The Utility of Neuromuscular Assessment to Identify ADHD Among Patients with a Complex Symptom Picture.

OBJECTIVE: Diagnostic assessment of ADHD is challenging due to comorbid psychopathologies and symptoms overlapping with other psychiatric disorders. In this study, we investigate if a distinct pattern of neuromuscular dysregulation previously reported in ADHD, can help identifying ADHD in psychiatric patients with diverse and complex symptoms. METHOD: We explored the impact of neuromuscular dysregulation, as measured by The Motor Function Neurologic Assessment (MFNU), on the likelihood of being diagnosed with ADHD, affective disorder, anxiety disorder, or personality disorder among adults (n = 115) referred to a psychiatric outpatient clinic. RESULTS: Logistic regression revealed that neuromuscular dysregulation was significantly associated with ADHD diagnosis only (OR 1.15, p < .01), and not with affective-, anxiety-, or personality disorders. Sensitivity and specificity for ADHD at different MFNU scores is provided. CONCLUSIONS: A test of neuromuscular dysregulation may promote diagnostic accuracy in differentiating ADHD from other psychiatric disorders in patients with an overlapping symptom picture. This may have important implications for clinical practice. More studies are needed.

Evaluation and management of dyspnea as the dominant presenting feature in neuromuscular disorders.

Dyspnea is a common symptom in neuromuscular disorders and, although multifactorial, it is usually due to respiratory muscle involvement, associated musculoskeletal changes such as scoliosis or, in certain neuromuscular conditions, cardiomyopathy. Clinical history can elicit symptoms such as orthopnea, trepopnea, sleep disruption, dysphagia, weak cough, and difficulty with secretion clearance. The examination is essential to assist with the diagnosis of an underlying neurologic disorder and determine whether dyspnea is from a cardiac or pulmonary origin. Specific attention should be given to possible muscle loss, use of accessory muscles of breathing, difficulty with neck flexion/extension, presence of thoraco-abdominal paradox, conversational dyspnea, cardiac examination, and should include a detailed neurological examination directed at the suspected differential diagnosis. Pulmonary function testing including sitting and supine spirometry, measures of inspiratory and expiratory muscle strength, cough peak flow, sniff nasal inspiratory pressure, pulse oximetry, transcutaneous CO2, and arterial blood gases will help determine the extent of the respiratory muscle involvement, assess for hypercapnic or hypoxemic respiratory failure, and qualify the patient for noninvasive ventilation when appropriate. Additional testing includes dynamic imaging with sniff fluoroscopy or diaphragm ultrasound, and diaphragm electromyography. Polysomnography is indicated for sleep related symptoms that are not otherwise explained. Noninvasive ventilation alleviates dyspnea and nocturnal symptoms, improves quality of life, and prolongs survival. Therapy targeted at neuromuscular disorders may help control the disease or favorably modify its course. For patients who have difficulty with secretion clearance, support of expiratory function with mechanical insufflation-exsufflation, oscillatory devices can reduce the aspiration risk.

[Sleep in chronic neuropediatric diseases]. / Sueño en enfermedades neuropediátricas crónicas.

The prevalence of sleep disorders (SD) is notoriously increased in children with chronic neurological disease, with a negative bidirectional link that aggravates their symptomatology and has a negative impact on the quality of life of the child and their families. Identifying and recognizing this association is key for the child neurologist since the treatment of SD significantly improves daytime symptomatology in neurodevelopmental disorders, epilepsy, primary headaches, cerebral palsy and neuromuscular diseases.

La prevalencia de los trastornos del sueño (TS) se incrementa notoriamente en niños con enfermedad neurológica crónica, con un vínculo bidireccional negativo que agrava su sintomatología y repercute negativamente en la calidad de vida del niño y su familia. Identificar y reconocer dicha asociación es clave para el neuropediatra, ya que el tratamiento del TS mejora significativamente la sintomatología diurna de los trastornos del neurodesarrollo, epilepsia, cefaleas primarias, parálisis cerebral y enfermedades neuromusculares.

[Neurophysiological substantiation and validity assessment of manual muscle testing in clinical practice. (A literature review)]. / Neirofiziologicheskoe obosnovanie i otsenka validnosti manual'nogo myshechnogo testirovaniya v klinicheskoi praktike. (Obzor literatury).

The relevance of the study of neuromuscular dysfunction's causes and mechanisms is undeniable, considering the large number of nosologies accompanied by malfunction of muscles. Adequate diagnosis and correction of these disorders is impossible without understanding of their pathogenetic mechanisms. Currently, manual muscle testing (MMT) is a widespread technique. MMT is an agile diagnostic tool used by physiatrists, doctors in sports medicine, osteopaths and rehabilitation physicians to assess the functional status of muscles. Unconditionally, this method attracts with its low cost, which will optimize the financial costs of hospital and the healthcare system as a whole. In addition, there is no clear substantiation of the objectivity and validity of the MMT to date. The article considers the issues of neurophysiological principles, classification of methods and approaches, assessment criteria of repeatability and accuracy of MMT. Understanding of the pathophysiological mechanisms of MMT effectiveness will allow to timely correct the therapy and improve the results of treatment and rehabilitation of patients with neuromuscular dysfunction.

Utility of the Clustering Index method for diagnosing neuromuscular disorders as compared with needle electromyography.

INTRODUCTION/AIMS: Concentric needle electromyography (CNEMG) is an essential examination for evaluating neuromuscular disorders, although pain is a drawback. Clustering Index (CI) method is a non-invasive quantitative analysis for surface electromyography (SEMG) that evaluates whether the signal area is clustered into the few large motor unit potentials (MUPs) or is evenly distributed. However, the diagnostic yield of the CI method in comparison with CNEMG is not known. In this study, we aimed to compare the sensitivity of the CI method with MUP parameters in CNEMG for diagnosing neurogenic or myogenic disorders. METHODS: We retrospectively identified patients for whom both SEMG and CNEMG were performed on the same tibialis anterior (TA) muscle. In CNEMG, seven MUP parameters were evaluated, including size index (SI) and revised size indices for neurogenic (rSIn) and myogenic (rSIm) disorders. RESULTS: Identified were 21 patients with neurogenic and 21 patients with myogenic disorders. Control data were constructed from 30 control subjects. The sensitivities of the CI method for the neurogenic and myogenic groups were 76% and 62%, respectively, which were not significantly different from MUP parameters, except for being significantly higher than those of amplitude and duration for myopathy (24%). Among MUP parameters, the sensitivities of rSIn (62%) and rSIm (57%) for myopathy were significantly higher than those of amplitude and duration. The CI method significantly correlated with the strength of the TA muscle in myopathy. DISCUSSION: The CI method, having comparable diagnostic yields to MUP parameters, is promising as a non-invasive diagnostic measure.

Phrenic neuropathy etiologies and recovery trajectories in outpatient rehabilitation and neuromuscular medicine clinics: A retrospective analysis.

INTRODUCTION/AIMS: Phrenic neuropathy (PhN) impairs diaphragm muscle function, causing a spectrum of breathing disability. PhN etiologies and their natural history are ill-defined. This knowledge gap hinders informed prognosis and management decisions. This study aims to help fill this knowledge gap on PhN etiologies, outcomes, and recovery patterns, especially in the context of nonsurgical clinical practice. METHODS: This was a retrospective study from two interdisciplinary clinics, physiatry and neurology based. Patients were included if PhN was identified, and other causes of hemi-diaphragm muscle dysfunction excluded. Patients were followed serially at the discretion of the neuromuscular-trained neurologist or physiatrist. Recovery was assessed using pulmonary function tests (PFTs), diaphragm muscle ultrasound (US) thickening ratio, and patient-reported outcomes in patients presenting within 2 years of PhN onset. RESULTS: We identified 151 patients with PhN. The most common etiologies were idiopathic (27%), associated with cardiothoracic procedure (24%), and intensive care unit (17%). Of these patients, 117 (77%) were evaluated within 2 years of PhN onset. Of patients included in outcome analyses, 64% saw improvement on serial US, 50% on serial PFTs and 79% reported symptomatic improvement at an average of 15, 16, and 17 months, respectively. DISCUSSION: A clear majority of PhN patients show improvement in diaphragm muscle function, but on average, improvements took 15-17 months depending on the assessment type. These insights are vital for developing tailored treatments and can guide physicians in prognosis and decision-making, especially if more invasive interventions are being considered.

Design and Development of a Powered Myoelectric Elbow Orthosis for Neuromuscular Injuries.

INTRODUCTION: Recovering from neuromuscular injuries or conditions can be a challenging journey that involves complex surgeries and extensive physical rehabilitation. During this process, individuals often rely on orthotic devices to support and enable movement of the affected limb. However, users have criticized current commercially available powered orthotic devices for their bulky and heavy design. To address these limitations, we developed a novel powered myoelectric elbow orthosis. MATERIALS AND METHODS: The orthosis incorporates 3 mechanisms: a solenoid brake, a Bowden cable-powered constant torque elbow mechanism, and an extension limiter. The device controller and battery are in a backpack to reduce the weight on the affected arm. We performed extensive calculations and testing to ensure that the orthosis could withstand at least 15 Nm of elbow torque. We developed a custom software effectively control the orthosis, enhancing its usability and functionality. A certified orthotist fitted a subject who had undergone a gracilis free functioning muscle transfer surgery with the device. We studied the subject under Mayo clinic IRB no. 20-006849 and obtained objective measurements to assess the orthosis's impact on upper extremity functionality during daily activities. RESULTS: The results are promising since the orthosis significantly improved elbow flexion range of motion by 40° and reduced compensatory movements at the shoulder (humerothoracic joint) by 50°. Additionally, the subject was able to perform tasks which were not possible before, such as carrying a basket with weights, highlighting the enhanced functionality provided by the orthosis. CONCLUSION: In brief, by addressing the limitations of existing devices, this novel powered myoelectric elbow orthosis offers individuals with neuromuscular injuries/conditions improved quality of life. Further research will expand the patient population and control mechanisms.

Impact of Disease-modifying Therapies on Respiratory Function in People with Neuromuscular Disorders.

Spinal muscular atrophy (SMA) and Duchenne muscular dystrophy (DMD) are neuromuscular disorders that affect muscular function. The most common causes of morbidity and mortality are respiratory complications, including restrictive lung disease, ineffective cough, and sleep-disordered breathing. The paradigm of care is changing as new disease-modifying therapies are altering disease trajectory, outcomes, expectations, as well as patient and caregiver experiences. This article provides an overview on therapeutic advances for SMA and DMD in the last 10 years, with a focus on the effects of disease-modifying therapies on respiratory function.

Airway Clearance in Neuromuscular Disease.

High-quality respiratory care and airway clearance is essential for people with neuromuscular disease (pwNMD) as respiratory tract infections are a major cause of morbidity and mortality. This review expands on published guidelines by highlighting the role of cough peak flow along with other options for cough evaluation, and discusses recent key research findings which have influenced the practice of respiratory therapy for pwNMD.

274th ENMC international workshop: recommendations for optimizing bone strength in neuromuscular disorders. Hoofddorp, The Netherlands, 19-21 January 2024.

The 274th ENMC workshop for optimizing bone strength in neuromuscular disorders (NMDs) was held on January 19-21, 2024. The group of participants included experts in the fields of bone health and neuromuscular medicine along with the patient voice. Bone strength represents a crucial aspect of the management of pediatric and adult patients with NMDs. Bone strength may be compromised due to different pathophysiologic mechanisms, including disrupted bone-muscle "cross-talk", loss of biomechanical loading, nutritional insufficiency, inadequate weight-bearing physical activity, muscle weakness and/or immobility, and drug treatment. While for Duchenne muscular dystrophy recommendations for evaluation and treatment of bone strength have been published, evidence on bone strength in other hereditary and acquired NMDs is scarce. Enhanced knowledge is needed to understand the development and maintenance of bone strength in patients with NMDs. This workshop aimed to develop a strategy to improve bone strength and thus prevent fractures in patients with NMDs.

Nociceptive Pain in Patients with Neuromuscular Disorders: A Cross-Sectional Clinical Study.

Background: Muscle pain is a common symptom in patients with neuromuscular disorders (NMD) and accounts for severely reduced quality of life. OBJECTIVE: This clinical study aimed to observe possible differences in pain prevalence among distinct NMDs and to determine whether the patients' nociceptive pain is influenced by gender, muscle strength and psychological factors and to examine potential pain-associated alterations in muscle properties. Methods: The cross-sectional study on nociceptive pain in various NMDs involved patient-reported outcomes, muscle strength evaluations (dynamometry and quick motor function test (QMFT)), nociceptive pain evaluations (muscular pressure pain threshold (PPT)), and non-invasive measurement of muscle stiffness, frequency, decrement, relaxation, and creep (myotonometry). Results: Involving 81 NMD patients and a control group, the study found high variability in pain prevalence among the subgroups. Patients with DM2 and FSHD had significantly higher levels of pain prevalence compared to other examined NMD subgroups and the control group. Female gender, high fatigue levels (representing factors such as depression, anxiety, stress, and impairment of quality of life), and low QMFT scores (representing reduced muscle strength) showed an association with increased sensitivity to pressure pain in the arm and leg region. As assessed by myotonometry, less pain is experienced in neck muscles with a high muscle tone, high stiffness, and a short relaxation time highlighting the importance of intrinsic muscular tone for their pressure pain sensitivity. Conclusion: Individualized therapeutic concepts including psychological and physical approaches in the pain management of patients with NMDs, especially in women, should be considered. Further research in this field is necessary to gain a more detailed insight into the perception of muscle pain.

Validity of Cardiopulmonary Exercise Testing for Assessing Aerobic Capacity in Neuromuscular Diseases.

OBJECTIVES: To determine the content validity of cardiopulmonary exercise testing (CPET) for assessing peak oxygen uptake (VO2peak) in neuromuscular diseases (NMD). DESIGN: Baseline assessment of a randomized controlled trial. SETTING: Academic hospital. PARTICIPANTS: Eighty-six adults (age: 58.0±13.9 y) with Charcot-Marie-Tooth disease (n=35), postpolio syndrome (n=26), or other NMD (n=25). INTERVENTION: Not applicable. MAIN OUTCOME MEASURES: Workload, gas exchange variables, heart rate, and ratings of perceived exertion were measured during CPET on a cycle ergometer, supervised by an experienced trained assessor. Muscle strength of the knee extensors was assessed isometrically with a fixed dynamometer. Criteria for confirming maximal cardiorespiratory effort during CPET were established during 3 consensus meetings of an expert group. The percentage of participants meeting these criteria was assessed to quantify content validity. RESULTS: The following criteria were established for maximal cardiorespiratory effort: a plateau in oxygen uptake (VO2plateau) as the primary criterion, or 2 of 3 secondary criteria: (1) peak respiratory exchange ratio (RERpeak) ≥1.10 (2), peak heart rate ≥85% of predicted maximal heart rate; and (3) peak rating of perceived exertion (RPEpeak) ≥17 on the 6-20 Borg scale. These criteria were attained by 71 participants (83%). VO2plateau, RERpeak ≥1.10, peak heart rate ≥85%, and RPEpeak ≥17 were attained by 31%, 73%, 69%, and 72% of the participants, respectively. Peak workload, VO2peak, and knee extension muscle strength were significantly higher, and body mass index was lower (all P<.05), in participants with maximal cardiorespiratory effort than other participants. CONCLUSIONS: Most people with NMD achieved maximal cardiorespiratory effort during CPET. This study provides high quality evidence of sufficient content validity of VO2peak as a maximal aerobic capacity measure. Content validity may be lower in more severely affected people with lower physical fitness.

Neuromuscular disease auxiliary diagnosis using a portable magnetomyographic system.

Objective. The measurement of electromyography (EMG) signals with needle electrodes is widely used in clinical settings for diagnosing neuromuscular diseases. Patients experience pain during needle EMG testing. It is significant to develop alternative diagnostic modalities.Approach. This paper proposes a portable magnetomyography (MMG) measurement system for neuromuscular disease auxiliary diagnosis. Firstly, the design and operating principle of the system are introduced. The feasibility of using the system for auxiliary diagnosis of neuromuscular diseases is then studied. The magnetic signals and needle EMG signals of thirty subjects were collected and compared.Main results. It is found that the amplitude of muscle magnetic field signal increases during mild muscle contraction, and the signal magnitudes of the patients are smaller than those of normal subjects. The diseased muscles tested in the experiment can be distinguished from the normal muscles based on the signal amplitude, using a threshold value of 6 pT. The MMG diagnosis results align well with the needle EMG diagnosis. In addition, the MMG measurement indicates that there is a persistence of spontaneous activity in the diseased muscle.Significance.The experimental results demonstrate that it is feasible to auxiliary diagnose neuromuscular diseases using the portable MMG system, which offers the advantages of non-contact and painless measurements. After more in-depth, systematic, and quantitative research, the portable MMG could potentially be used for auxiliary diagnosis of neuromuscular diseases. The clinical trial registration number is ChiCTR2200067116.

Neuromuscular Disorders in Pediatric Respiratory Disease.

Respiratory sequelae are a frequent cause of morbidity and mortality in children with NMD. Impaired cough strength and resulting airway clearance as well as sleep disordered breathing are the two main categories of respiratory sequelae. Routine clinical evaluation and diagnostic testing by pulmonologists is an important pillar of the multidisciplinary care required for children with NMD. Regular surveillance for respiratory disease and timely implementation of treatment including pulmonary clearance techniques as well as ventilation can prevent respiratory related morbidity including hospital admissions and improve survival. Additionally, novel disease modifying therapies for some NMDs are now available which has significantly improved the clinical trajectories of patients resulting in a paradigm shift in clinical care. Pulmonologists are 'learning' the new natural history for these diseases and adjusting clinical management accordingly.

Practical recommendations for swallowing and speaking during NIV in people with neuromuscular disorders.

Objectives: The functions of eating, drinking, speaking, and breathing demand close coordination of the upper airway musculature which may be challenged by the long-term use of daytime non-invasive ventilation (NIV). This rapid review explores the challenges and practicalities of these interactions in people with neuromuscular disorders. Methods: A search was performed on PubMed (period 2000-2023) using generic terms to refer to eating, drinking, and speaking related to people with neuromuscular disorders on NIV. A narrative approach was used to summarize the available literature. Results: Our review shows only a small number of studies exist exploring the use of NIV on swallowing and speaking in people with neuromuscular disorders. We summarize study findings and provide practical advice on eating, drinking and speaking with NIV. Conclusions: By understanding breathing, NIV mechanics and upper airway interactions, it is possible to optimize swallowing and speaking whilst using NIV. There is a lack of specific guidelines, and concerns regarding aspiration warrant further research and guideline development.

Enhancing respiratory function in neuromuscular disease: the role of non-invasive ventilation. A narrative review.

Neuromuscular diseases (NMDs) comprise a heterogeneous group of conditions characterized by extreme progressive muscle weakness leading to respiratory failure. Noninvasive mechanical ventilation (NIV) has emerged as a cornerstone in the management of respiratory complications associated with NMDs. This review aims to elucidate the role of NMV in respiratory function, improving quality of life, and prolonging survival in individuals with NMD. The physiological basis of respiratory impairment in NMDs, principles of NMV application, evidence supporting its efficacy, patient selection criteria, and potential challenges in its application are discussed.

Rasch analysis of the modified Fatigue Severity Scale in neuromuscular disorders and comparison between sex, age and diagnoses.

INTRODUCTION/AIMS: Fatigue is a common and debilitating symptom encountered in the neuromuscular clinic. The 7-item Fatigue Severity Scale (FSS-7) is a Rasch-modified assessment validated in inflammatory neuropathies but not across a typical neuromuscular patient population. The aim of this study was to validate this measure in neuromuscular disorders and to compare between patient sex, age and diagnoses. METHODS: The modified FSS-7 was mailed to patients recruited from a specialist neuromuscular clinic at the Walton Centre. Responses were subjected to Rasch analysis and descriptive statistics were performed on the Rasch converted data. RESULTS: The mFSS-7 met the Rasch model expectations with an overall Chi-square probability of 0.4918, a strict unidimensional scale free from differential item functioning (DIF) that satisfied the model with substantial test-retest reliability using Lin's concordance correlation coefficient 0.71 (95% CI 0.63-0.77). A 15.7% ceiling effect was observed in this patient cohort. Post hoc analysis did not show any significant difference in fatigue between sex, age or neuromuscular diagnoses. DISCUSSION: The self-completed Rasch mFSS-7 showed acceptable test-retest reliability across patients with varied disorders under follow-up in a specialist neuromuscular clinic. The ceiling effect constrains its use for those with the most severe fatigue. Future considerations could include assessment of the benefits of clinical interventions, particularly multidisciplinary team input or dedicated fatigue clinics.

Impact of Wheelchair Seating Systems on Scoliosis Progression for Children With Neurologic and Neuromuscular Disorders: A Retrospective Study of Custom-Contoured Wheelchair Seating and Modular Wheelchair Seating.

OBJECTIVES: To retrospectively evaluate the comparative effect of 2 wheelchair seating systems, Custom-Contoured Wheelchair Seating (CCS) and Modular Wheelchair Seating (MWS), on scoliosis progression in children with neuromuscular and neurologic disorders and to determine any predictors for scoliosis progression. DESIGN: Longitudinal, retrospective cohort study. SETTING: A national health service regional posture and mobility service. PARTICIPANTS: Nonambulant pediatric wheelchair users with neuromuscular and neurologic disorders (N=75; 36 men, 39 women; mean age at seating intervention, 10.50±3.97y) issued CCS and MWS by the South Wales Posture and Mobility Service from 2012-2022. INTERVENTIONS: Two specialized wheelchair seating systems, CCS and MWS. MAIN OUTCOME MEASURES: A generalized least squares model was used to estimate the effect of seat type on Cobb angle over time. RESULTS: Of the 75 participants enrolled, 51% had cerebral palsy. Fifty were issued CCS and 25 were issued MWS. Baseline Cobb angle was 32.9±18.9° for the MWS group and 48.0±31.0° for the CCS group. The generalized least squares model demonstrated that time since seating intervention (χ2=122, P<.0001), seating type (χ2=52.5, P<.0001), and baseline scoliosis severity (χ2=41.6, P<.0001) were predictive of scoliosis progression. Condition was not a strong predictor (χ2=9.96, P =.0069), and sex (χ2=5.67, P=.13) and age at intervention (χ2=4.47, P=.35) were not predictive. Estimated contrasts of medical condition with seat type over time demonstrated smaller differences between MWS and CCS over time. Predicted scoliosis velocity was found to attenuate with use of CCS over time compared with MWS, although scoliosis deteriorated regardless of intervention. CONCLUSIONS: Our findings showed pediatric wheelchair users with neurologic and neuromuscular disorders prescribed CCS showed greater mitigation of scoliosis progression over time compared with those issued MWS.

Future Directions for Respiratory Muscle Training in Neuromuscular Disorders: A Scoping Review.

BACKGROUND: Respiratory muscle training (RMT) aims to improve inspiratory and/or expiratory muscle function in neuromuscular disorders (NMDs). A comprehensive overview of the available literature is lacking. This scoping review explores methodological characteristics, (adverse) effects, and adherence of RMT studies in NMDs. Moreover, it identifies limitations and research gaps in the literature and provides future research directions. SUMMARY: Eligible studies were identified using MEDLINE, Embase, Cochrane Database of Systematic Reviews, and Cochrane Central Register of Controlled Trials databases. Three reviewers independently selected articles. Inclusion criteria were English language, original research articles on RMT using a device, patients with an NMD, and pulmonary function tests or respiratory muscle strength as outcome measures. We included NMDs with slow, intermediate and fast progression. Exclusion criteria were critically ill patients, weaning from mechanical ventilation, other neurological disorders, and RMT combined with non-respiratory interventions. One reviewer extracted the data on patients' characteristics, methodological characteristics, results of outcome measures, adverse events, and patient adherence. Forty-five studies were identified. We found a large diversity in study designs and training protocols. The effects of RMT on respiratory muscle strength and/or endurance are variable. Patient adherence was high and no serious adverse events were reported. KEY MESSAGES: The diversity in studies across the available literature precludes definitive conclusions regarding the effects of RMT on respiratory muscle function and clinically relevant outcomes in NMDs. Therefore, well-powered and -designed studies that focus on clinically relevant outcomes and assess whether RMT can improve or offset deterioration of respiratory muscle weakness in NMDs are needed.

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.