Night splinting does not increase ankle range of motion in people with Charcot-Marie-Tooth disease: a randomised, cross-over trial. Progressive resistance training in neuromuscular patients. Effects on force and surface EMG. Resistance training exercise and creatine in patients with Charcot-Marie-Tooth disease. Strength training in patients with myotonic dystrophy and hereditary motor and sensory neuropathy: a randomized clinical trial. A randomized controlled trial of early surgery in Duchenne muscular dystrophy. Effects of training and albuterol on pain and fatigue in facioscapulohumeral muscular dystrophy. Effects of exercise and creatine on myosin heavy chain isoform composition in patients with Charcot-Marie-Tooth disease. The effect of lip strengthening exercises in children and adolescents with myotonic dystrophy type 1. A randomized trial investigating an exercise program to prevent reduction of bone mineral density and impairment of motor performance during treatment for childhood acute lymphoblastic leukemia.
What is the effect of wearing splints at night to stretch the plantarflexors on dorsiflexion range of motion (ROM) in people with Charcot-Marie-Tooth disease?
                Randomised, assessor-blinded, cross-over trial.
                14 people (1 dropout) aged 7 to 30 years with Charcot-Marie-Tooth disease Type 1A and with < or = 15 degrees dorsiflexion range of motion (ROM).
                A splint holding the ankle in maximum dorsiflexion was worn nightly on one leg for 6 weeks followed by the opposite leg for the subsequent 6 weeks.
                The primary outcome was dorsiflexion ROM; secondary outcomes were eversion ROM, and dorsiflexion, eversion, and inversion strength, measured before and after splinting, and three months later.
                There was no significant difference between the experimental and the control intervention in terms of ROM or strength. Wearing the splint at night increased dorsiflexion ROM by 1 degree (95% CI -3 to 4; p = 0.72) and eversion ROM by 1 degree (95% CI -1 to 3; p = 0.28) compared to not wearing the splint. Wearing the splint increased dorsiflexion strength by 41 N (95% CI -53 to 135; p = 0.38), reduced eversion strength by 6 N (95% CI -112 to 101; p = 0.92) and reduced inversion strength by 8 N (95% CI -110 to 95; p = 0.88) compared to not wearing the splint.
                Wearing night splints does not increase ankle ROM or strength in people with Charcot-Marie-Tooth disease Type 1A. In a randomized clinical trial the efficacy of strength training was studied in patients with myotonic dystrophy (n = 33) and in patients with Charcot-Marie-Tooth disease (n = 29). Measurements were performed at the start and after 8, 16 and 24 weeks of progressive resistance training. Surface electromyography (SEMG) of proximal leg muscles was recorded during isometric knee extension at maximum voluntary contraction (MVC) and at 20, 40, 60 and 80% of MVC. Changes in MVC, maximum electrical activity and torque-EMG ratios (TER) were calculated. Fatigue was studied by determining the changes in endurance and in the decline of the median frequency (Fmed) of the SEMG during a sustained contraction at 80% MVC. These parameters showed no significant changes after the training in either of the diagnostic groups. Only the Charcot-Marie-Tooth training group showed a gradual significant increase in mean MVC over the whole training period (21%). After 24 weeks, the increase in mean RMS was similar (25%), but this was mainly due to a sharp rise during the first 8 weeks of training (20%). The findings indicate that the initial strength increase was due to a neural factor, while the subsequent increase was mainly due to muscle hypertrophy. Resistance exercise and creatine supplementation independently improve strength and function in patients with certain neuromuscular diseases. The purpose of this study was to examine the effects of resistance training with and without creatine supplementation on muscle, strength, and function in patients with Charcot-Marie-Tooth (CMT) disease. Twenty patients with CMT consumed 5 g/day creatine or placebo while participating in resistance training for 12 weeks. Energy metabolites, muscle fiber type and size, strength, and timed activities of daily living were measured before and after training. There were no differences between creatine or placebo groups for any outcome. For the groups combined, exercise training increased type I muscle fiber diameter (48.2 +/- 14.2 microm vs. 55.4 +/- 14.8 microm), strength, and activities of daily living (ADL) times. Thus, patients respond to resistance training with muscle fiber adaptations, and improvements in strength and function. Creatine was not beneficial.
                Copyright 2004 Wiley Periodicals, Inc. Muscle Nerve 30: 69-76, 2004 A randomized clinical trial on the effects of strength training was performed in myotonic dystrophy (MyD) patients and patients with hereditary motor and sensory neuropathy (HMSN). Training and most measurement tools involved the proximal lower extremity muscles. The participants trained 3 times a week for 24 weeks with weights adapted to their force. Strength was evaluated by isokinetically measured knee torque. Fatiguability was assessed by the time an isometric contraction could be sustained. Functional performance was measured by timed motor performance and by questionnaires on functional performance. Serum myoglobin (Mb) levels were determined to detect changes in muscle fiber membrane permeability. The MyD group included 33 participants, and the HMSN group included 29 participants. Within each diagnostic group, patients were individually matched and subsequently randomized for treatment allocation. In the MyD patients, none of the measurement techniques showed any training effect. Neither were there signs of deterioration caused by the training. In the HMSN group, knee torques increased. Timed motor performance did not change, although the questionnaires showed an improvement on items related to upper-leg function. Mb levels did not change significantly as a result of the training. In conclusion, the MyD group showed neither positive nor negative effects of the training protocol, whereas the training produced a moderate increase in strength and leg-related functional performance in the HMSN group. We performed a randomized controlled trial of early surgical treatment of contractures in 20 boys with Duchenne muscular dystrophy, age 4-6 yr. Surgery consisted of release of hip flexors, removal of iliotibial bands, and lengthening of tendo Achilles bilaterally. All patients were monitored for at least 12 months post-randomization, and assessed quantitatively for muscle strength and function. Surgery corrected the deformities, but had no beneficial effect on strength or function. Indeed, data in the second year showed more rapid deterioration of function in some of the operated boys. There appeared to be continued evolution of pathology following surgery, as assessed by sequential muscle ultrasound and muscle biopsy. We cannot recommend this type of surgery as a routine treatment. We recently reported a randomised controlled trial on the efficacy of strength training and the beta2-adrenergic agonist albuterol in patients with facioscapulohumeral muscular dystrophy (FSHD). Strength training and albuterol appeared safe interventions with limited positive effect on muscle strength and volume. We concurrently explored the prevalence and the characteristics of pain and fatigue in the participating FSHD patients, because these are probably underreported but clinically relevant symptoms in this disorder. Next, we studied the effects of albuterol and strength training on pain, experienced fatigue, health-related functional status and psychological distress.
                Sixty-five patients were randomised to strength training of elbow flexors and ankle dorsiflexors or non-training. After 26 weeks, albuterol (sustained-release, 8 mg bid) was added in a randomised, double-blind, placebo-controlled design. Outcomes comprised self-reported pain, experienced fatigue, functional status and psychological distress obtained with validated questionnaires at 52 weeks.
                Eighty percent of patients reported chronic persistent or periodic, multifocal pains. Thirty-four percent of the participants were severely fatigued. Strength training and albuterol failed to have a significant effect on all outcomes.
                Pain and fatigue are important features in FSHD. Strength training and albuterol do not have a positive or negative effect on pain, experienced fatigue, functional status and psychological distress. It is not known whether myosin heavy chain (MHC) content changes in response to exercise training or creatine supplementation in subjects with Charcot-Marie-Tooth disease (CMT). Based on previous data, we hypothesized that resistance exercise and creatine would increase the percentage of type I MHC composition in the vastus lateralis muscle and that myosin isoform changes would correlate with improved chair rise-time in CMT subjects. To test this hypothesis, 18 CMT subjects were randomly assigned to either a placebo or creatine group. All subjects performed a 12-week, home-based, moderate-intensity resistance training program. Chair rise-time was measured before and after the training program. Muscle biopsies were obtained from the vastus lateralis before and after the 12-week program. Gel electrophoresis showed a significant decrease (approximately 30%) in MHC type I in CMT subjects given creatine supplementation when compared with placebo. There was a nonsignificant increase in both MHC type IIa (approximately 23%) and MHC type IIx (approximately 7%) in CMT subjects given creatine. Reduced MHC type I content and increased MHC type IIa content correlated with faster chair rise-times (i.e., improved muscle performance). The training-induced change in MHC IIa content was inversely correlated with chair rise-time in CMT subjects given creatine. When the two subject groups were combined, there was a linear, negative relationship between the change in MHC type IIa content and chair rise-time after training and a positive relationship between the training-induced change in MHC type I content and chair rise-time. These data suggest that improved function (chair rise-time) was associated with a lower level of MHC type I and increased MHC type IIa composition. Furthermore, the data are consistent with the hypothesis that creatine supplementation alters MHC composition in CMT patients undergoing resistance training and that MHC changes associated with creatine supplementation can improve muscle function. Myotonic dystrophy type 1 (DM1) is a slowly progressive neuromuscular disease. Most children and adolescents with DM1 have weak lips and impaired lip function. The primary aim of the present study was to investigate if regular training with an oral screen could strengthen the lip muscles in children and adolescents with DM1. If lip strength improved, a secondary aim would be to see if this could have an immediate effect on lip functions such as lip mobility, eating and drinking ability, saliva control, and lip articulation.
                Eight school aged children and adolescents (7-19 years) with DM1 were enrolled in an intervention study with a single group counterbalanced design. After three baseline measurements four children (Subgroup A) were randomly chosen to start 16 weeks therapy while the others (Subgroup B) acted as controls without therapy. After 16 weeks the subgroups changed roles. During treatment the participants exercised lip strength with an oral screen for 16 min, 5 days/week. Lip force, grip force (control variable), and lip articulation were followed-up every fourth week. At baseline, after treatment, and after maintenance, the assessment protocol was completed with measurements of lip mobility using 3D motion analysis and parental reports concerning eating ability and saliva control.
                Seven of eight participants improved maximal lip strength and endurance but only four showed significant change. Increased lip strength did not automatically lead to improved function. There was a wide intra-individual variation concerning speech and eating ability within and between assessments. The treatment programme could be carried out without major problems but the frequency and the effect of training were affected by recurrent infections in some.
                Maximal lip force and lip force endurance can improve in school aged children and adolescents with DM1. Improved lip strength alone cannot be expected to have an effect on lip articulation, saliva control, or eating and drinking ability in this population. Lip strengthening exercises can be a complement but not a replacement for speech therapy and dysphagia treatment. A prefabricated oral screen is an easy to use tool suitable for strengthening lip exercises.
                Copyright © 2010 Elsevier Ireland Ltd. All rights reserved. Reduced bone mineral density (BMD), altered body composition, impaired motor performance and passive ankle dorsiflexion are side effects of acute lymphoblastic leukemia (ALL) treatment. We performed a randomized study investigating whether an exercise program could prevent these side effects.
                At diagnosis we randomized 51 ALL patients (median age: 5.4 years) into a group receiving a 2-year exercise program or a control group receiving standard care. BMD of total body (BMD(TB)), lumbar spine (BMD(LS)) and body composition were measured using dual energy X-ray absorptiometry, motor performance with Bayley Scales of Infant Development or Movement-ABC, and passive ankle dorsiflexion with a goniometer. The investigator was blinded to the randomization.
                Body fat increased equally during treatment in both groups. One year after cessation of therapy more rapid decline of excessive body fat was observed in the intervention group than in the controls (P = 0.01). Lean body mass, BMD(TB) and BMD(LS) of both groups decreased equally during treatment and increased equally thereafter. Both groups showed a similar decrease in passive ankle dorsiflexion and motor performance during treatment. Adherence to the intervention program varied considerably. Adherence to intervention: 11% of children exercised daily, 37% > once a week, 16% once weekly, 36% < once a week.
                The exercise program was not more beneficial than standard care in preventing reduction in BMD, motor performance and passive ankle dorsiflexion than standard care, most likely due to unsatisfactory compliance. Increased BMI and body fat in the intervention group normalized faster after cessation of chemotherapy.
                Copyright 2009 Wiley-Liss, Inc.
Only one study, involving people with Charcot-Marie-Tooth disease, demonstrated a statistically significant positive effect of strength training. No effect of strength training was found in people with either myotonic dystrophy or facioscapulohumeral muscular dystrophy. Surgery had no significant effect in children with Duchenne muscular dystrophy and night splinting of the ankle had no significant effect in people with Charcot-Marie-Tooth disease. More evidence generated by methodologically sound trials is required.