine criteria. Due to the low level of evidence in the available literature, only one practice guideline could be recommended: orthotic use should be considered as a treatment choice for improving range of motion or reducing contracture in adults who have sustained a burn injury. To address the rehabilitation-specific gaps found in the literature regarding orthotic use in burn rehabilitation and provide guidance to clinicians, a formal expert consensus exercise was conducted as a final step to the project. The resultant manuscript provides a summary of the literature regarding orthotic use with burn patients, one practice guideline, proposed orthotic terminology and additional practice recommendations based on expert opinion. The limitations in the current literature are also discussed, and suggestions are made for future studies in the area of orthotic use after burn injury.

What is the Fracture Risk in Patients at a Multidisciplinary Neuromuscular Clinic?

Many neuromuscular diseases (NMD) result in muscle weakness, immobility and greater fracture risk. The objective of this study is to determine the fracture risk of adult patients at a multidisciplinary NMD clinic. Fracture risk was calculated using the Fracture Risk Assessment Tool, the presence of osteoporosis was quantified using bone densitometry and contributing co-morbidities were screened through serum markers. Of the 36 patients studied, 47% were found to be of moderate and high fracture risk. Two thirds of these patients had not been previously screened or treated for osteoporosis. These findings suggest that NMD patients warrant routine screening for osteoporosis and early treatment to reduce fragility fracture.

Secondary hemochromatosis as a result of acute transfusion-induced iron overload in a burn patient.

BACKGROUND: Red blood cell transfusions are critical in burn management. The subsequent iron overload that can occur from this treatment can lead to secondary hemochromatosis with multi-organ damage. CASE PRESENTATION: While well recognized in patients receiving chronic transfusions, we present a case outlining the acute development of hemochromatosis secondary to multiple transfusions in a burn patient. CONCLUSIONS: Simple screening laboratory measures and treatment options exist which may significantly reduce morbidity; thus, we believe awareness of secondary hemochromatosis in those treating burn patients is critical.

Practice Guidelines for Cardiovascular Fitness and Strengthening Exercise Prescription After Burn Injury.

The objective of this review was to systematically evaluate the available clinical evidence for the prescription of strength training and cardiovascular endurance exercise programs for pediatric and adult burn survivors so that practice guidelines could be proposed. This review provides evidence-based recommendations specifically for rehabilitation professionals who are responsible for burn survivor rehabilitation. Summary recommendations were made after the literature was retrieved by systematic review, was critically appraised by multiple authors and the level of evidence determined in accordance with the Oxford Centre for Evidence-based Medicine criteria. Although gaps in the literature persist and should be addressed in future research projects, currently, strong research evidence supports the prescription of strength training and aerobic conditioning exercise programs for both adult and pediatric burn survivors when in the presence of strength limitations and/or decreased cardiovascular endurance after evaluation.

Axial signs and magnetic resonance imaging correlates in Parkinson's disease.

BACKGROUND: Age-related brain changes may contribute to axial features in Parkinson's disease (PD). OBJECTIVES: To determine if ventricular volume and white matter high signal changes (WMC) are related to motor signs in PD and controls independent of age. METHODS: Patients were rated with the Unified Parkinson's Disease Rating Scale (subscore A: tremor, rigidity, bradykinesia, and facial expression; subscore B: speech and axial impairment). Steps and time taken to walk 9.144 meters were measured. Total ventricular volume (TVV) and intracranial volume (ICV) were measured on T1-weighted MRI using manual tracing software. WMC were rated on axial T2-weighted, dual-echo or FLAIR MR images using a visual scale. RESULTS: TVV (cm3) (PD: 36.48 +/- 15.93; controls: 32.16 +/- 14.20, p = 0.21) and WMC did not differ between groups (PD: 3.7 +/- 4.2; controls: 3.2 +/- 3.1, p = 0.55). Age correlated positively with ICV-corrected TVV and WMC in PD (cTVV: r = 0.48, p = 0.003; WMC: r = 0.42, p = 0.01) and controls (cTVV: r = 0.31, p = 0.04; WMC: r = 0.44, p = 0.003). Subscore B (r = 0.42, p = 0.01) but not subscore A (r = 0.25, p = 0.14) correlated with cTVV in PD. Steps and walking time correlated with cTVV and WMC in PD; cadence correlated with cTVV and steps with WMC in controls. Age-adjustment eliminated correlations. CONCLUSION: Subscore B, but not subscore A correlated positively with ventricular volume in PD, though this association was accounted for by age. Age-related brain change super-imposed on PD may contribute to axial features.

Behavioral and electromyographic characterization of mice lacking EphA4 receptors.

EphA4 receptors play an important role in axon guidance during development. Disrupting the expression of these receptors in mice has been shown to modify neuronal connections in the spinal cord and results in the production of a characteristic hopping gait. The EphA4-null mouse has been used in numerous investigations aimed at establishing mechanisms responsible for patterning motor activity during walking. However, there have been no detailed behavioral or electrophysiological studies on adult EphA4-null mice. We used high-speed video recordings to determine the coordination of leg movements during locomotion in adult EphA4-null mice. Our data show that the hopping movements of the hind legs are not always associated with synchronous movements of forelegs. The coupling between the forelegs is weak, resulting in changes in their phase relationship from step to step. The synchronous coordination of the hind legs can switch to an alternating pattern for a short period of time during recovery from isoflurane anesthesia. Comparison of the kinematics of hind leg movements in EphA4-null mice and wild-type animals shows that besides the synchronous coordination in EphA4-null mice, the swing durations and the swing amplitude are shorter. Electromyographic recordings from a knee extensor muscle show double bursting in the EphA4-null animals but single bursts in wild types. This double burst changes to single-burst activity during swimming and when hind legs are stepping in alternation. These observations suggest an influence of sensory feedback in shaping the pattern of muscle activity during locomotion in the mutant animals. Our data give the first detailed description of the locomotor behavior of an adult mouse with genetically manipulated spinal networks.