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Objective: To determine whether a home-based activity and dietary intervention can increase activity level, reduce caloric intake, and impact positively components of metabolic syndrome in a disabled population., Design: Testing occurred at 3 points during the 6-month intervention period (baseline, 3mo, 6mo) and at 6 months postintervention. Each test point included laboratory testing of anthropometric and metabolic variables and 3 days of home-based activity and dietary monitoring. A personally tailored activity and dietary prescription based on baseline testing was implemented during the 6-month intervention period., Setting: Human performance laboratory of a university and each subject's home., Participants: Twenty adult volunteer ambulatory subjects with several types of slowly progressive neuromuscular disease (NMD)., Intervention: Using a pedometer, subjects were instructed to increase number of steps by 25% over their baseline determined from home monitoring. An individualized dietary prescription was provided focusing on problematic issues identified from the baseline dietary profile., Main Outcome Measures: Body composition, physical activity, dietary intake, energy expenditure, gait efficiency, metabolic variables, and quality of life., Results: At the end of the protocol, mean step count increased approximately 27% above baseline (P=.001) and caloric intake decreased over 300kcal/d (P=.002). Body fat percentage significantly decreased (from 33.3%+/-1.5% to 32.6%+/-1.6%, P=.032). Gait efficiency did not change, and metabolic variables did not show statistically significant improvement, although 2 of the 5 subjects originally meeting the criteria for metabolic syndrome at baseline no longer met the criteria at the end of the intervention period. Six months after completing the protocol, caloric intake remained significantly reduced (P=.02), but although mean step count remained elevated, it was not statistically significant., Conclusions: Using a home-based protocol, people with NMD can increase activity and reduce caloric intake. Although this 6-month program showed positive changes, it was insufficient to affect risk factors associated with metabolic syndrome. It remains to be seen if a program longer than 6 months or a more rigorous program could lead to a reduction in the risk factors associated with metabolic syndrome.

Objectives: To test the hypotheses that (1) people with neuromuscular disease (NMD) have multiple risk factors for cardiovascular disease and diabetes and (2) these risk factors worsen over time., Design: Longitudinal testing with average 2.5-year follow-up., Setting: Human performance laboratory of a university., Participants: Eleven ambulatory volunteers with slowly progressive NMD and 8 able-bodied controls, group-matched for age and body mass index (BMI) at baseline., Interventions: Not applicable., Main Outcome Measures: Percentage of body fat (%BF), physical activity, energy expenditure, blood lipids and glucose, and blood pressure., Results: At baseline, NMD subjects were more obese (37%BF vs 34%BF, respectively) and more sedentary than the controls, spending less time in total activity (144 min/d vs 214 min/d) and in exercise (11 min/d vs 45 min/d). The NMD group also had numerous cardiovascular and metabolic risk factors, with low high-density lipoprotein cholesterol, high BMI, and high triglyceride being the most common. Additionally, 55% of the NMD group satisfied the criteria for metabolic syndrome, versus 0% in the control group. Most parameters did not significantly worsen during the average 2.5-year follow-up period in either group., Conclusions: People with NMD are at high risk for developing chronic diseases resulting from obesity and a sedentary lifestyle. Intervention studies aimed at reducing their risk for such chronic diseases are warranted.

There is currently no gold standard to definitively diagnose carpal tunnel syndrome. It remains a clinical diagnosis supported by characteristic electrodiagnostic abnormalities. Properly performed electrodiagnostic studies should provide the hand surgeon with information regarding severity, progression if a previous study was performed, and a reasonable assurance that concomitant peripheral nervous system abnormalities are not present. Hand surgeons do not need to discern nuances of an electrodiagnostic evaluation; however, the ability to identify state-of-the-art techniques coupled with a thoughtful interpretation by the electrodiagnostician will improve their confidence in using this important diagnostic tool to evaluate carpal tunnel syndrome.

Objectives: To determine the test-retest reliability of selected simulated work performance tasks in persons with neuropathic and myopathic weakness and control subjects, and the association between muscular performance during these work tasks and conventional isolated muscle group testing., Design: Measurement of three tasks performed on a work simulation device on two different days separated by 1 week. Associations between work task performance and previously reported strength measures in six muscle groups by hand-held dynamometry (HHD) were examined., Setting: Human performance laboratory of a university., Participants: Convenience sample of ambulatory outpatients with hereditary motor and sensory neuropathy, type I (n = 9), myotonic muscular dystrophy (n = 10), and able-bodied controls (n = 11)., Main Outcome Measures: For work simulation, isokinetic peak torque and total work; and for HHD, maximal isometric torque., Results: Mean between-session differences for work simulation tasks ranged from -11% to +4% for peak torque and from -12% to +12% for total work; test sessions did not differ significantly for either patient or control groups. All groups had between-session intraclass correlation coefficients usually >.80, indicating good consistency. In general, correlations between peak torque during work simulation and HHD were strongest in the control group., Conclusion: Persons with neuromuscular weakness reliably performed the simulated work tasks examined in this investigation. Work simulation tasks may be a useful tool to assess muscular performance in persons with neuromuscular weakness.

Objective: To determine test-retest reliability of hand-held dynamometry (HHD) in measuring strength of persons with neuropathic weakness., Design: Intratester and intertester reliability of HHD-measured strength over a 7- to 10-day period. In addition, HHD knee strength was compared with criterion standard of fixed dynamometry (FD)., Setting: Human performance laboratory of a university., Participants: Convenience sample of ambulatory outpatients with Hereditary Motor and Sensory Neuropathy, Type I (HMSN) (n = 10) and able-bodied controls (CTL) (n = 11)., Main Outcome Measure: Maximal isometric torque., Results: Intratester intraclass correlation coefficients (ICCs) were high, generally ranging from .82 to .96 for HHD- and FD-measured strength for both HMSN and CTL groups. There were no significant differences between sessions for HHD-measured strength, while FD-measured strength was only significantly different for knee extension (p < .01). Intertester reliability was generally good for both HHD- and FD-measured strength, with ICCs ranging from .72 to .97 for HMSN and CTL groups. Exceptions were knee extensors and ankle dorsiflexors for the CTL group. Knee extensor strength was significantly lower measured by HHD compared with FD (p < .01), but knee flexor strength was similar for the two methods., Conclusion: HHD appears to be a reliable method to measure maximal isometric strength in persons with neurogenic weakness, and may be useful to quickly and objectively evaluate strength in the clinical setting.

Objective: Poor cardiorespiratory endurance is a common finding in neuromuscular disease (NMD), and the capacity of such patients to respond to aerobic training is unclear. This study was conducted to determine if a 12-week walking program results in increased aerobic capacity in slowly progressive NMD subjects, whether such a program is safely tolerated, and whether such patients can adhere to a self-monitored, home-based training program., Design: Before-after trial., Setting: Subjects' homes., Patients: A cohort of 8 slowly progressive NMD subjects (4 men, 4 women) followed in the neuromuscular disease clinic participated (age, 36.6 +/- 8.0 yrs; ht, 170 +/- 11 cm; wt, 74.3 +/- 19.0 kg) (Mean +/- SD)., Intervention: Subjects walked 15 to 30 min 3 to 4 days a week at 50% to 60% of their heart rate reserve., Main Outcome Measures: Resting, submaximal, and peak heart rates, systolic and diastolic blood pressures, oxygen uptake, and peak power output., Results: Graded exercise testing to volitional fatigue using a semirecumbent cycle ergometer before and after the training program found significant decreases in submaximal heart rate by 7 +/- 3 beats/min (Mean +/- SEM) (95% CI = -23 to 9) (p = .046) and submaximal systolic blood pressure by 11 +/- 4 mmHg (95% CI = -31 to 9) (p = .019), and nonsignificant increases in peak power output and VO2., Conclusions: These results suggest that moderate-intensity aerobic exercise training is well tolerated and may provide modest improvement in aerobic capacity in slowly progressive NMD subjects.

A 12-week high resistance home exercise program was completed by 10 subjects with slowly progressive neuromuscular diseases (NMD) and 6 normal control subjects (CTL). After evaluating baseline maximal isometric and isokinetic strength of the elbow flexors and knee extensors, subjects completed the home program using adjustable ankle and wrist cuff weights. One side of the body was randomly chosen for exercise. Subjects initially performed 1 set of 10 repetitions on 3 days a week and gradually increased to 5 sets of 10 repetitions on 4 days a week. The NMD group demonstrated significant (p < 0.05) gains in several knee extension isokinetic strength measures but loss of elbow flexion eccentric peak torque and work per degree. The CTL group demonstrated significant gains in all measures of knee extension strength, with improvement of elbow isokinetic eccentric work per degree as well. There was evidence of cross training to the nonexercised limbs for both groups. A high resistance training program, although well tolerated in NMD subjects with mild to moderate weakness, may cause some deleterious effects to diseased skeletal muscle. A high resistance training program appears to offer no advantage over a moderate resistance training program in this population.

Serial measurements of muscle strength of 34 muscle groups were obtained for up to a seven-year period using manual muscle testing (MMT) in a group of 63 boys (mean age = 11.5 years) with Duchenne muscular dystrophy (DMD) not using lower extremity bracing or corrective surgery for ambulation, in order to define the natural course of strength loss and its relation to anthropometric variables and ambulatory status. When correlating the first MMT assessment of each patient with age, strength was found to decrease linearly (-0.26 +/- 0.30 MMT units/year) with time until approximately age 14 when a marked decrease in progression was noted (-0.06 +/- 0.03 MMT units/year). Analyzing individual patients longitudinally yielded a similar rate of strength loss. The average muscle score was 3.0 MMT units at the point of full-time wheelchair use. The marked decline in MMT deterioration around age 14 may be an important consideration when assessing the efficacy of therapeutic interventions in adolescent boys with DMD.

A 12-week moderate resistance exercise program was performed by 27 patients with slowly progressive neuromuscular diseases (NMD) and 14 control subjects (CTL) in order to determine safety and efficacy of a strengthening program. A 3-day per week submaximal regimen of home exercise using ankle and wrist weights and hand grip exerciser was prescribed. One side of the body was randomly chosen for exercise. Subjects were tested for maximal isokinetic and isometric strength at baseline and after weeks 4 and 12 of the training protocol, and the prescribed amount of work was gradually increased throughout the program. Both the NMD and CTL groups demonstrated significant (p < .05) increases in most strength measures. Both groups responded similarly to the exercise program, and strength gains did not significantly differ between the exercised and nonexercised limbs in either group. This study provides evidence that a 12-week submaximal strength training program is practical and safe in slowly progressive NMD and produces moderate improvement in measured strength.