Physical strain of walking in people with neuromuscular diseases is high and relates to step activity in daily life.

OBJECTIVE: To determine the physical strain of walking and assess its relationship with daily steps and intensity of daily activity in people with neuromuscular diseases. DESIGN: Cross-sectional study. SUBJECTS/PATIENTS: Sixty-one adults with neuromuscular diseases. METHODS: Physical strain of walking, defined as oxygen consumption during comfortable walking relative to peak oxygen uptake. Daily step count and daily time spent in moderate and vigorous physical activity were assessed using accelerometry and heart rate measurements, respectively. Regression analyses assessed the relationships between log daily step count and log daily time spent in moderate and vigorous physical activity, and physical strain of walking. RESULTS: The mean (standard deviation) physical strain of walking was 73 (20)% Log daily step count and physical strain were negatively associated (ß = -0.47). No association was found with log daily time spent in moderate and vigorous physical activity. CONCLUSIONS: The highly increased physical strain of comfortable walking indicates that walking is very demanding for people with neuromuscular diseases and is associated with a reduction in daily step activity. The absence of a relationship between intensity of activities and physical strain indicates that, despite a reduction in daily step activity, strenuous daily activities may still be performed.

ExoBand, A Passive Wearable Device as a Walking Aid in Neuromuscular Patients: First Quantitative Assessment.

Objective: Exoband (by Moveo, Padova, Italy) functions as a walking brace, comprising a belt and two leg loops connected by a mechanism that stores energy during the initial phase of the gait cycle and releases it in the subsequent phase. This enhances hip flexor thrust, leading to functional improvement in walking for individuals with conditions characterized by proximal weakness. It has been approved as a passive wearable device for individuals with impaired walking abilities. Objective of this study was to establish a protocol to assess the use of Exoband in patients with various neuromuscular disorders. Methods: This exploratory retrospective study includes consecutive patients diagnosed with neuromuscular disorders (CIDP, motor polyneuropathy, MND), exhibiting a proximal involvement and gait abnormalities. The evaluation protocol incorporated specific walking-related outcome measures, the 10-meter walk test (10mWT), Time-up-and-go test (TUG), and 2-minute walking test (2MWT). The assessments were conducted both with and without the Exoband under standard conditions. Results: Eight patients (6 males, aged 60-78 years) were tested. An increase in velocity was observed in the 10mWT (median 13.4 sec, IQR 12.0-15.7 vs. 12.2 sec, IQR 11.3-14.2 seconds, p < 0.05) and the TUG (14.0 sec, IQR 13-16.2 vs 13.35 sec, IQR 11-13.8; p < 0.05, by non-parametric Wilcoxon test), and a trend of increase in 2MWT (median 88.2 vs 92.6 m, n.s.). Six out of 8 patients reported subjective benefits from the very first use, including improved walking stability, speed, confidence, and reduced fatigue. Conclusions: Our protocol provides a quantitative assessment of Exoband usefulness for patients affected by neuropathies with gait abnormalities. Further investigations are warranted to assess the long-term effects of its regular Exoband use, its efficacy in specific neuromuscular diseases, and its potential role as a rehabilitation device.

Effect of stiffness-optimized ankle foot orthoses on joint work in adults with neuromuscular diseases is related to severity of push-off deficits.

BACKGROUND: People with plantar flexor weakness generate less ankle push-off work during walking, resulting in inefficient proximal joint compensations. To increase push-off work, spring-like ankle foot orthoses (AFOs) can be provided. However, whether and in which patients AFOs increase push-off work and reduce compensatory hip and knee work is unknown. METHODS: In 18 people with bilateral plantar flexor weakness, we performed a 3D gait analysis at comfortable walking speed with shoes-only and with AFOs of which the stiffness was optimized. To account for walking speed differences between conditions, we compared relative joint work of the hip, knee and ankle joint. The relationships between relative work generated with shoes-only and changes in joint work with AFO were tested with Pearson correlations. RESULTS: No differences in relative ankle, knee and hip work over the gait cycle were found between shoes-only and AFO (p>0.499). Percentage of total ankle work generated during pre-swing increased with the AFO (AFO: 85.3±9.1% vs Shoes: 72.4±27.1%, p=0.026). At the hip, the AFO reduced relative work in pre-swing (AFO: 31.9±7.4% vs Shoes: 34.1±10.4%, p=0.038) and increased in loading response (AFO: 18.0±11.0% vs Shoes: 11.9±9.8%, p=0.022). Ankle work with shoes-only was inversely correlated with an increase in ankle work with AFO (r=-0.839, p<0.001) and this increase correlated with reduction in hip work with AFO (r=-0.650, p=0.004). DISCUSSION: Although stiffness-optimized AFOs did not alter the work distribution across the ankle, knee and hip joint compared to shoes-only walking, relative more ankle work was generated during push-off, causing a shift in hip work from pre-swing to loading response. Furthermore, larger ankle push-off deficits when walking with shoes-only were related with an increase in ankle work with AFO and reduction in compensatory hip work, indicating that more severely affected individuals benefit more from the energy storing-and-releasing capacity of AFOs.

Qualitative feedback from caregivers in a multidisciplinary pediatric neuromuscular clinic.

OBJECTIVE: This study explored family satisfaction and perceived quality of care in a pediatric neuromuscular care clinic to assess the value of the multidisciplinary clinic (MDC) model in delivering coordinated care to children with neuromuscular disorders, such as cerebral palsy. METHODS: Caregivers of 22 patients were administered a qualitative survey assessing their perceptions of clinic efficiency, care coordination, and communication. Surveys were audio-recorded and transcribed. Thematic analysis was completed using both deductive and inductive methods. RESULTS: All caregivers reported that providers adequately communicated next steps in the patient's care, and most reported high confidence in caring for the patient as a result of the clinic. Four major themes were identified from thematic analysis: Care Delivery, Communication, Care Quality, and Family-Centeredness. Caregivers emphasized that the MDC model promoted access to care, enhanced efficiency, promoted provider teamwork, and encouraged shared care planning. Caregivers also valued a physical environment that was suitable for patients with complex needs. CONCLUSION: This study demonstrated that caregivers believed the MDC model was both efficient and convenient for pediatric patients with neuromuscular disorders. This model has the potential to streamline medical care and can be applied more broadly to improve care coordination for children with medical complexity.

Ageing with neuromuscular disease: getting lost in transitions.

PURPOSE: To explore the lived experiences of people ageing with neuromuscular disease (NMD). INTRODUCTION: NMD refers to several chronic types of hereditary and progressive NMDs. Owing to advances in rehabilitation and treatment, life expectancy has increased for some subtypes, resulting in life continuing into adulthood and even old age; however, knowledge of people's lived experiences with NMD is sparse. METHODS: A qualitative study using a phenomenological-hermeneutic approach inspired by Ricoeur was conducted. Fifteen persons with NMD were interviewed in 2018. The Consolidated Criteria for Reporting Qualitative Research (COREQ) checklist was used. RESULTS: Four themes were identified: "A time-framed paradox of striving for independent dependency arises as age increases", "Ageing means entering no man's land", "Exercising is caught between shrinking surplus of physical energy and demands of everyday life" and "Ending work life is a jumble of relief, concern and altered self-perception". CONCLUSION: The pathway to old age with NMD encompasses several transitions, all potentially including the risk of getting lost. Physical changes, changes in legislation, experiences of uncertainty regarding where one socially belongs and how to balance reduced physical strength in everyday life are indicated as key areas that affect ageing life with NMD.Implications for rehabilitationRehabilitation professionals should address ageing with NMD from a life course perspective and not with a singled minded focus on chronological age.A biopsychosocial focus is needed to prevent gaps and pave the pathway to old age with NMD.The experiences of multiple transitions when ageing with NMD should be in focus.

Care for capabilities: Implementing the capability approach in rehabilitation of patients with neuromuscular diseases. Study protocol of the controlled before-after ReCap-NMD study.

BACKGROUND: High quality care of patients with neuromuscular diseases requires a personalised approach that focuses on achieving and maintaining a level of functioning that enables them to be in a state of well-being. The capability approach states that well-being should be understood in terms of capabilities, the substantial opportunities that people have to be and do things they have reasons to value. In this Rehabilitation and Capability care for patients with Neuromuscular diseases (ReCap-NMD) study, we want to investigate whether providing care based on the capability approach (capability care) has an added value in the rehabilitation of patients with neuromuscular diseases (NMD). METHODS: Two groups of 30 adult patients with facioscapulohumeral muscular dystrophy or myotonic dystrophy type 1 will be included. The first group will receive rehabilitation care as usual with a follow-up period of 6 months. Then, based on theory, and experiences of patients and healthcare professionals, capability care will be developed. During the following 3 months, the multidisciplinary outpatient rehabilitation care team will be trained in providing this newly developed capability care. Subsequently, the second group will receive capability care, with a follow-up period of 6 months. A mixed methods approach is used with both qualitative and quantitative outcome measures to evaluate the effect of capability care and to perform a process evaluation. The primary outcome measure will be the Canadian Occupational Performance Measure. DISCUSSION: The ReCap-NMD study is the first study to design and implement a healthcare intervention based on the capability approach. The results of this study will expand our knowledge on how the capability approach can be applied in delivering and evaluating healthcare, and will show whether implementing such an intervention leads to a higher well-being for patients with NMD. TRIAL REGISTRATION: Registered at Trialregister.nl (Trial NL8946) on 12th of October, 2020.

Interventions for promoting physical activity in people with neuromuscular disease.

BACKGROUND: The World Health Organization (WHO) recommends that people of all ages take regular and adequate physical activity. If unable to meet the recommendations due to health conditions, international guidance advises being as physically active as possible. Evidence from community interventions of physical activity indicate that people living with medical conditions are sometimes excluded from participation in studies. In this review, we considered the effects of activity-promoting interventions on physical activity and well-being in studies, as well as any adverse events experienced by participants living with inherited or acquired neuromuscular diseases (NMDs).  OBJECTIVES: To assess the effects of interventions designed to promote physical activity in people with NMD compared with no intervention or alternative interventions. SEARCH METHODS: On 30 April 2020, we searched Cochrane Neuromuscular Specialised Register, CENTRAL, Embase, MEDLINE, and ClinicalTrials.Gov. WHO ICTRP was not accessible at the time. SELECTION CRITERIA: We considered randomised or quasi-randomised trials, including cross-over trials, of interventions designed to promote physical activity in people with NMD compared to no intervention or alternative interventions. We specifically included studies that reported physical activity as an outcome measure. Our main focus was studies in which promoting physical activity was a stated aim but we also included studies in which physical activity was assessed as a secondary or exploratory outcome. DATA COLLECTION AND ANALYSIS: We used standard Cochrane procedures. MAIN RESULTS: The review included 13 studies (795 randomised participants from 12 studies; number of participants unclear in one study) of different interventions to promote physical activity. Most studies randomised a minority of invited participants. No study involved children or adolescents and nine studies reported minimal entry criteria for walking. Participants had one of nine inherited or acquired NMDs. Types of intervention included structured physical activity support, exercise support (as a specific form of physical activity), and behaviour change support that included physical activity or exercise. Only one included study clearly reported that the aim of intervention was to increase physical activity. Other studies reported or planned to analyse the effects of intervention on physical activity as a secondary or exploratory outcome measure. Six studies did not report results for physical activity outcomes, or the data were not usable. We judged 10 of the 13 included studies at high or unclear risk of bias from incomplete physical activity outcome reporting. We did not perform a meta-analysis for any comparison because of differences in interventions and in usual care. We also found considerable variation in how studies reported physical activity as an outcome measure. The studies that reported physical activity measurement did not always clearly report intention-to-treat (ITT) analysis or whether final assessments occurred during or after intervention. Based on prespecified measures, we included three comparisons in our summary of findings. A physical activity programme (weight-bearing) compared to no physical activity programme One study involved adults with diabetic peripheral neuropathy (DPN) and reported weekly duration of walking during and at the end of a one-year intervention using a StepWatch ankle accelerometer. Based on the point estimate and low-certainty evidence, intervention may have led to an important increase in physical activity per week; however, the 95% confidence interval (CI) included the possibility of no difference or an effect in either direction at three months (mean difference (MD) 34 minutes per week, 95% CI -92.19 to 160.19; 69 participants), six months (MD 68 minutes per week, 95% CI -55.35 to 191.35; 74 participants), and 12 months (MD 49 minutes per week, 95% CI -75.73 to 173.73; 70 participants). Study-reported effect estimates for foot lesions and full-thickness ulcers also included the possibility of no difference, a higher, or lower risk with intervention. A sensor-based, interactive exercise programme compared to no sensor-based, interactive exercise programme One study involved adults with DPN and reported duration of walking over 48 hours at the end of four weeks' intervention using a t-shirt embedded PAMSys sensor. It was not possible to draw conclusions about the effectiveness of the intervention from the very low-certainty evidence (MD -0.64 hours per 48 hours, 95% CI -2.42 to 1.13; 25 participants). We were also unable to draw conclusions about impact on the Physical Component Score (PCS) for quality of life (MD 0.24 points, 95% CI -5.98 to 6.46; 35 participants; very low-certainty evidence), although intervention may have made little or no difference to the Mental Component Score (MCS) for quality of life (MD 5.10 points, 95% CI -0.58 to 10.78; 35 participants; low-certainty evidence). A functional exercise programme compared to a stretching exercise programme One study involved adults with spinal and bulbar muscular atrophy and reported a daily physical activity count at the end of 12 weeks' intervention using an Actical accelerometer. It was not possible to draw conclusions about the effectiveness of either intervention (requiring compliance) due to low-certainty evidence and unconfirmed measurement units (MD -8701, 95% CI -38,293.30 to 20,891.30; 43 participants). Functional exercise may have made little or no difference to quality of life compared to stretching (PCS: MD -1.10 points, 95% CI -5.22 to 3.02; MCS: MD -1.10 points, 95% CI -6.79 to 4.59; 49 participants; low-certainty evidence). Although studies reported adverse events incompletely, we found no evidence of supported activity increasing the risk of serious adverse events. AUTHORS' CONCLUSIONS: We found a lack of evidence relating to children, adolescents, and non-ambulant people of any age. Many people living with NMD did not meet randomised controlled trial eligibility criteria. There was variation in the components of supported activity intervention and usual care, such as physical therapy provision. We identified variation among studies in how physical activity was monitored, analysed, and reported. We remain uncertain of the effectiveness of promotional intervention for physical activity and its impact on quality of life and adverse events. More information is needed on the ITT population, as well as more complete reporting of outcomes. While there may be no single objective measure of physical activity, the study of qualitative and dichotomous change in self-reported overall physical activity might offer a pragmatic approach to capturing important change at an individual and population level.

Effects of Air Stacking on Dyspnea and Lung Function in Neuromuscular Diseases.

OBJECTIVE: To investigate whether the decrease in dyspnea in neuromuscular diseases after air stacking (AS) occurs mostly in patients with decreased inspiratory muscle force and ensuing chest wall restriction or heterogeneous ventilation across the lungs. DESIGN: Interventional, before-after study. SETTING: A neurorehabilitation inpatient and outpatient center. PARTICIPANTS: Fifteen consecutive adult patients affected by neuromuscular diseases (N=15). INTERVENTIONS: AS treatment. MAIN OUTCOME MEASURES: Patients had vital capacity (VC) and sniff nasal inspiratory pressure (SNIP) measured. We measured Borg score, oxygen saturation, and ventilation heterogeneity across the lung as estimated from the difference between respiratory resistance at 5 and 19 Hz (R5-19) with the forced oscillation technique before and 5, 30, 60, and 120 minutes after applying AS. RESULTS: Before AS, Borg score was significantly related to R5-19 (r2 0.46, P<.05) but not to VC % predicted, SNIP % predicted, and time since symptom onset. After AS, average Borg score gradually decreased (P=.005), whereas inspiratory flow resistance at 5 Hz, R5-19, and inspiratory reactance at 5 Hz tended to improve, despite not reaching statistical significance. The decrease in dyspnea at 60 and 120 minutes after AS significantly correlated with baseline R5-19 (r2 0.49, P<.01 and r2 0.29, P<.05, respectively), but not with VC % predicted, SNIP % predicted, time since symptom onset, and clinical severity score for patients affected by amyotrophic lateral sclerosis. CONCLUSIONS: These findings suggest that dyspnea in neuromuscular diseases is related to heterogeneous ventilation rather than inspiratory muscle force and/or lung volumes decrease. Restoring ventilation distribution across the lungs with AS appears to improve dyspnea.

Individualized Aerobic Exercise in Neuromuscular Diseases: A Pilot Study on the Feasibility and Preliminary Effectiveness to Improve Physical Fitness.

OBJECTIVE: Clear guidelines to prescribe aerobic exercise in neuromuscular diseases (NMD) are lacking, which hampers effective application in neuromuscular rehabilitation. This pilot study evaluated the feasibility and preliminary effectiveness of an individualized aerobic exercise program according to a recently developed training guide (B-FIT) to improve physical fitness in individuals with NMD. METHODS: Thirty-one individuals who were ambulatory and had 15 different slowly progressive NMD participated in a 4-month, polarized, home-based, aerobic exercise program. The program included 2 low-intensity sessions and 1 high-intensity session per week. Feasibility outcomes were the following: completion rate, proportion of followed sessions, adverse events, and participant and therapist satisfaction based on a self-designed questionnaire. Submaximal incremental exercise tests were used to assess the effects on physical fitness. RESULTS: Twenty-six participants (84%) completed the B-FIT program, and the proportion of followed sessions was >75%. Three adverse events were reported and resolved. Regarding satisfaction, participants (based on n = 9) reported feeling fitter, but training was considered insufficiently challenging. Physical therapists (n = 5) reported that B-FIT provides a clear, well-grounded guidance. They perceived the time investment for initiating the program and the carry-over to primary care as the main barriers. The mean (SD) submaximal heart rate (based on n = 20) reduced significantly by -6.5 beats per minute (95% CI = -11.8 to -1.2), from 121.7 (16.5) at baseline to 115.2 (14.3) after intervention. Submaximal ratings of perceived exertion, anaerobic threshold, and peak workload also improved significantly (P < .05). CONCLUSION: The outcomes of this pilot study suggest that individualized aerobic exercise according to B-FIT is feasible and has potential to improve physical fitness in a wide variety of slowly progressive NMD. However, some barriers must be addressed before investigating the efficacy in a randomized controlled trial. IMPACT: The outcomes of this study demonstrate the feasibility of individualized aerobic exercise according to the B-FIT training guide and the potential to improve physical fitness in NMD. Physical therapists indicated that the use of B-FIT provides a clear, well-grounded guidance. The training guide can support health care professionals in the application of aerobic exercise in adult neuromuscular rehabilitation. LAY SUMMARY: Individualized exercise according to the B-FIT training guide is feasible in a wide variety of slowly progressive NMD and might help improve physical fitness.

Acute flaccid myelitis: cause, diagnosis, and management.

Acute flaccid myelitis (AFM) is a disabling, polio-like illness mainly affecting children. Outbreaks of AFM have occurred across multiple global regions since 2012, and the disease appears to be caused by non-polio enterovirus infection, posing a major public health challenge. The clinical presentation of flaccid and often profound muscle weakness (which can invoke respiratory failure and other critical complications) can mimic several other acute neurological illnesses. There is no single sensitive and specific test for AFM, and the diagnosis relies on identification of several important clinical, neuroimaging, and cerebrospinal fluid characteristics. Following the acute phase of AFM, patients typically have substantial residual disability and unique long-term rehabilitation needs. In this Review we describe the epidemiology, clinical features, course, and outcomes of AFM to help to guide diagnosis, management, and rehabilitation. Future research directions include further studies evaluating host and pathogen factors, including investigations into genetic, viral, and immunological features of affected patients, host-virus interactions, and investigations of targeted therapeutic approaches to improve the long-term outcomes in this population.

Effect of Lung Volume Recruitment on Pulmonary Function in Progressive Childhood-Onset Neuromuscular Disease: A Systematic Review.

OBJECTIVES: The focus of this systematic review was to consider whether lung volume recruitment (LVR) has an effect on pulmonary function test parameters in individuals with progressive childhood-onset neuromuscular diseases. The review was registered on PROSPERO (No. CRD42019119541). DATA SOURCES: A systematic search of the CINAHL, MEDLINE, AMED, EMCARE, Scopus, and Open Grey databases was undertaken in January 2019 considering LVR in the respiratory management of childhood-onset neuromuscular diseases. STUDY SELECTION: Studies were included if either manual resuscitator bags or volume-controlled ventilators were used to perform LVR with participants older than 6 years of age. Critical appraisal tools from the Joanna Briggs Institute were used to assess the quality of studies. Nine studies were identified, 6 of which were of sufficient quality to be included in the review. DATA EXTRACTION: Data extraction used a tool adapted from the Cochrane effective practice and organization of care group. DATA SYNTHESIS: Results were compiled using a narrative synthesis approach focused on peak cough flow, forced vital capacity, and maximum inspiratory capacity outcomes. CONCLUSIONS: Limited evidence suggests an immediate positive effect of LVR on peak cough flow and a potential long-term effect on the rate of forced vital capacity decline. Considering the accepted correlation between forced vital capacity and morbidity, this review suggests that LVR be considered for individuals with childhood-onset neuromuscular diseases once forced vital capacity starts to deteriorate. This review is limited by small sample sizes and the overall paucity of evidence considering LVR in this population group. Controlled trials with larger sample sizes are urgently needed.

Recommendations for Therapy following Nerve Transfer for Children with Acute Flaccid Myelitis.

AIM: To provide recommendations for pre- and post-operative occupational and physical therapy for children with acute flaccid myelitis (AFM). METHODS: Writing panel members consisted of an interdisciplinary team of seven healthcare professionals specializing in the care of children with AFM. The panel reviewed background material on AFM, nerve transfer, and rehabilitation principles applied to pediatrics. Recommendations were prioritized if evidence was available. Where there was no known evidence to support a recommendation, this was noted. RECOMMENDATIONS: Communication and coordination among interprofessional team members are vital to a comprehensive family-centered rehabilitation program. Surgical planning should include team preparation accounting for frequency, duration, and timing of treatment, as well as individual characteristics and developmental status of the child. Recommendations for pre-operative and six phases of post-operative therapy address assessment, strengthening, range of motion, orthoses, performance of functional activity, and support of the family. CONCLUSION: Rehabilitation following nerve transfer in children with AFM requires interdisciplinary collaboration and a multisystem approach to assessment and treatment. As new evidence becomes available, recommendations may be revised or replaced accordingly.

Development and evaluation of a novel music-based therapeutic device for upper extremity movement training: A pre-clinical, single-arm trial.

Restoration of upper limb motor function and patient functional independence are crucial treatment targets in neurological rehabilitation. Growing evidence indicates that music-based intervention is a promising therapeutic approach for the restoration of upper extremity functional abilities in neurologic conditions such as cerebral palsy, stroke, and Parkinson's Disease. In this context, music technology may be particularly useful to increase the availability and accessibility of music-based therapy and assist therapists in the implementation and assessment of targeted therapeutic goals. In the present study, we conducted a pre-clinical, single-arm trial to evaluate a novel music-based therapeutic device (SONATA) for upper limb extremity movement training. The device consists of a graphical user interface generated by a single-board computer displayed on a 32" touchscreen with built-in speakers controlled wirelessly by a computer tablet. The system includes two operational modes that allow users to play musical melodies on a virtual keyboard or draw figures/shapes whereby every action input results in controllable sensory feedback. Four motor tasks involving hand/finger movement were performed with 21 healthy individuals (13 males, aged 26.4 ± 3.5 years) to evaluate the device's operational modes and main features. The results of the functional tests suggest that the device is a reliable system to present pre-defined sequences of audiovisual stimuli and shapes and to record response and movement data. This preliminary study also suggests that the device is feasible and adequate for use with healthy individuals. These findings open new avenues for future clinical research to further investigate the feasibility and usability of the SONATA as a tool for upper extremity motor function training in neurological rehabilitation. Directions for future clinical research are discussed.

Daily Life Benefits and Usage Characteristics of Dynamic Arm Supports in Subjects with Neuromuscular Disorders.

Neuromuscular disorders cause progressive muscular weakness, which limits upper extremity mobility and performance during activities of daily life. Dynamic arm supports can improve mobility and quality of life. However, their use is often discontinued over time for unclear reasons. This study aimed to evaluate whether users of dynamic arm supports demonstrate and perceive quantifiable mobility benefits over a period of two months. Nine users of dynamic arm supports were included in this observational study. They had different neuromuscular disorders and collectively used four different arm supports. They were observed for three consecutive weeks during which they were equipped with a multi-sensor network of accelerometers to assess the actual use of the arm support and they were asked to provide self-reports on the perceived benefits of the devices. Benefits were experienced mainly during anti-gravity activities and the measured use did not change over time. The self-reports provided contextual information in domains such as participation to social life, in addition to the sensor system. However self-reports overestimated the actual use by up to three-fold compared to the accelerometer measures. A combination of objective and subjective methods is recommended for meaningful and quantifiable mobility benefits during activities of daily life.

Acute Flaccid Myelitis in an Adult.

INTRODUCTION: Acute flaccid myelitis (AFM) is a condition causing acute onset flaccid limb weakness primarily in children. Correlations with specific viral illnesses have been found, however, specific pathogenesis remains unknown. CASE REPORT: A 21-year-old female individual presented with progressive weakness provoking multiple falls, severe fatigue, headaches, and body aches after an upper respiratory illness. Deep tendon reflexes were absent, and cerebrospinal fluid analysis was remarkable for lymphocytic pleocytosis and elevated protein. Magnetic resonance imaging revealed T2 hyperintensity extending from C1-T2 predominantly involving the anterior horns. Weakness continued to increase before subtly improving over the course of a 10-day hospital stay. Functional improvements had been achieved by the patient at 1-year follow-up with intensive physical therapy. CONCLUSIONS: AFM should be included in the differential for the presentation of acute weakness in adults and pediatric patients. As the incidence of AFM continues to rise, awareness of the condition and prompt obtainment of specimens in suspected patients is crucial to aid in the investigation.

Neural feedback strategies to improve grasping coordination in neuromusculoskeletal prostheses.

Conventional prosthetic arms suffer from poor controllability and lack of sensory feedback. Owing to the absence of tactile sensory information, prosthetic users must rely on incidental visual and auditory cues. In this study, we investigated the effect of providing tactile perception on motor coordination during routine grasping and grasping under uncertainty. Three transhumeral amputees were implanted with an osseointegrated percutaneous implant system for direct skeletal attachment and bidirectional communication with implanted neuromuscular electrodes. This neuromusculoskeletal prosthesis is a novel concept of artificial limb replacement that allows to extract control signals from electrodes implanted on viable muscle tissue, and to stimulate severed afferent nerve fibers to provide somatosensory feedback. Subjects received tactile feedback using three biologically inspired stimulation paradigms while performing a pick and lift test. The grasped object was instrumented to record grasping and lifting forces and its weight was either constant or unexpectedly changed in between trials. The results were also compared to the no-feedback control condition. Our findings confirm, in line with the neuroscientific literature, that somatosensory feedback is necessary for motor coordination during grasping. Our results also indicate that feedback is more relevant under uncertainty, and its effectiveness can be influenced by the selected neuromodulation paradigm and arguably also the prior experience of the prosthesis user.

Efficacy of a physical activity programme combining individualized aerobic exercise and coaching to improve physical fitness in neuromuscular diseases (I'M FINE): study protocol of a randomized controlled trial.

BACKGROUND: In individuals with neuromuscular diseases (NMD), symptoms of muscle weakness, fatigue and pain may limit physical activity. Inactivity leads to reduced physical fitness, which further complicates daily life functioning. Due to inconclusive evidence regarding exercise in NMD, the optimal training approach and strategies to preserve an active lifestyle remain to be determined. The physical activity programme I'M FINE, consisting of individualized aerobic exercise to improve physical fitness and coaching to preserve an active lifestyle, was therefore developed. The primary objective of this study will be to evaluate the efficacy of the I'M FINE programme in terms of improved physical fitness in individuals with slowly progressive NMD, compared to usual care. METHODS: A multicentre, assessor-blinded, two armed, randomized controlled trial will be conducted in a sample of 90 individuals with slowly progressive NMD. Participants motivated to improve their reduced physical fitness will be randomized (ratio 1:1) to the I'M FINE intervention or usual care. The I'M FINE intervention consists of a six-month physical activity programme, including individualized home-based aerobic exercise to improve physical fitness (i.e. peak oxygen uptake), and motivational interviewing coaching (e.g. goal setting, self-management) to adopt and preserve an active lifestyle. Measurements will be performed at baseline, post-intervention, and at 12- and 18-months follow-up. The primary outcome is peak oxygen uptake (VO2 peak) directly post intervention. Main secondary outcomes are physical capacity, muscle strength, self-efficacy, daily activity, quality of life and markers of metabolic syndrome. The primary analysis compares change in VO2 peak post-intervention between the intervention and usual care group, with analysis of covariance. DISCUSSION: The I'M FINE study will provide evidence regarding the efficacy of a physical activity intervention on the physical fitness and active lifestyle over the short- and long-term in individuals with slowly progressive NMD. These outcomes could potentially improve the (inter)national guidelines for efficacy of aerobic exercise programmes and provide insight in achieving a more active lifestyle in NMD. TRIAL REGISTRATION: (5/11/2018): Netherlands Trial Register NTR7609 (retrospectively registered), https://www.trialregister.nl/trial/7344. However, the Ethics Review Committee of the Amsterdam Medical Center (AMC) approved the study protocol on 7/11/2017. No adjustments were made to the approved study protocol before the first participant enrolment and registration. Registration was done after the second participant enrolment and the information in the register corresponds one on one with the approved study protocol.

Emotiv EPOC+ fed electrical muscle stimulation system; an inexpensive brain-computer interface for rehabilitation of neuro muscular disorders.

Advancements in the Neuro-rehabilitation across Pakistan is warranted to effectively and efficiently deal with the disease burden of neurological conditions. Being a developing country, an in-expensive treatment approach is required to culminate the rise in the disease occurrence in Pakistan. Brain-Computer Interfaces (BCIs) have come up as a new channel for communication and control, eliminating the need of physical input, opening doors to a wide array of applications in terms of assistive and rehabilitative devices for paralyzed patients and those with neuromuscular disorders. Even with a promising prospect, BCIs and electroencephalograms (EEG) can be very expensive and therefore, they are not practically applicable. For this reason, the purpose of the current study was to come up with a possibility of an inexpensive BCI for rehabilitation of patients with neuro-muscular disorders in Pakistan by using a low-cost and readily available equipment like Emotiv EPOC+ EEG headset and electrical muscle stimulator.