Effectiveness of an outpatient rehabilitation programme in patients with neuralgic amyotrophy and scapular dyskinesia: a randomised controlled trial.

BACKGROUND: Neuralgic amyotrophy (NA) is an acute inflammation of nerves within the brachial plexus territory leading to severe pain and multifocal paresis resulting in >60% of patients having residual complaints and functional limitations correlated with scapular dyskinesia. Our primary aim was to compare the effects of multidisciplinary rehabilitation (MR), focused on motor relearning to improve scapular dyskinesia and self-management strategies for reducing pain and fatigue, with usual care (UC) on shoulder, arm and hand functional capability in patients with NA. METHODS: In a non-blinded randomised controlled trial (RCT), patients with NA (aged≥18 years, scapular dyskinesia, >8 weeks after onset) were randomised to either an MR or an UC group. MR consisted of a diagnostic multidisciplinary consultation and eight sessions of physical and occupational therapy. Primary outcome was functional capability of the shoulder, arm and hand assessed with the Shoulder Rating Questionnaire-Dutch Language Version (SRQ-DLV). RESULTS: We included 47 patients with NA; due to drop-out, there were 22 participants in MR and 15 in UC for primary analysis. The mean group difference adjusted for sex, age and SRQ-DLV baseline score was 8.60 (95%CI: 0.26 to 16.94, p=0.044). The proportion attaining a minimal clinically relevant SRQ-DLV improvement (≥12) was larger for the MR group (59%) than the UC group (33%) with a number needed to treat of 4. CONCLUSION: This RCT shows that an MR programme focused on motor relearning to improve scapular dyskinesia, combined with self-management strategies for reducing pain and fatigue, shows more beneficial effects on shoulder, arm and hand functional capability than UC in patients with NA. TRIAL REGISTRATION NUMBER: NCT03441347.

Validity and reliability of visual assessment of orofacial muscle ultrasound images using a modified Heckmatt scale.

INTRODUCTION/AIMS: Orofacial muscle ultrasound images can be evaluated quantitatively or using a visual grading system. Quantitative muscle ultrasound (QMUS) is currently the most sensitive technique to detect pathology, but can be time-consuming. The aim of this study was to investigate the validity and reliability of two visual grading systems (the original Heckmatt scale or a modified 3-point version) for the optimal grading of orofacial muscle images. METHODS: A retrospective, comparative, reliability and validity study was performed. Ultrasound images of the digastric, geniohyoid, masseter, temporalis muscles, and intrinsic muscles of the tongue of healthy participants and of patients (suspected of) having a neuromuscular disease were included. QMUS was used as the "gold standard." Two expert raters and one inexperienced rater rated all ultrasound images using both visual grading systems. RESULTS: A total of 511 ultrasound images were included. Criterion validity showed Spearman rho correlation coefficients of >0.59. Construct validity analysis showed strong to very strong associations between the visual grading systems and mastication and/or swallowing. Inter- and intrarater reliability of the original Heckmatt scale and the modified scale were good and comparable. Rater experience had a beneficial effect on the interrater reliability of both scales. DISCUSSION: Both the original Heckmatt and the modified Heckmatt scale are valid and reliable tools for the visual grading of orofacial ultrasound images. The modified Heckmatt scale, with only three grades and including an "uncertain" category, is considered easier to use in clinical practice.

Quantitative assessment of plantar pressure patterns in relation to foot deformities in people with hereditary motor and sensory neuropathies.

BACKGROUND: Hereditary motor and sensory neuropathies (HMSN), also known as Charcot-Marie-Tooth disease, are characterized by affected peripheral nerves. This often results in foot deformities that can be classified into four categories: (1) plantar flexed first metatarsal, neutral hindfoot, (2) plantar flexed first metatarsal, correctable hindfoot varus, (3) plantar flexed first metatarsal, uncorrectable hindfoot varus, and (4) hindfoot valgus. To improve management and for the evaluation of surgical interventions, a quantitative evaluation of foot function is required. The first aim of this study was to provide insight into plantar pressure of people with HMSN in relation to foot deformities. The second aim was to propose a quantitative outcome measure for the evaluation of surgical interventions based on plantar pressure. METHODS: In this historic cohort study, plantar pressure measurements of 52 people with HMSN and 586 healthy controls were evaluated. In addition to the evaluation of complete plantar pressure patterns, root mean square deviations (RMSD) of plantar pressure patterns from the mean plantar pressure pattern of healthy controls were calculated as a measure of abnormality. Furthermore, center of pressure trajectories were calculated to investigate temporal characteristics. Additionally, plantar pressure ratios of the lateral foot, toes, first metatarsal head, second/third metatarsal heads, fifth metatarsal head, and midfoot were calculated to measure overloading of foot areas. RESULTS: Larger RMSD values were found for all foot deformity categories compared to healthy controls (p < 0.001). Evaluation of the complete plantar pressure patterns revealed differences in plantar pressure between people with HMSN and healthy controls underneath the rearfoot, lateral foot, and second/third metatarsal heads. Center of pressure trajectories differed between people with HMSN and healthy controls in the medio-lateral and anterior-posterior direction. The plantar pressure ratios, and especially the fifth metatarsal head pressure ratio, differed between healthy controls and people with HMSN (p < 0.05) and between the four foot deformity categories (p < 0.05). CONCLUSIONS: Spatially and temporally distinct plantar pressure patterns were found for the four foot deformity categories in people with HMSN. We suggest to consider the RMSD in combination with the fifth metatarsal head pressure ratio as outcome measures for the evaluation of surgical interventions in people with HMSN.

Reachable workspace analysis is a potential measurement for impairment of the upper extremity in neuralgic amyotrophy.

INTRODUCTION/AIMS: Neuralgic amyotrophy (NA) is a multifocal neuropathy involving the nerves of the upper extremity, limiting functional capability and reducing range of motion. The reachable workspace (RWS) is a computerized three-dimensinal analysis system that evaluates the relative surface area (RSA) of an individual's arm reachability and has shown utility in several neuromuscular disorders. The aims of this study were to examine the ability of the RWS to quantitatively detect limitations in upper extremity active range of motion in patients with NA, and correlate these with other upper extremity functional outcome measures. METHODS: Forty-seven patients with NA and 25 healthy age- and sex-matched controls were measured with the RWS. Study participants' RSAs were correlated with scores on the Shoulder Rating Questionnaire (SRQ), the Disabilities of Arm Shoulder and Hand (DASH) questionnaire, and upper extremity strength measurements using hand-held dynamometry. RESULTS: Patients with NA showed significantly lower values in the affected arm for all quadrants (except for the ipsilateral lower quadrant) and total RSA compared with controls (P < 0.001). We found moderate correlations between the reachable workspace, the DASH questionnaire result (r = -0.415), and serratus anterior muscle strength (r = 0.414). DISCUSSION: RWS is able to detect limitations in active range of motion of the affected arm in patients with NA, and is moderately correlated with upper extremity functional measures. RWS can demonstrate impairment of the affected upper extremity in NA and it has potential as a clinical outcome measure.

Impaired foot placement strategy during walking in people with incomplete spinal cord injury.

BACKGROUND: Impaired balance during walking is a common problem in people with incomplete spinal cord injury (iSCI). To improve walking capacity, it is crucial to characterize balance control and how it is affected in this population. The foot placement strategy, a dominant mechanism to maintain balance in the mediolateral (ML) direction during walking, can be affected in people with iSCI due to impaired sensorimotor control. This study aimed to determine if the ML foot placement strategy is impaired in people with iSCI compared to healthy controls. METHODS: People with iSCI (n = 28) and healthy controls (n = 19) performed a two-minute walk test at a self-paced walking speed on an instrumented treadmill. Healthy controls performed one extra test at a fixed speed set at 50% of their preferred speed. To study the foot placement strategy of a participant, linear regression was used to predict the ML foot placement based on the ML center of mass position and velocity. The accuracy of the foot placement strategy was evaluated by the root mean square error between the predicted and actual foot placements and was referred to as foot placement deviation. Independent t-tests were performed to compare foot placement deviation of people with iSCI versus healthy controls walking at two different walking speeds. RESULTS: Foot placement deviation was significantly higher in people with iSCI compared to healthy controls independent of walking speed. Participants with iSCI walking in the self-paced condition exhibited 0.40 cm (51%) and 0.33 cm (38%) higher foot placement deviation compared to healthy controls walking in the self-paced and the fixed-speed 50% condition, respectively. CONCLUSIONS: Higher foot placement deviation in people with iSCI indicates an impaired ML foot placement strategy in individuals with iSCI compared to healthy controls.

Using Sensor Technology to Measure Gait Capacity and Gait Performance in Rehabilitation Inpatients with Neurological Disorders.

The aim of this study was to objectively assess and compare gait capacity and gait performance in rehabilitation inpatients with stroke or incomplete spinal cord injury (iSCI) using inertial measurement units (IMUs). We investigated how gait capacity (what someone can do) is related to gait performance (what someone does). Twenty-two inpatients (11 strokes, 11 iSCI) wore ankle positioned IMUs during the daytime to assess gait. Participants completed two circuits to assess gait capacity. These were videotaped to certify the validity of the IMU algorithm. Regression analyses were used to investigate if gait capacity was associated with gait performance (i.e., walking activity and spontaneous gait characteristics beyond therapy time). The ankle positioned IMUs validly assessed the number of steps, walking time, gait speed, and stride length (r ≥ 0.81). The walking activity was strongly (r ≥ 0.76) related to capacity-based gait speed. Maximum spontaneous gait speed and stride length were similar to gait capacity. However, the average spontaneous gait speed was half the capacity-based gait speed. Gait capacity can validly be assessed using IMUs and is strongly related to gait performance in rehabilitation inpatients with neurological disorders. Measuring gait performance with IMUs provides valuable additional information about walking activity and spontaneous gait characteristics to inform about functional recovery.

Independent and sensitive gait parameters for objective evaluation in knee and hip osteoarthritis using wearable sensors.

BACKGROUND: Although it is well-established that osteoarthritis (OA) impairs daily-life gait, objective gait assessments are not part of routine clinical evaluation. Wearable inertial sensors provide an easily accessible and fast way to routinely evaluate gait quality in clinical settings. However, during these assessments, more complex and meaningful aspects of daily-life gait, including turning, dual-task performance, and upper body motion, are often overlooked. The aim of this study was therefore to investigate turning, dual-task performance, and upper body motion in individuals with knee or hip OA in addition to more commonly assessed spatiotemporal gait parameters using wearable sensors. METHODS: Gait was compared between individuals with unilateral knee (n = 25) or hip OA (n = 26) scheduled for joint replacement, and healthy controls (n = 27). For 2 min, participants walked back and forth along a 6-m trajectory making 180° turns, with and without a secondary cognitive task. Gait parameters were collected using 4 inertial measurement units on the feet and trunk. To test if dual-task gait, turning, and upper body motion had added value above spatiotemporal parameters, a factor analysis was conducted. Effect sizes were computed as standardized mean difference between OA groups and healthy controls to identify parameters from these gait domains that were sensitive to knee or hip OA. RESULTS: Four independent domains of gait were obtained: speed-spatial, speed-temporal, dual-task cost, and upper body motion. Turning parameters constituted a gait domain together with cadence. From the domains that were obtained, stride length (speed-spatial) and cadence (speed-temporal) had the strongest effect sizes for both knee and hip OA. Upper body motion (lumbar sagittal range of motion), showed a strong effect size when comparing hip OA with healthy controls. Parameters reflecting dual-task cost were not sensitive to knee or hip OA. CONCLUSIONS: Besides more commonly reported spatiotemporal parameters, only upper body motion provided non-redundant and sensitive parameters representing gait adaptations in individuals with hip OA. Turning parameters were sensitive to knee and hip OA, but were not independent from speed-related gait parameters. Dual-task parameters had limited additional value for evaluating gait in knee and hip OA, although dual-task cost constituted a separate gait domain. Future steps should include testing responsiveness of these gait domains to interventions aiming to improve mobility.

Orofacial muscles may be affected in early stages of Becker muscular dystrophy: A preliminary study.

BACKGROUND: Dysphagia is reported in patients with Duchenne or Becker muscular dystrophy. Our clinical experience suggests that, compared with Duchenne patients, impaired mastication and swallowing occur early in Becker patients relative to their skeletal muscle involvement. The aim of this study was to assess dysphagia in Duchenne and Becker patients in relation to ambulatory capacity. METHODS: In patients in the early ambulatory stage, clinical symptoms, quantitative muscle ultrasound of the orofacial muscles, and maximum bite force were assessed. The 6-Minute Walk Test (6MWT) was used to measure ambulatory capacity. RESULTS: Eleven Duchenne and 11 Becker patients were included. Although Becker patients had a greater 6MWT distance than Duchenne patients, the occurrence of mastication and swallowing difficulties was similar. The temporalis muscle was significantly thicker in Becker patients. CONCLUSIONS: Clinicians should be aware of dysphagia in both groups, even when ambulation is still well preserved.

Changes in severity and impact of drooling after submandibular gland botulinum neurotoxin A injections in children with neurodevelopmental disabilities.

AIMS: To examine changes in objective and subjective drooling severity measures after submandibular botulinum neurotoxin A injection in children with neurodevelopmental disabilities, explore their relationship, and evaluate if clinically relevant responses relate to changes in the impact of drooling. METHOD: This longitudinal, observational cohort study involved 160 children (92 males, 68 females; 3-17y, mean 9y 1mo, SD 3y 6mo) treated between 2000 and 2012 at the Radboud University Medical Center, Nijmegen, the Netherlands. Repeated measures analysis of variance was used to compare the 5-minute Drooling Quotient (DQ5) and Visual Analogue Scale (VAS) for drooling severity pretreatment and posttreatment, and Pearson's rho to assess their association. A parent questionnaire was used to assess drooling impact in responders (defined as ≥50% reduction in DQ5 and/or ≥2 SD reduction in VAS for drooling severity 8wks postintervention) and non-responders. RESULTS: One hundred and twelve children (70%) were responders. Their mean VAS for drooling severity and DQ5 scores were significantly lower 32 weeks postintervention compared to baseline. At baseline, the VAS for drooling severity-DQ5 relationship was 'weak' (rs =0.15, p=0.060), whereas it was 'fair' at 8 weeks (rs =0.43, p=0.000) and 32 weeks (rs =0.30, p=0.000). For responders, a significant change was found regarding the impact of drooling on daily care and social interactions at 8 weeks after intervention; most of these effects were maintained at 32 weeks. INTERPRETATION: A clinically relevant response based on a combination of objective and subjective measures of drooling severity was accompanied by positive changes regarding the impact of drooling on daily care and social interactions. WHAT THIS PAPER ADDS: Botulinum neurotoxin A injection into the submandibular glands reduced drooling severity in 70% of children with neurodevelopmental disabilities. Objective (5-minute Drooling Quotient) and subjective (Visual Analogue Scale for drooling severity) measures correlated 8 and 32 weeks after treatment. Objective and subjective measures complemented each other when changes in drooling severity were assessed. Reduced drooling severity was accompanied by positive changes with regard to the impact of drooling.

Interventions to improve upper limb function for children with bilateral cerebral palsy: a systematic review.

AIM: To systematically review the efficacy of interventions on upper limb function in children 0 to 19 years of age with bilateral cerebral palsy on the basis of outcome measures of upper limb function and measures of activities and/or participation according to the International Classification of Functioning, Disability and Health. METHOD: Cochrane, PubMed, Embase, CINAHL, and Web of Science were searched from inception to September 2017. Methodological quality and strength of evidence were analysed by two independent raters using Sackett's level of evidence and the American Academy for Cerebral Palsy and Developmental Medicine (AACPDM) guidelines. RESULTS: Fifteen studies with a large variety of interventions and heterogeneity in outcome measures met the inclusion criteria. Twelve studies provided level IV evidence according to AACPDM guidelines. For three small randomized controlled trials the level of evidence was II. Only one of these trials showed strong methodological quality: a study on hand-arm bimanual intensive therapy including lower extremities. INTERPRETATION: We identified a large variety of interventions, heterogeneity in outcome measures, and generally weak to moderate methodological quality for most studies. We recommend further research specifically aimed at bimanual-intensive, goal-directed, and task-specific training programmes for the upper limb in children with bilateral cerebral palsy, using either high-quality (multicentre) trials or well-designed single-case trials. WHAT THIS PAPER ADDS: There is a large variety of interventions on upper limb function in children with bilateral cerebral palsy. Heterogeneity of outcome measures and interventions impeded firm conclusions about intervention efficacy. Most studies had low-level evidence and weak to moderate methodological quality. The strongest evidence from a small randomized controlled trial was for hand-arm bimanual intensive therapy including lower extremities.

Intervenciones para mejorar la función de las extremidades superiores en niños con parálisis cerebral bilateral: una revisión sistemática OBJETIVO: Revisar sistemáticamente la eficacia de la función de la extremidad superior con las intervenciones realizadas en niños de 0 a 19 años de edad con parálisis cerebral bilateral basada en medidas de la función de la extremidad superior, de actividades y / o participación, según la Clasificación Internacional del Funcionamiento, de la Discapacidad y de la Salud. MÉTODO: Se investigaron desde su inicio hasta septiembre del 2017 las siguientes bases de datos: Cochrane, PubMed, Embase, CINAHL y Web of Science. Tres evaluadores independientes analizaron la calidad metodológica y la calidad de la evidencia utilizando el nivel de evidencia de Sackett y las guias de la Academia Americana para la Parálisis Cerebral y Medicina del Desarrollo (AACPDM). RESULTADOS: Quince estudios con una gran variedad de intervenciones y heterogeneidad en las escalas de resultado cumplieron con los criterios de inclusión. Doce estudios proporcionaron evidencia nivel IV de acuerdo con las guías de la AACPDM. Otros tres ensayos pequeños controlados y aleatorios se clasificaron como nivel II de evidencia. Solo uno de estos ensayos mostró una calidad metodológica sólida que consista en un estudio sobre terapia bimanual intensiva mano-brazo que incluía extremidades inferiores. INTERPRETACIÓN: Se identificaron una gran variedad de intervenciones, heterogeneidad en las escalas de medición de los resultados, y en general una calidad metodológica de débil a moderada para la mayoría de los estudios. Recomendamos investigaciones adicionales dirigidas específicamente a programas de entrenamiento bimanual, orientado a objetivos específicos para la tarea del miembro superior en niños con parálisis cerebral bilateral, utilizando ensayos de alta calidad (multicéntricos) o ensayos dirigidos a estudiar solo un concepto bien diseñados.

Intervenções para melhorar a função do membro superior em crianças com paralisia cerebral bilateral: uma revisão sistemática OBJETIVO: Revisar sistematicamente a eficácia de intervenções para a função do membro superior em crianças de 0 a 19 anos de idade com paralisia cerebral bilateral com base em medidas de resultado da função do membro superior e medidas de atividades e/ou participação de acordo com a Classificação Internacional de Funcionalidade, Incapacidade e Saúde. MÉTODO: Cochrane, PubMed, Embase, CINAHL, e Web of Science foram pesquisadas do início até setembro de 2017. A qualidade metodológica e força da evidência foram analisados por três avaliadores independentes usando o nível Sackett's e evidência e as diretrizes da Academia Americana de Paralisia Cerebral e Medicina do Desenvolviemnto (AACPDM). RESULTADOS: Quinze estudos com uma grande variedade de intervenções e heterogeneidade de medidas de resultado atenderam aos critérios de inclusão. Doze estudos forneceram evidência nível IV de acordo com as diretrizes da AACPDM. Para três pequenos estudos randomizados controlados o nível de evidência foi II. Apenas um destes estudos mostrou forte qualidade metodológica: um estudo sobre terapia intensive bimanual mão-braço incluindo as extremidades inferiores. INTERPRETAÇÃO: Identificamos uma grande variedade de intervenções, heterogeneidade em medidas de resultado, e em geral qualidade metodológica de fraca a moderada para a maioria dos estudos. Recomendamos mais pesquisas especificamente voltadas para programas de treinamento intensivos bimanuais, direcionados a objetivos e específicos para tarefas para o membro superior de crianças com paralisia cerebral usando ou estudos de alta qualidade (multicêntricos) ou estudos de sujeito único bem desenhados.

Strength training and aerobic exercise training for muscle disease.

BACKGROUND: Strength training or aerobic exercise programmes, or both, might optimise muscle and cardiorespiratory function and prevent additional disuse atrophy and deconditioning in people with a muscle disease. This is an update of a review first published in 2004 and last updated in 2013. We undertook an update to incorporate new evidence in this active area of research. OBJECTIVES: To assess the effects (benefits and harms) of strength training and aerobic exercise training in people with a muscle disease. SEARCH METHODS: We searched Cochrane Neuromuscular's Specialised Register, CENTRAL, MEDLINE, Embase, and CINAHL in November 2018 and clinical trials registries in December 2018. SELECTION CRITERIA: Randomised controlled trials (RCTs), quasi-RCTs or cross-over RCTs comparing strength or aerobic exercise training, or both lasting at least six weeks, to no training in people with a well-described muscle disease diagnosis. DATA COLLECTION AND ANALYSIS: We used standard methodological procedures expected by Cochrane. MAIN RESULTS: We included 14 trials of aerobic exercise, strength training, or both, with an exercise duration of eight to 52 weeks, which included 428 participants with facioscapulohumeral muscular dystrophy (FSHD), dermatomyositis, polymyositis, mitochondrial myopathy, Duchenne muscular dystrophy (DMD), or myotonic dystrophy. Risk of bias was variable, as blinding of participants was not possible, some trials did not blind outcome assessors, and some did not use an intention-to-treat analysis. Strength training compared to no training (3 trials) For participants with FSHD (35 participants), there was low-certainty evidence of little or no effect on dynamic strength of elbow flexors (MD 1.2 kgF, 95% CI -0.2 to 2.6), on isometric strength of elbow flexors (MD 0.5 kgF, 95% CI -0.7 to 1.8), and ankle dorsiflexors (MD 0.4 kgF, 95% CI -2.4 to 3.2), and on dynamic strength of ankle dorsiflexors (MD -0.4 kgF, 95% CI -2.3 to 1.4). For participants with myotonic dystrophy type 1 (35 participants), there was very low-certainty evidence of a slight improvement in isometric wrist extensor strength (MD 8.0 N, 95% CI 0.7 to 15.3) and of little or no effect on hand grip force (MD 6.0 N, 95% CI -6.7 to 18.7), pinch grip force (MD 1.0 N, 95% CI -3.3 to 5.3) and isometric wrist flexor force (MD 7.0 N, 95% CI -3.4 to 17.4). Aerobic exercise training compared to no training (5 trials) For participants with DMD there was very low-certainty evidence regarding the number of leg revolutions (MD 14.0, 95% CI -89.0 to 117.0; 23 participants) or arm revolutions (MD 34.8, 95% CI -68.2 to 137.8; 23 participants), during an assisted six-minute cycle test, and very low-certainty evidence regarding muscle strength (MD 1.7, 95% CI -1.9 to 5.3; 15 participants). For participants with FSHD, there was low-certainty evidence of improvement in aerobic capacity (MD 1.1 L/min, 95% CI 0.4 to 1.8, 38 participants) and of little or no effect on knee extension strength (MD 0.1 kg, 95% CI -0.7 to 0.9, 52 participants). For participants with dermatomyositis and polymyositis (14 participants), there was very low-certainty evidence regarding aerobic capacity (MD 14.6, 95% CI -1.0 to 30.2). Combined aerobic exercise and strength training compared to no training (6 trials) For participants with juvenile dermatomyositis (26 participants) there was low-certainty evidence of an improvement in knee extensor strength on the right (MD 36.0 N, 95% CI 25.0 to 47.1) and left (MD 17 N 95% CI 0.5 to 33.5), but low-certainty evidence of little or no effect on maximum force of hip flexors on the right (MD -9.0 N, 95% CI -22.4 to 4.4) or left (MD 6.0 N, 95% CI -6.6 to 18.6). This trial also provided low-certainty evidence of a slight decrease of aerobic capacity (MD -1.2 min, 95% CI -1.6 to 0.9). For participants with dermatomyositis and polymyositis (21 participants), we found very low-certainty evidence for slight increases in muscle strength as measured by dynamic strength of knee extensors on the right (MD 2.5 kg, 95% CI 1.8 to 3.3) and on the left (MD 2.7 kg, 95% CI 2.0 to 3.4) and no clear effect in isometric muscle strength of eight different muscles (MD 1.0, 95% CI -1.1 to 3.1). There was very low-certainty evidence that there may be an increase in aerobic capacity, as measured with time to exhaustion in an incremental cycle test (17.5 min, 95% CI 8.0 to 27.0) and power performed at VO2 max (maximal oxygen uptake) (18 W, 95% CI 15.0 to 21.0). For participants with mitochondrial myopathy (18 participants), we found very low-certainty evidence regarding shoulder muscle (MD -5.0 kg, 95% CI -14.7 to 4.7), pectoralis major muscle (MD 6.4 kg, 95% CI -2.9 to 15.7), and anterior arm muscle strength (MD 7.3 kg, 95% CI -2.9 to 17.5). We found very low-certainty evidence regarding aerobic capacity, as measured with mean time cycled (MD 23.7 min, 95% CI 2.6 to 44.8) and mean distance cycled until exhaustion (MD 9.7 km, 95% CI 1.5 to 17.9). One trial in myotonic dystrophy type 1 (35 participants) did not provide data on muscle strength or aerobic capacity following combined training. In this trial, muscle strength deteriorated in one person and one person had worse daytime sleepiness (very low-certainty evidence). For participants with FSHD (16 participants), we found very low-certainty evidence regarding muscle strength, aerobic capacity and VO2 peak; the results were very imprecise. Most trials reported no adverse events other than muscle soreness or joint complaints (low- to very low-certainty evidence). AUTHORS' CONCLUSIONS: The evidence regarding strength training and aerobic exercise interventions remains uncertain. Evidence suggests that strength training alone may have little or no effect, and that aerobic exercise training alone may lead to a possible improvement in aerobic capacity, but only for participants with FSHD. For combined aerobic exercise and strength training, there may be slight increases in muscle strength and aerobic capacity for people with dermatomyositis and polymyositis, and a slight decrease in aerobic capacity and increase in muscle strength for people with juvenile dermatomyositis. More research with robust methodology and greater numbers of participants is still required.

Interventions for preventing falls in people after stroke.

BACKGROUND: Falls are one of the most common complications after stroke, with a reported incidence ranging between 7% in the first week and 73% in the first year post stroke. This is an updated version of the original Cochrane Review published in 2013. OBJECTIVES: To evaluate the effectiveness of interventions aimed at preventing falls in people after stroke. Our primary objective was to determine the effect of interventions on the rate of falls (number of falls per person-year) and the number of fallers. Our secondary objectives were to determine the effects of interventions aimed at preventing falls on 1) the number of fall-related fractures; 2) the number of fall-related hospital admissions; 3) near-fall events; 4) economic evaluation; 5) quality of life; and 6) adverse effects of the interventions. SEARCH METHODS: We searched the trials registers of the Cochrane Stroke Group (September 2018) and the Cochrane Bone, Joint and Muscle Trauma Group (October 2018); the Cochrane Central Register of Controlled Trials (CENTRAL; 2018, Issue 9) in the Cochrane Library; MEDLINE (1950 to September 2018); Embase (1980 to September 2018); CINAHL (1982 to September 2018); PsycINFO (1806 to August 2018); AMED (1985 to December 2017); and PEDro (September 2018). We also searched trials registers and checked reference lists. SELECTION CRITERIA: Randomised controlled trials of interventions where the primary or secondary aim was to prevent falls in people after stroke. DATA COLLECTION AND ANALYSIS: Two review authors (SD and WS) independently selected studies for inclusion, assessed trial quality and risk of bias, and extracted data. We resolved disagreements through discussion, and contacted study authors for additional information where required. We used a rate ratio and 95% confidence interval (CI) to compare the rate of falls (e.g. falls per person-year) between intervention and control groups. For risk of falling we used a risk ratio and 95% CI based on the number of people falling (fallers) in each group. We pooled results where appropriate and applied GRADE to assess the quality of the evidence. MAIN RESULTS: We included 14 studies (of which six have been published since the first version of this review in 2013), with a total of 1358 participants. We found studies that investigated exercises, predischarge home visits for hospitalised patients, the provision of single lens distance vision glasses instead of multifocal glasses, a servo-assistive rollator and non-invasive brain stimulation for preventing falls.Exercise compared to control for preventing falls in people after strokeThe pooled result of eight studies showed that exercise may reduce the rate of falls but we are uncertain about this result (rate ratio 0.72, 95% CI 0.54 to 0.94, 765 participants, low-quality evidence). Sensitivity analysis for single exercise interventions, omitting studies using multiple/multifactorial interventions, also found that exercise may reduce the rate of falls (rate ratio 0.66, 95% CI 0.50 to 0.87, 626 participants). Sensitivity analysis for the effect in the chronic phase post stroke resulted in little or no difference in rate of falls (rate ratio 0.58, 95% CI 0.31 to 1.12, 205 participants). A sensitivity analysis including only studies with low risk of bias found little or no difference in rate of falls (rate ratio 0.88, 95% CI 0.65 to 1.20, 462 participants). Methodological limitations mean that we have very low confidence in the results of these sensitivity analyses.For the outcome of number of fallers, we are very uncertain of the effect of exercises compared to the control condition, based on the pooled result of 10 studies (risk ratio 1.03, 95% CI 0.90 to 1.19, 969 participants, very low quality evidence). The same sensitivity analyses as described above gives us very low certainty that there are little or no differences in number of fallers (single interventions: risk ratio 1.09, 95% CI 0.93 to 1.28, 796 participants; chronic phase post stroke: risk ratio 0.94, 95% CI 0.73 to 1.22, 375 participants; low risk of bias studies: risk ratio 0.96, 95% CI 0.77 to 1.21, 462 participants).Other interventions for preventing falls in people after strokeWe are very uncertain whether interventions other than exercise reduce the rate of falls or number of fallers. We identified very low certainty evidence when investigating the effect of predischarge home visits (rate ratio 0.85, 95% CI 0.43 to 1.69; risk ratio 1.48, 95% CI 0.71 to 3.09; 85 participants), provision of single lens distance glasses to regular wearers of multifocal glasses (rate ratio 1.08, 95% CI 0.52 to 2.25; risk ratio 0.74, 95% CI 0.47 to 1.18; 46 participants) and a servo-assistive rollator (rate ratio 0.44, 95% CI 0.16 to 1.21; risk ratio 0.44, 95% CI 0.16 to 1.22; 42 participants).Finally, transcranial direct current stimulation (tDCS) was used in one study to examine the effect on falls post stroke. We have low certainty that active tDCS may reduce the number of fallers compared to sham tDCS (risk ratio 0.30, 95% CI 0.14 to 0.63; 60 participants). AUTHORS' CONCLUSIONS: At present there exists very little evidence about interventions other than exercises to reduce falling post stroke. Low to very low quality evidence exists that this population benefits from exercises to prevent falls, but not to reduce number of fallers.Fall research does not in general or consistently follow methodological gold standards, especially with regard to fall definition and time post stroke. More well-reported, adequately-powered research should further establish the value of exercises in reducing falling, in particular per phase, post stroke.

Validity and reproducibility of the Functional Gait Assessment in persons after stroke.

OBJECTIVE:: To evaluate construct validity and reproducibility of the Functional Gait Assessment (FGA) for measuring walking balance capacity in persons after stroke. DESIGN:: Cross-sectional study. SETTING:: Inpatient and outpatient rehabilitation center. SUBJECTS:: Fifty-two persons post-stroke (median (25% and 75% percentiles)) time post-stroke 6 (5-10) weeks) with independent walking ability (mean gait speed 1.1 ± .4 m/s). METHODS:: Subjects completed a standardized FGA twice within one to eight days by the same investigator. Validity was evaluated by testing hypotheses on the association with two timed walking tests, Berg Balance Scale, and the mobility domain of the Stroke Impact Scale using correlation coefficients ( r), and with Functional Ambulation Categories using the Kruskal-Wallis test. Reproducibility of FGA scores was assessed with intraclass correlation coefficient and standard error of measurement. RESULTS:: Subjects scored a median of 22 out of 30 points at the first FGA. Moderate to high significant correlations ( r .61-.83) and significant differences in FGA median scores between the Functional Ambulation Categories were found. Eight hypotheses (80%) could be confirmed. Inter-rater, intra-rater, and test-retest reliability of the total scores were excellent. The standard error of measurement and minimal detectable change were 2 and 6 points, respectively. No relevant ceiling effect was observed. CONCLUSION:: The FGA demonstrated good measurement properties in persons after stroke and yielded no ceiling effect in contrast to other capacity measures. In clinical practice, a measurement error of 6 points should be taken into account in interpreting changes in walking balance.

Caregivers' effects of augmented cognitive-behavioural therapy for post-stroke depressive symptoms in patients: secondary analyses to a randomized controlled trial.

OBJECTIVE: To investigate the concomitant effects of two patient-directed interventions for post-stroke depressive symptoms on caregivers' well-being. DESIGN: Secondary analyses of the results of a randomized controlled trial. SUBJECTS: Fifty caregivers of stroke patients receiving outpatient rehabilitation. INTERVENTIONS: Stroke patients and their caregivers were randomly allocated to either cognitive-behavioural therapy augmented with movement or occupational therapy ( n = 23) or computerized cognitive training ( n = 27) to alleviate depressive symptoms in patients. MAIN MEASURES: Emotional burden (Involvement Evaluation Questionnaire), practical burden (Caregiver Strain Index), mental health (General Health Questionnaire) and emotional complaints (Hospital Anxiety and Depression Scale). RESULTS: Caregivers of patients who received cognitive-behavioural therapy reported significantly higher mental health levels (mean difference (MD) = 1.78, 95% confidence interval (CI) = 0.43-3.13, P = 0.01) and less worrying about patients' well-being (MD = 1.9, 95% CI = 0.56-3.24, P < 0.01). In addition, there were positive time effects on the Involvement Evaluation Questionnaire for Brain Injury, particularly the subscales Worrying, Supervision and Tension. CONCLUSION: The results suggest that augmented cognitive-behavioural therapy aimed at improving patients' emotional, behavioural and social functioning positively affects some aspects of caregivers' well-being.

Validity and reliability of serratus anterior hand held dynamometry.

BACKGROUND: Strength testing of the serratus anterior muscle with hand held dynamometry (HDD) in supine subjects has low reproducibility, and is influenced by compensatory activity of other muscles like the pectoralis major and upper trapezius. Previously, two manual maximum voluntary isometric contraction tests of the serratus anterior muscle were reported that recruited optimal surface electromyography (sEMG) activity in a sitting position. We adapted three manual muscle tests to make them suitable for HHD and investigated their validity and reliability. METHODS: Twenty-one healthy adults were examined by two assessors in one supine and two seated positions. Each test was repeated twice. Construct validity was determined by evaluating force production (assessed with HHD) in relation to sEMG of the serratus anterior, upper trapezius and pectoralis major muscles, comparing the three test positions. Intra- and interrater reliability were determined by calculating intra-class correlation coefficients (ICC) smallest detectable change (SDC) and standard error of measurement (SEM). RESULTS: Serratus anterior muscle sEMG activity was most isolated in a seated position with the humerus in 90° anteflexion in the scapular plane. This resulted in the lowest measured force levels in this position with a mean force of 296 N (SEM 15.8 N). Intrarater reliability yielded an ICC of 0.658 (95% CI 0.325; 0.846) and an interrater reliability of 0.277 (95% CI -0.089;0.605). SDC was 127 Newton, SEM 45.8 Newton. CONCLUSION: The results indicate that validity for strength testing of the serratus anterior muscle is optimal with subjects in a seated position and the shoulder flexed at 90° in the scapular plane. Intrarater reliability is moderate and interrater reliability of this procedure is poor. However the high SDC values make it difficult to use the measurement in repeated measurements.

Correction to: Validity and reliability of serratus anterior hand held dynamometry.

Following publication of the original article [1], the authors reported that the headers in Table 2 in their paper were omitted.

Long-term use of implanted peroneal functional electrical stimulation for stroke-affected gait: the effects on muscle and motor nerve.

BACKGROUND: Peripheral changes to muscle and motor nerves occur following stroke, which may further impair functional capacity. We investigated whether a year-long use of an implanted peroneal FES system reverses stroke-related changes in muscles and motor nerves in people with foot drop in the chronic phase after supratentorial stroke. METHODS: Thirteen persons with a chronic stroke (mean age 56.1 years, median Fugl-Meyer Assessment leg score 71%) were included and received an implanted peroneal FES system (ActiGait®). Quantitative muscle ultrasound (QMUS) images were obtained bilaterally from three leg muscles (i.e. tibialis anterior, rectus femoris, gastrocnemius). Echogenicity (muscle ultrasound gray value) and muscle thickness were assessed over a one-year follow-up and compared to age-, sex-, height- and weight-corrected reference values. Compound motor action potentials (CMAPs) and motor evoked potentials (MEPs) were obtained from the tibialis anterior muscle. Generalized estimated equation modeling was used to assess changes in QMUS, CMAPs and MEPs outcomes over the follow-up period. RESULTS: Echogenicity of the tibialis anterior decreased significantly during the follow-up on the paretic side. Z-scores changed from 0.88 at baseline to - 0.15 after 52 weeks. This was accompanied by a significant increase in muscle thickness on the paretic side, where z-scores changed from - 0.32 at baseline to 0.48 after 52 weeks. Echogenicity of the rectus femoris normalized on both the paretic and non-paretic side (z-scores changed from - 1.09 and - 1.51 to 0.14 and - 0.49, respectively). Amplitudes of CMAP and MEP (normalized to CMAP) were reduced during follow-up, particularly on the paretic side (ΔCMAP = 20% and ΔMEP = 14%). CONCLUSIONS: We show that the structural changes to muscles following stroke are reversible with FES and that these changes might not be limited to electrically stimulated muscles. No evidence for improvement of the motor nerves was found.

Offline effects of transcranial direct current stimulation on reaction times of lower extremity movements in people after stroke: a pilot cross-over study.

BACKGROUND: Transcranial direct current stimulation (tDCS) is a non-invasive brain stimulation technique that has shown promise for rehabilitation after stroke. Ipsilesional anodal tDCS (a-tDCS) over the motor cortex increases corticospinal excitability, while contralesional cathodal tDCS (c-tDCS) restores interhemispheric balance, both resulting in offline improved reaction times of delayed voluntary upper-extremity movements. We aimed to investigate whether tDCS would also have a beneficial effect on delayed leg motor responses after stroke. In addition, we identified whether variability in tDCS effects was associated with the level of leg motor function. METHODS: In a cross-over design, 13 people with chronic stroke completed three 15-min sessions of anodal, cathodal and sham stimulation over the primary motor cortex on separate days in an order balanced across participants. Directly after stimulation, participants performed a comprehensive set of lower-extremity tasks involving the paretic tibialis anterior (TA): voluntary ankle-dorsiflexion, gait initiation, and backward balance perturbation. For all tasks, TA onset latencies were determined. In addition, leg motor function was determined by the Fugl-Meyer Assessment - leg score (FMA-L). Repeated measures ANOVA was used to reveal tDCS effects on reaction times. Pearson correlation coefficients were used to establish the relation between tDCS effects and leg motor function. RESULTS: For all tasks, TA reaction times did not differ across tDCS sessions. For gait initiation and backward balance perturbation, differences between sham and active stimulation (a-tDCS or c-tDCS) did not correlate with leg motor function. Yet, for ankle dorsiflexion, individual reaction time differences between c-tDCS and sham were strongly associated with FMA-L, with more severely impaired patients exhibiting slower paretic reaction times following c-tDCS. CONCLUSION: We found no evidence for offline tDCS-induced benefits. Interestingly, we found that c-tDCS may have unfavorable effects on voluntary control of the paretic leg in severely impaired patients with chronic stroke. This finding points at potential vicarious control from the unaffected hemisphere to the paretic leg. The absence of tDCS-induced effects on gait and balance, two functionally relevant tasks, shows that such motor behavior is inadequately stimulated by currently used tDCS applications. TRIAL REGISTRATION: The study is registered in the Netherlands Trial Register (NL5684; April 13th, 2016).

Tarsal fusion for pes equinovarus deformity improves gait capacity in chronic stroke patients.

BACKGROUND: Gait impairments are common and disabling in chronic stroke patients. Pes equinovarus deformity is one of the primary motor deficits underlying reduced gait capacity after stroke. It predisposes to stance-phase instability and subsequent ankle sprain or falls. This instability is most pronounced when walking barefoot. Tarsal fusion is a recommended treatment option for varus deformity, but scientific evidence is sparse. We therefore evaluated whether a tarsal fusion improved barefoot walking capacity in chronic stroke patients with pes equinovarus deformity. METHODS: Ten patients with a pes equinovarus deformity secondary to supratentorial stroke underwent surgical correction involving a tarsal fusion of one or more joints. Instrumented gait analysis was performed pre- and postoperatively using a repeated-measures design. Primary outcome measure was gait speed. RESULTS: Walking speed significantly improved by 32% after surgery (0.38 m/s ± 0.20 to 0.50 m/s ± 0.17, p = 0.007). Significant improvement was also observed when looking at cadence (p = 0.028), stride length (p = 0.016), and paretic step length (p = 0.005). Step length on the nonparetic side did not change. Peak ankle moment increased significantly on the nonparetic side (p = 0.021), but not on the paretic side (p = 0.580). In addition, functional ambulation scores increased significantly (p = 0.008), as did satisfaction with gait performance (p = 0.017). CONCLUSIONS: Tarsal fusion for equinovarus deformity in chronic stroke patients improves gait capacity, and the degree of improvement is of clinical relevance. Our results suggest that the improved gait capacity may be related to better prepositioning and loading of the paretic foot, leading to larger paretic step length and nonparetic ankle kinetics.

Hand-Use-at-Home Questionnaire: validity and reliability in children with neonatal brachial plexus palsy or unilateral cerebral palsy.

OBJECTIVE: To investigate construct validity and test-retest reliability of the parent-rated Hand-Use-at-Home questionnaire (HUH) in children with neonatal brachial plexus palsy or unilateral cerebral palsy. DESIGN AND SUBJECTS: For this cross-sectional study, children with neonatal brachial plexus palsy or unilateral cerebral palsy, aged 3-10 years, were eligible. MAIN MEASURES: The HUH, Pediatric Outcome Data Collection Instrument Upper Extremity Scale (neonatal brachial plexus palsy only), and Children's Hand-Use Experience Questionnaire (unilateral cerebral palsy only) were completed. The HUH was completed twice in subgroups of both diagnoses. Lesion-extent (indication of involved nerve rootlets in neonatal brachial plexus palsy as confirmed during clinical observation and/or nerve surgery) and Manual Ability Classification System levels (unilateral cerebral palsy) were obtained from the medical records. Spearman correlation coefficients between the HUH and all clinical variables, agreement, standard error of measurement, smallest detectable change and intra-class correlation were calculated. RESULTS: A total of 260 patients participated (neonatal brachial plexus palsy: 181), of which 56 completed the second HUH (neonatal brachial plexus palsy: 16). Median age was 6.9 years for children with neonatal brachial plexus palsy, 116 had C5-C6 lesions. Median age for children with unilateral cerebral palsy was 6.4 years, 33 had Manual Ability Classification System Level II. The HUH correlated moderately with lesion-extent ( rs =-0.5), Pediatric Outcome Data Collection Instrument Upper Extremity Scale ( rs = 0.6) and Children's Hand-Use Experience Questionnaire ( rs = 0.5) but weakly with Manual Ability Classification System levels ( rs = -0.4). Test-retest reliability was excellent (intra-class correlation2,1 = 0.89, standard error of measurement = 0.599 and smallest detectable change = 1.66 logits) and agreement was good (mean difference HUH1 - HUH2 = 0.06 logits). CONCLUSION: The HUH showed good construct validity and test-retest reliability in children with neonatal brachial plexus palsy or unilateral cerebral palsy.