We describe a long-term observational study of a large cohort of patients with sporadic inclusion body myositis and propose a sporadic inclusion body myositis weakness composite index that is easy to perform during a clinic. Data collection from two groups of patients (Paris and Oxford) was completed either during a clinic visit (52%), or by extraction from previous medical records (48%). One hundred and thirty-six patients [57% males, 61 (interquartile range 55-69) years at onset] were included. At the last visit all patients had muscle weakness (proximal British Medical Research Council scale <3/5 in 48%, distal British Medical Research Council scale <3/5 in 40%, swallowing problems in 46%). During their follow-up, 75% of patients had significant walking difficulties and 37% used a wheelchair (after a median duration from onset of 14 years). The sporadic inclusion body myositis weakness composite index, which correlated with grip strength (correlation coefficient: 0.47; P < 0.001) and Rivermead Mobility Index (correlation coefficient: 0.85; P < 0.001), decreased significantly with disease duration (correlation coefficient: -0.47; P < 0.001). The risk of death was only influenced by older age at onset of first symptoms. Seventy-one (52%) patients received immunosuppressive treatments [prednisone in 91.5%, associated (in 64.8%) with other immunomodulatory drugs (intravenous immunoglobulins, methotrexate or azathioprine) for a median duration of 40.8 months]. At the last assessment, patients who had been treated were more severely affected on disability scales (Walton P = 0.007, Rivermead Mobility Index P = 0.004) and on the sporadic inclusion body myositis weakness composite index (P = 0.04). The first stage of disease progression towards handicap for walking was more rapid among patients receiving immunosuppressive treatments (hazard ratio = 2.0, P = 0.002). This study confirms that sporadic inclusion body myositis is slowly progressive but not lethal and that immunosuppressive treatments do not ameliorate its natural course, thus confirming findings from smaller studies. Furthermore, our findings suggest that immunosuppressant drug therapy could have modestly exacerbated progression of disability. The sporadic inclusion body myositis weakness composite index might be a valuable outcome measure for future clinical trials, but requires further assessment and validation.
Aims The study aimed to determine the opinions of individuals with neurological conditions on factors facilitating their physical activity participation. Methods Four condition-specific focus groups (muscular dystrophy, multiple sclerosis, motor neurone disease and Parkinson's disease) were run with a total of 24 people. Themes that emerged were used to create an eight-item self-completed questionnaire, which explored barriers to participation, preferred activities and support networks. A cross-sectional survey was then conducted using this questionnaire with individuals with a range of neurological conditions. Findings Three themes categories emerged from the focus groups: ‘opinions of physical activity’, ‘barriers to physical activity’, and ‘factors that would encourage increased physical activity involvement’. Of the 115 distributed questionnaires, 80 (70%) responses were received. Swimming, stretching and walking were the three most popular activities. The most common barriers were embarrassment, perceived lack of condition-specific knowledge of the fitness professionals about neurological disease and the impact of that on exercise advice. Facilitators were use of specific groupbased exercise sessions and the presence of specifically trained staff. Conclusions People with neurological conditions enjoy participating in physical activity but reported several barriers that prevent their participation in exercise. Respondents identified barriers and facilitators in functional, psychological and environmental domains. It is important that healthcare professionals and fitness professionals identify and remove these barriers to promote greater participation in exercise among people with neurological conditions.
People with long-term neurological conditions can safely exercise in community gyms when supported and achieve similar attendance to standard exercise referral schemes, but may reduce other life activities in order to participate at a gym.
Aim: To investigate the feasibility and effect of a home-based exercise programme on walking endurance, muscle strength, fatigue and function in people with neuromuscular disorders (NMDs). Methods: 20 adults with NMDs recruited to a control (n = 11) or exercise (n = 9) group were assessed by blinded assessors at baseline and at week 8. Walking and strengthening exercises were given to the exercise group in an 8-week home exercise programme. A 2-min walk distance was the main outcome measurement; isometric muscle strength, fatigue and function were secondary measurements. 1 Regular exercise leads to health and social benefits even in people with disease.2 The limited clinical research on adults with NMD suggests that they benefit from targeted aerobic and muscle training exercise programmes. These interventions may be effectively provided in the community setting 5 6 and are more effective if supported by a therapist. 7Patients with NMD in the UK receive specialist support from regional centres, which may be located some distance from their home. We developed a training programme that could be delivered with a single demonstration of exercises in the clinic, with follow-up support delivered through a leaflet and by telephone. We carried out a pilot investigation on the effect of an exercise programme on walking distance, specifically developed for the treatment of a range of NMDs in adults. We also investigated the effect on fatigue, isometric muscle strength and performance, and perceived ability in targeted functional activities. METHODParticipants were adults >16 years old, diagnosed with primary muscle disease and with an abnormal gait pattern attending a regional neuromuscular clinic, or those with a 10 m time exceeding the normal age-related time by >2 s, but able to walk 10 m without physical help (aids were permitted). People who had physical, cognitive, sensory or psychological impairments, or other conditions precluding full engagement with the experimental paradigm, were excluded. Informed consent was obtained before participation.On first assessment, the following measurements were obtained:
ObjectiveTo describe decline in muscle strength and physical function in patients with sporadic inclusion body myositis (IBM).MethodsManual muscle testing (MMT), quantitative muscle testing (QMT) and disability scoring using the IBM Functional Rating Scale (IBMFRS) were undertaken for 181 patients for up to 7.3 years. The relationship between MMT, QMT and IBMFRS composite scores and time from onset were examined using linear mixed effects models adjusted for gender and age of disease onset. Adaptive LASSO regression analysis was used to identify muscle groups that best predicted the time elapsed from onset. Cox proportional hazards regression was used to evaluate time to use of a mobility aid.ResultsMultilevel modelling of change in percentage MMT, QMT and IBMFRS score over time yielded an average decline of 3.7% (95% CI 3.1% to 4.3%), 3.8% (95% CI 2.7% to 4.9%) and 6.3% (95% CI 5.5% to 7.2%) per year, respectively. The decline, however, was not linear, with steeper decline in the initial years. Older age of onset was associated with a more rapid IBMFRS decline (p=0.007), but did not influence the rate of MMT/QMT decline. Combination of selected muscle groups allowed for generation of single measures of patient progress (MMT and QMT factors). Median (IQR) time to using a mobility aid was 5.4 (3.6–9.2) years, significantly affected by greater age of onset (HR 1.06, 95% CI 1.04 to 1.09, p<0.001).ConclusionThis prospective observational study represents the largest IBM cohort to date. Measures of patient progress evaluated in this study accurately predict disease progression in a reliable and useful way to be used in trial design.
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