An expert opinion: Rehabilitation options for people with polyneuropathy

Polyneuropathies encompass a number of diseases of the peripheral nerves. Causes vary, and some types of polyneuropathy have a chronic disease course, and some have acute onset. Polyneuropathies, such as Guillain Barré Syndrome, improve after acute deterioration, but some people are left with longer term disability. Physical management to manage longer term impairments and maintain function, are gathering evidence of efficacy, though trials remain small in number and size. Exercise trials show some positive improvements in proximal muscle strength, aerobic capacity, balance and distal strength in children. People with polyneuropathy often present with distal weakness and joint malalignment. Orthotics interventions may address these impairments and can vary from ankle foot orthoses to insoles. Weakness and sensory impairment also lead to altered hand function which can be addressed by adaptive strategies and exercise. People with polyneuropathies can beneft from rehabilitation interventions through addressing the impact of impairments and compensatory strategies necessary for optimised function

Physical Activity During and After Haematological Cancer Treatment: A Cross-Sectional Survey of Haematology Healthcare Professionals in the United Kingdom

Purpose: Health professionals’ (HPs) knowledge of recommended guidelines for physical activity (PA) is thought to influence the advice they provide to their patients. Little is known about the knowledge or provision of PA advice by HPs working with haematological cancer patients. This study examined awareness of PA guidance, beliefs and practices in provision of advice given by UK HPs working with haematological cancer patients. Methods: Online survey including questions on awareness of PA guidance, levels of agreement/disagreement with statements related to PA in haematological cancer and reported provision of advice in practice. Open text responses sought detail regarding guidance knowledge and exampled advice given by respondents. Predictors of familiarity of guidance and provision of advice were examined. Results: Complete responses were received from 156 professionals, mostly nurses, allied HPs and doctors. Many (31%) reported knowing relevant guidance and nearly half (48.6%) reported routinely giving PA advice. Nurses and allied AHPs give advice to more patients than doctors and knowledge of guidelines among doctors was poor. Conclusion: Beliefs of haematology professionals regarding the role of PA during and after treatment for haematological cancer were generally positive. Those reporting familiarity with guidance were more likely to give advice. Misalignment exists between guidelines and advice given by professionals to their patients. Increasing knowledge of guidelines among HPs, including nurses, may lead to increased provision of PA advice and promotion of PA to more of their patients. HPs education in haematology on PA guidance tailored to professional group is needed

PERCEPT myeloma: a protocol for a pilot randomised controlled trial of exercise prehabilitation before and during autologous stem cell transplantation in patients with multiple myeloma

INTRODUCTION: Myeloma, a blood cancer originating from plasma cells, is the most common indication for autologous stem cell transplantation (SCT). Patients with myeloma undergoing autologous SCT (ASCT) experience treatment-related morbidity and reduction in function and well-being for many months post-treatment. Interventions targeting physical functioning delivered prior to and during SCT have shown promising results in mixed haematological populations and may offer a non-pharmacological solution to physically optimising and preparing patients for SCT. The aim of this study is to investigate the feasibility of a physiotherapist-led exercise intervention as an integral part of the myeloma ASCT pathway at a UK tertiary centre. METHODS AND ANALYSIS: PERCEPT is a single-site, pilot randomised controlled trial of an exercise intervention embedded within the myeloma ASCT pathway, compared with usual care. The primary study end points will be feasibility measures of study and intervention delivery including recruitment rates, acceptability of intervention, study completion rate and any adverse events. Secondary end points will evaluate differences between the exercise intervention group and the usual care control group in cancer-related fatigue, quality of life, functional capacity (6 min walk test; handheld dynamometry; a timed sit-to-stand test) and objective and self-reported physical activity. Outcomes will be assessed at four time points, approximately 6-8 weeks prior to SCT, on/around day of SCT, on discharge from SCT hospital admission and 12 weeks post-discharge. The exercise intervention comprises of partly supervised physiotherapist-led aerobic and resistance exercise including behaviour change techniques to promote change in exercise behaviour. The primary outcomes from the trial will be summarised as percentages or mean values with 95% CIs. Group differences for secondary outcomes at each time point will be analysed using appropriate statistical models. ETHICS AND DISSEMINATION: This study has NHS REC approval (Camden and Kings Cross, 19/LO/0204). Results will be disseminated through publication and presentations at haematology and rehabilitation-related meetings. TRIAL REGISTRATION NUMBER: ISRCTN15875290

Orthotic management of instability of the knee related to neuromuscular and central nervous system disorders: qualitative interview study of patient perspectives

Objectives: Adults with knee instability related to neuromuscular disorders or central nervous conditions often experience mobility problems and rely on orthoses to improve function and mobility. Patient views of device effectiveness and acceptability are underexplored. Our study aimed to elicit device users’ perspectives regarding fitting, acceptability, effectiveness and use of orthoses, and identify important treatment outcomes. / Design: Qualitative descriptive study using in-depth semistructured interviews. Interview transcriptions were coded and thematically analysed, using ‘Framework’. / Setting and participants: A purposive sample of 24 adult users of orthotic devices. Nineteen patients were recruited across three National Health Service sites, and five people through charities/patient support groups in England. Half of the participants had been diagnosed with poliomyelitis, and the remainder with multiple sclerosis, Charcot-Marie-Tooth disease, spinal injury or spina bifida, and stroke. The median age of participants was 64.5 years (range 36–80 years). / Results: Patients’ medical condition impacted significantly on daily life. Participants relied on orthotic devices to enable engagement in daily activities. Patient goals for mobility were linked to individual circumstances. Desired treatment outcomes included reduction in pain, trips and falls, with improved balance and stability. Effectiveness, reliability, comfort and durability were the most valued features of orthoses and associated with reported use. Obtaining suitable footwear alongside orthotic devices was a significant concern. Time pressures during device fitting were viewed negatively. / Conclusions: Orthotic devices for knee instability play a crucial role in promoting, maintaining and enhancing physical and psychological health and well-being, enabling patients to work, engage in family life and enjoy social activities. Future research should consider how best to measure the impact of orthotic devices on patient quality of life and daily functioning outside the clinic setting, as well as device use and any adverse effects. / Trial registration number: This qualitative study was retrospectively registered as Current Controlled Trials ISRCTN65240228

Use of Twitter in Neurology: Boon or Bane?

Twitter is a free, open access social media platform that is widely used in medicine by physicians, scientists, and patients. It provides an opportunity for advocacy, education, and collaboration. However, it is likely not utilized to its full advantage by many disciplines in medicine, and pitfalls exist in its use. In particular, there has not been a review of Twitter use and its applications in the field of neurology. This review seeks to provide an understanding of the current use of Twitter in the field of neurology to assist neurologists in engaging with this potentially powerful application to support their work

Physical activity interventions and therapeutic exercise in adults with rare neurological disorders: development of a core outcome measure set

måsjekke

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

Community exercise is feasible for neuromuscular diseases and can improve aerobic capacity

Objective: The aim of this phase 2 trial was to ascertain the feasibility and effect of community based aerobic exercise training for people with two of the more common neuromuscular diseases: Charcot-Marie-Tooth disease type 1A (CMT) and Inclusion Body Myositis (IBM). Methods: A randomised single blinded cross over trial design was used to compare a 12-week aerobic training programme using recombinant exercise bicycles compared to a control period. The training occurred three times per week in community gyms local to the participants. Support was available from trained gym staff and a research physiotherapist. The two disease groups were analysed separately. The primary outcome measure was peak oxygen uptake (VO2 peak) during a maximal exercise test, with secondary measures of muscle strength, function and patient reported measures. Results: Data from 23 people with CMT and 17 people with IBM was included in the analysis. Both disease groups had high levels of participation and demonstrated improvements in VO2 peak, with a moderate effect size in the CMT participants (Cohen’s d = 0.53) and a strong effect size in the IBM group (Cohen’s d = 1.72). No major changes were observed in the secondary outcome measures. Qualitative interviews revealed that participants valued the support of gym instructors and the research physiotherapists in overcoming challenges to participation. Conclusion: Twelve weeks of aerobic training in community gyms was feasible, safe and improved aerobic capacity in people with CMT and IBM. Classification of Evidence: This study provides Class II evidence that for patients with CMT type 1A and IBM, an aerobic training program increases aerobic capacity

X inactivation in females with X-linked Charcot-Marie-Tooth disease.

X-linked Charcot-Marie-Tooth disease (CMT1X) is the second most common inherited neuropathy, caused by mutations in gap junction beta-1 (GJB1). Males have a uniformly moderately severe phenotype while females have a variable phenotype, suggested to be due to X inactivation. We aimed to assess X inactivation pattern in females with CMT1X and correlate this with phenotype using the CMT examination score to determine whether the X inactivation pattern accounted for the variable phenotype in females with CMT1X. We determined X inactivation pattern in 67 females with CMT1X and 24 controls using the androgen receptor assay. We were able to determine which X chromosome carried the GJB1 mutation in 30 females. There was no difference in X inactivation pattern between patients and controls. In addition, there was no correlation between X inactivation pattern in blood and phenotype. A possible explanation for these findings is that the X inactivation pattern in Schwann cells rather than in blood may explain the variable phenotype in females with CMT1X