Introduction. - The Physical Self Inventory-NeuroMuscular Diseases (PSI-NMD) is a scale which evaluates overall self-esteem and physical esteem in patients with neuromuscular disease. It comes from the Physical Self Inventory -10 item (PSI -10), which comes from the Physical Self Inventory -25 items, a validated questionnaire for French population. Objective. - The purpose of this study is to present the first step of the validation of this scale in patients with neuromuscular diseases. This scale assesses the overall self-esteem as well as the self-esteem in physical field. Method. - In all, 108 patients with myopathy or neuropathy and 129 individuals from the general population answered to the ISP-10 and the Rosenberg's self-esteem scale. Results. - Exploratory factorial analysis showed the weakness of two items, which have been deleted. In addition, results indicated good indices of internal consistency, convergent and discriminant validity. Conclusion. - The results showed satisfactory psychometric qualities of the PSI-NMD (Physical Self Inventory-Neuromuscular disease). This scale assessing global and physical self-esteem, is quick and simple to answer, and will be useful to clinicians as well as researchers. (c) 2022 Elsevier Masson SAS. All rights reserved.

Purpose (the aim of the study): We performed a systematic review with individual participant data (IPD) meta-analysis (MA) of randomized controlled trials (RCTs) investigating mechanical devices to treat knee OA. We aimed to identify subgroup characteristics that moderate treatment effects.

L'inventaire du soi physique-maladies neuromusculaires (ISP-MNM) est une échelle évaluant l'estime globale de soi et le soi physique auprès de patients atteints d'une maladie neuromusculaire. Elle est issue de l'inventaire du soi physique-10 items (ISP-10) lui-même issu de l'Inventaire du soi physique-25 items, questionnaire validé auprès de la population française. Étudier les qualités psychométriques de l'Inventaire du Soi Physique-10 items (ISP-10) chez des personnes atteintes d'une maladie neuromusculaire. Cent huit personnes atteintes de myopathie ou de neuropathie (âge : 49,2 ± 15 ans) et 129 personnes d'un échantillon contrôle (âge : 42,5 ± 15,8) ont été invitées à remplir l'ISP-10 et l'échelle d'estime de soi de Rosenberg. L'analyse factorielle exploratoire a permis de retenir une solution en 2 facteurs, composée de 8 items, et expliquant 65,4 % de la variance totale. De plus, les résultats indiquent des indices de consistance interne, de validité convergente et discriminante satisfaisants. Cette étude exploratoire montre des qualités psychométriques satisfaisantes du questionnaire renommé ISP-MNM (inventaire du soi physique-maladies neuromusculaires) pour évaluer l'estime globale de soi et physique chez les personnes souffrant de myopathie ou de neuropathie. La fidélité test-retest est encore à évaluer. Rapide, simple de passation, et sensible, l'échelle se révèle utile aux cliniciens et aux chercheurs qui travaillent avec cette population. The Physical Self Inventory-NeuroMuscular Diseases (PSI-NMD) is a scale which evaluates overall self-esteem and physical esteem in patients with neuromuscular disease. It comes from the Physical Self Inventory-10 item (PSI-10), which comes from the Physical Self Inventory -25 items, a validated questionnaire for French population. The purpose of this study is to present the first step of the validation of this scale in patients with neuromuscular diseases. This scale assesses the overall self-esteem as well as the self-esteem in physical field. In all, 108 patients with myopathy or neuropathy and 129 individuals from the general population answered to the ISP-10 and the Rosenberg's self-esteem scale. Exploratory factorial analysis showed the weakness of two items, which have been deleted. In addition, results indicated good indices of internal consistency, convergent and discriminant validity. The results showed satisfactory psychometric qualities of the PSI-NMD (Physical Self Inventory-Neuromuscular disease). This scale assessing global and physical self-esteem, is quick and simple to answer, and will be useful to clinicians as well as researchers.

Bellicha, A, Giroux, C, Ciangura, C, Menoux, D, Thoumie, P, Oppert, J-M, and Portero, P. Vertical jump on a force plate for assessing muscle strength and power in women with severe obesity: reliability, validity, and relations with body composition. J Strength Cond Res 36(1): 75-81, 2022-Muscle strength and power, particularly when assessed during multijoint movements such as vertical jump (VJ), are important predictors of health status and physical function. Vertical jump is mainly used in athletes, also in untrained or older adults, but has not yet been used in subjects with obesity. We aimed to assess the following in this population: (a) the reliability of VJ parameters, (b) their validity compared with isokinetic testing, and (c) their relations with body composition. In 20 women with severe obesity (mean [SD] age: 41.1 [11.6] years; body mass index: 43.9 [4.4] kg·m-2) without severe orthopedic disorders, VJ parameters, knee extension torque, and body composition were assessed using a force plate, an isokinetic dynamometer, and dual-energy x-ray absorptiometry, respectively. Excellent reliability was found for absolute peak power and peak force in VJ (intraclass correlation coefficient [95% confidence interval]: 0.95 [0.88-0.98] and 0.90 [0.77-0.96], respectively), and moderate to good validity of peak power and peak force compared with isokinetic torque (r = 0.79 and r = 0.67, respectively; all p < 0.01). Positive relations were found between peak force and peak power during VJ and lean body mass (r = 0.89 and r = 0.60, respectively; p < 0.01) and a negative relation was found between peak velocity or VJ height and fat mass (r = -0.65 and -0.64, respectively; p < 0.01). These results suggest that VJ on a force plate is a reliable and valid test for assessing muscle strength and power in severely obese subjects. Vertical jump testing is easy to implement, which can facilitate its use in both research and clinical testing in this setting (ClinicalTrials.govID: NCT03325764).

Purpose: Treatment for knee osteoarthritis (OA) may aim to alter biomechanics and reduce joint loads through the application or wear of mechanical interventions like bracing, taping, insoles, footwear, or mobility aids. The OARSI guidelines conditionally recommend use of mobility aids and wedged insoles for knee OA management, but conditionally to strongly recommend against taping and bracing, based on a lack of clear efficacy from low quality evidence. However, knee OA is a heterogeneous condition, and it may be that clearly defined subgroups of patients may be more likely to respond to mechanical interventions.We are conducting a systematic review with individual participant data (IPD) meta-analysis of randomized controlled trials (RCTs) investigating mechanical interventions to treat painful knee OA.

The foot-sole cutaneous receptors (section 2), their function in stance control (sway minimisation, exploratory role) (2.1), and the modulation of their effects by gait pattern and intended behaviour (2.2) are reviewed. Experimental manipulations (anaesthesia, temperature) (2.3 and 2.4) have shown that information from foot sole has widespread influence on balance. Foot-sole stimulation (2.5) appears to be a promising approach for rehabilitation. Proprioceptive information (3) has a pre-eminent role in balance and gait. Reflex responses to balance perturbations are produced by both leg and foot muscle stretch (3.1) and show complex interactions with skin input at both spinal and supra-spinal levels (3.2), where sensory feedback is modulated by posture, locomotion and vision. Other muscles, notably of neck and trunk, contribute to kinaesthesia and sense of orientation in space (3.3). The effects of age-related decline of afferent input are variable under different foot-contact and visual conditions (3.4). Muscle force diminishes with age and sarcopenia, affecting intrinsic foot muscles relaying relevant feedback (3.5). In neuropathy (4), reduction in cutaneous sensation accompanies the diminished density of viable receptors (4.1). Loss of foot-sole input goes along with large-fibre dysfunction in intrinsic foot muscles. Diabetic patients have an elevated risk of falling, and vision and vestibular compensation strategies may be inadequate (4.2). From Charcot-Marie-Tooth 1A disease (4.3) we have become aware of the role of spindle group II fibres and of the anatomical feet conditions in balance control. Lastly (5) we touch on the effects of nerve stimulation onto cortical and spinal excitability, which may participate in plasticity processes, and on exercise interventions to reduce the impact of neuropathy.

In France, the epidemic phase of COVID-19 caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) began in February 2020 and resulted in the implementation of emergency measures and a degradation in the organization of neuromuscular reference centers. In this special context, the French Rare Health Care for Neuromuscular Diseases Network (FILNEMUS) has established guidance in an attempt to homogenize the management of neuromuscular (NM) patients within the French territory. Hospitalization should be reserved for emergencies, the conduct of treatments that cannot be postponed, check-ups for which the diagnostic delay may result in a loss of survival chance, and cardiorespiratory assessments for which the delay could be detrimental to the patient. A national strategy was adopted during a period of 1 to 2months concerning treatments usually administered in hospitalization. NM patients treated with steroid/immunosuppressants for a dysimmune pathology should continue all of their treatments in the absence of any manifestations suggestive of COVID-19. A frequently asked questions (FAQ) sheet has been compiled and updated on the FILNEMUS website. Various support systems for self-rehabilitation and guided exercises have been also provided on the website. In the context of NM diseases, particular attention must be paid to two experimental COVID-19 treatments, hydroxycholoroquine and azithromycin: risk of exacerbation of myasthenia gravis and QT prolongation in patients with pre-existing cardiac involvement. The unfavorable emergency context related to COVID-19 may specially affect the potential for intensive care admission (ICU) for people with NMD. In order to preserve the fairest medical decision, a multidisciplinary working group has listed the neuromuscular diseases with a good prognosis, usually eligible for resuscitation admission in ICU and, for other NM conditions, the positive criteria suggesting a good prognosis. Adaptation of the use of noninvasive ventilation (NIV) make it possible to limit nebulization and continue using NIV in ventilator-dependent patients.

Aim: to evaluate the impact of rehabilitation programs for patients with neuromuscular diseases, particularly the effect of balance training on self-esteem. Method: 22 participants, of whom 7 patients were suffering from peripheral neuropathy and 15 from myopathy. At the beginning and at the end of the rehabilitation program focused on balance rehabilitation, self-esteem was assessed with Rosenberg's self-esteem questionnaire. Balance was assessed independently by the physiotherapist using Berg's scale. Results: after 6 weeks of multidisciplinary rehabilitation in a daily hospital routine which proved to be effective on the balance settings, we observed a parallel significant improvement in self-esteem. Conclusion: this first research shows that physical rehabilitation focused on balance rehabilitation of patients suffering from a neuromuscular disorder not only improves posture and gait but also has effects on self-esteem, thus deserving further and more in-depth research.

Objective: The aim of this study was to examine the association of childhood sexual and physical abuse with disability in adulthood, and to assess how several demographic, physical, behavioral, psychosocial, and psychiatric factors may influence this association. Methods: This study used nationally representative cross-sectional data from 7403 people aged >= 16 years who participated in the 2007 Adult Psychiatric Morbidity Survey. Information on childhood sexual talk, sexual touching, sexual intercourse, and physical abuse occurring before the age of 16, and disability in activities of daily living and instrumental activities of daily living in adulthood were collected. Multivariable logistic regression analyses were conducted. Results: After adjusting for age, sex, and ethnicity, we found a positive association between different types of childhood abuse and adulthood disability: sexual talk (OR 1.54; 95% CI 1.27-1.85); sexual touching (OR 1.82; 95% CI 1.49-2.22); sexual intercourse (OR 2.58; 95% CI 1.75-3.81); physical abuse (OR 2.84; 95% CI 2.20-3.68). Increasing number of types of childhood abuse was associated with increased odds of adulthood disability. The odds of adulthood disability was increased for individuals who experienced all types of childhood abuse versus no childhood abuse (OR 3.59; 95% CI 1.64-7.84). Finally, the association between any childhood abuse and adulthood disability was largely explained by anxiety disorder, number of chronic physical conditions, and loneliness. Conclusions: Childhood abuse is positively associated with adulthood disability in England. Future longitudinal studies are warranted to understand the potentially complex interplay of factors that may increase risk for disability in individuals who experienced childhood abuse. (C) 2019 Elsevier Masson SAS. All rights reserved.

Background: No study has investigated the association between intelligence quotient (IQ) and disability (i.e., difficulties in activities of daily living [ADL] or instrumental activities of daily living [IADL]) in the general population. Objective: The goal of this nationally representative study was to analyse the potential IQ-disability association in England and identify influential factors in this association. Methods: Cross-sectional data were analyzed from the 2007 Adult Psychiatric Morbidity Survey (n = 6872). IQ was assessed by using the National Adult Reading Test, which consists of a list of 50 words and is scored by counting the number of errors in reading the words aloud. Disability was defined as difficulties in at least 1 of the 7 domains of ADL and IADL. Regression and mediation analyses were conducted to analyze the association between IQ and disability and identify potential factors involved in this relationship, estimating odds ratios (ORs) and 95% confidence intervals (CIs). Results: Among the 6872 participants, the mean (SD) age was 46.9 (18.9) years; 51.8% were women. The prevalence of disability increased from 27.7% with IQ 120-129 to 51.0% with IQ 70-79. After adjusting for sex, age and ethnicity, as compared with IQ 120-129, with IQ 110-119, 100-109, 90-99, 80-89, and 70-79, the probability of disability was increased (OR 1.22 [95% CI 1.01-1.48], 1.42 [1.16-1.72], 1.86 [1.54-2.25], 2.41 [1.92-3.03], and 4.71 [3.56-6.17], respectively). In addition, we found a positive association between a 1-SD decrease in IQ and disability (OR 1.53, 95% CI 1.43-1.63). Finally, income (mediated percentage 26.9%), social class (18.0%) and education (11.6%) strongly affected the IQ-disability association, and these socioeconomic factors collectively explained 37.1% of the association. Conclusions: Low IQ was positively associated with disability in England, and socioeconomic status explained more than one-third of this relationship. Crown Copyright (C) 2019 Published by Elsevier Masson SAS. All rights reserved.

We aimed to determine patient acceptable symptom state (PASS) and minimally clinically important improvement (MCII) estimates for patient-reported outcomes (PROs) in systemic sclerosis (SSc). Two hundred and twenty patients participated in the SCLEREDUC trial, a 12-month, randomised controlled study comparing the efficacy on disability assessed by the HAQ-DI at 12-months of physical therapy compared to usual care in patients with SSc. Self-rated state and change in patient health at 12 months post-randomization were assessed by using 2 external anchoring questions from the Medical Outcomes Study 36-Item Short Form. Patients who self-rated their health as "excellent", "very good" or "good" were considered as having an acceptable symptom state and those who self-rated their health change at 12 months as "somewhat better" were considered as minimally improved. PASS estimates were obtained using the 75th percentile method and the MCII estimates by using the mean change in scores method. Answers to both anchoring questions at 12 months were available for 151/220 (68.6%) participants. PASS (95% CI) and mean (SD) MCII estimates at 12 months were respectively: 53.75 (34.00 to 68.00) and −6.74 (32.02) for the joint-pain visual analog scale (range 0–100), 1.41 (1.13 to 1.63) and −0.21 (0.48) for the Health Assessment Questionnaire Disability Index (HAQ-DI, range 0–3), 1.27 (1.07 to 1.62) and −0.13 (0.45) for the scleroderma HAQ (sHAQ, range 0–3), 26.00 (17.00 to 37.00) and −3.38 (9.87) for the Cochin Hand Function Scale (range 0–90), and 19.40 (17.20 to 21.90) and −5.69 (6.79) for the McMaster-Toronto Arthritis Patient Preference Disability Questionnaire (range 0–30). For all PROs, higher scores indicate higher symptoms levels. The present study provides PASS and MCII estimates for commonly used PROs in SSc. These estimates are useful in interpreting the clinical relevance of outcomes used in patients' care and in clinical trials. ClinicalTrials.gov identifier: NCT00318188.

Les adultes atteints de paralysie cérébrale (PC), hémiplégiques ou diplégiques, chutent souvent par accrochage du pied en rotation interne. Nous formulons l’hypothèse que ces patients compensent par une augmentation du contrôle de la marche que nous proposons d’évaluer lors du traitement de la spasticité par injection de toxine dans le chef antérieur du moyen fessier. Neuf patients ont été suivis sur une période de cinq semaines selon trois modalités d’évaluation de la marche sur tapis de marche GAITRite® (vitesse normale, rapide et double tâche). L’impact de l’injection de toxine botulinique a été mesuré à partir des modifications de la vitesse de marche, de la rotation du pied et d’un index composite le Functional Ambulation Performance score (FAP). En vitesse standard, l’index FAP est diminué chez 2 patients, augmenté chez 5 et stable chez 2 patients : en moyenne, 86,3 ± 5,9 à J0 et 84,9 ± 8,8 à S5 (p = 0,57). En vitesse rapide, l’index est diminué chez quatre patients, augmenté chez deux et stable chez trois patients : 86,3 ± 9,9 et 83,1 ± 10,6 (p = 0,34). En double tâche, l’index est diminué chez un patient, augmenté chez sept et stable chez un patient avec une différence significative : 80,06 ± 11,4 et 87,1 ± 5,2 (p = 0,02). Aucune modification significative n’a été mesurée concernant les autres paramètres cinématiques. Cette étude a montré que la toxine botulinique augmentait la performance de la marche en double tâche des patients adultes atteints de paralysie cérébrale.

Evidence is still inconclusive for the benefits of bracing in patients with knee osteoarthritis. To assess the effect of REBEL RELIEVER unloading knee brace in conservative treatment of knee osteoarthritis, a randomized controlled trial was conducted in 67 patients with symptomatic medial knee osteoarthritis, who randomly received 6-week treatment with either REBEL RELIVER unloading knee brace + usual care (Brace group, N = 32) or usual care alone (Control group, N = 35). Primary outcome was the global last 24h-pain relief (100-mm visual analogic scale [VAS]) at 6 weeks. Secondary endpoints included pain on motion (100-mm VAS), function (Lequesne index), safety and observance. At 6 weeks, mean [SD] last 24h-pain decreased significantly more in Brace group versus Control group (−41.35 [3.37] vs −15.37 [3.23], difference −25.98, 95% CI −41.64 to −10.33, P < 0.0001). Higher mean [SD] pain on motion decrease (−51.91 [3.49] vs −19.91 [3.34], difference −32.01, 95% CI −48.21 to −15.80, P < 0.0001) and better improvement of Lequesne index score (−5.8 [0.5] vs −2.3 [0.5], difference −3.5, 95% CI −5.0 to −2.0, P < 0.0001) were observed in Brace group. Safety and observance to the brace were excellent. The additive clinical benefit of wearing REBEL RELIEVER unloading knee brace was demonstrated in knee osteoarthritis patients.

Can muscle or myofascial pain in muscular dystrophies be relieved by muscle-centred acupuncture? After a discussion of the key notions of "myofascial trigger points" and specific acupuncture needle manipulations, we report some complex clinical cases among patients with NeuroMuscular Diseases (NMD) and Charcot Marie Tooth (CMT) in whom myofascial pain could not be controlled by drug treatments and/or physical techniques. They were seen on outpatient basis between 2010 and 2017 at the Rothschild Hospital (Paris), in the Physical and Rehabilitation Medicine (PRM) department. After the initial medical appointment, several care strategies were proposed, including neuro-acupuncture performed by a PRM physician. This department was linked to the Expert Center for Rare NMD "Paris-Nord-Est-Ile-de- France"

The aim of this research was to investigate the physical functioning of patients with myopathy and to measure its influence on quality of life (QoL). Previous studies have observed a lower QoL in myopathy than in control patients but its relationship with physical functioning as not yet been evaluated. We hypothesized that motor abilities evolution would predict a lower physical and psychological QoL. Twenty-five patients with adult-form myopathy were included: 14 patients with myotonic dystrophy type 1, 6 with facioscapulohumeral dystrophy, 2 with limb girdle muscular dystrophy, 1 with mitochondrial myopathy, 1 with Central Cores myopathy, 1 with Ullrich muscular dystrophy (population description in Table 1, n = 25). They all answered two questionnaires of QoL: Medical Outcomes Short-Form 36 (SF-36) which gives a physical and a mental score and the Quality of Life of genetics neuromuscular diseases (QoL-gNMD) which gives three under-scores: Body symptom, Self-perception, Activity and participation. The physical functioning was evaluated with two measures by physiotherapists: balance with the Berg Scale and walking range with the 6 Minute Walk Test. Linear regression analysis showed the 6 Minute Walk Test was significantly predictor of the three scores of QoL-gNMD and the physical score of SF-36. The under-score "Activity and Participation" was also predicted by a balance trouble (Table 2). None independent variable was linked with the mental score of SF-36. Results reveal the negative influence of the physical function lost on QoL, especially the walking range. Effects are observed in body aspects like symptom or social relationship as well as deeper psychological aspects as the self-perception. So, it appears very important to take into account the motor abilities evolution of each patient in order to recommend an adapted rehabilitation, and if necessary, a psychological support to optimally preserve their quality of life.

Gait with internal hip rotation is often observed in adult with cerebral palsy both in diplegic and hemiplegic forms. Walking disabilities could be the consequence of gluteus medius anterior fibers spasticity. A monocentric prospective pilot study was to assess the clinical and paraclinical progression of hip internal rotation, velocity and Functional Ambulation Performance (FAP) score index. We included eight patients (nine hips) with a follow-up to five weeks. Clinical (questionnaire, spasticity) and instrumental (walk on "GAITRite®") assessments were performed at the beginning and at the end of the study. The FAP was significantly higher (10%) after botulinum toxin (80 to 87, P < 0.05). There was no significant impact on internal rotation and velocity. Patients felt better with a fluider gait and less fall. Botulinum toxin improved gait performances in adult with cerebral palsy and gluteus medius anterior fibers spasticity. A randomized prospective multicentric study is required to confirm the preliminary results and to assess the impact of quality of life.

Research exploring the effects of physical exercise in auto-immune myasthenia gravis (MG) is scarce. The few existing studies present methodological shortcomings limiting the conclusions and generalisability of results. It is hypothesised that exercise could have positive physical, psychological as well as immunomodulatory effects and may be a beneficial addition to current pharmacological management of this chronic disease. The aim of this study is to evaluate the benefits on perceived quality of life (QOL) and physical fitness of a home-based physical exercise program compared to usual care, for patients with stabilised, generalised auto-immune MG.

Proprioceptive impairment, as in the case of ataxic neuropathy, leads to visual compensation. The techniques of gaze analysis in static condition have been widely explored, but only recent technological innovations have allowed them to be associated with dynamic movements. In this context, we chose to use the eye-tracker glasses to analyze gaze and possible visual strategies established by these patients during gait. Three groups were selected: 15 health young subjects, 15 elderly subjects and 15 subjects with ataxic neuropathy. The experimental protocol consists of walk in a corridor with three different segments wearing the eye-tracker glasses. As for the gaze direction analysis we pre-defined classifications of the image called "observables": these classifications consists on the time spent looking at the floor, the middle and the side parts of the corridor. We observed a significant difference between the two groups of health subjects and the patients regarding the time spent looking at the "observables". Fisher's post-hoc results were: floor (F 2.132 = 37; P ≤ .0001); middle (F 2.132 = 9,8; P ≤ .0001) and the side parts (F 2.132 = 11,5; P ≤ .0001). We observed a better reproducibility of the evaluation in the three different segments by analyzing the time spent looking at the floor (F 4.84 = 1.4; P = .244), where no significant statistical difference on the performance of the subjects were found. The eye-tracker glasses have proven to be a relevant tool for the analysis of visual compensation, both reproducible and sensitive to the pathology. This technology has helped us to quantitatively characterize the visual strategy implemented by ataxic patients during gait.