Few studies have examined the effect of pain on the quality of life of individuals with slowly progressive neuromuscular disease (NMD). The purpose of this study was to determine the frequency and extent to which subjects with slowly progressive NMD report pain and the association between pain and health-related quality of life in persons with NMD. The study design was a descriptive, nonexperimental survey. Of a total of 1,432 subjects with slowly progressive NMDs recruited from a university-based NMD clinic and the membership rosters of worldwide NMD support organizations, 859 agreed to participate. The primary measurement tool used was the Medical Outcomes Study SF-36 health survey. Our results indicated that, with the exception of adult spinal muscular atrophy (SMA), the frequency and severity of pain reported in slowly progressive NMDs was significantly greater than levels of pain reported by the general US population and was comparable to pain reported by subjects with osteoarthritis and chronic low back pain. There was a significant correlation between increased pain and lower levels of general health, vitality, social function, and physical role. Pain was moderately associated with increased fatigue, inability to cope adequately with stress, and sleep disturbance. In conclusion, with the exception of adult SMA, the frequency and severity of pain reported in slowly progressive NMDs was significant.

Introduction Cardiomyopathy is a common cause of morbidity and death in patients with Duchenne muscular dystrophy (DMD). Methods A cross-sectional analysis of clinical data from a multi-institutional, international CINRG DMD Natural History Study of 340 DMD patients aged 2 to 28 years. Cardiomyopathy was defined as shortening fraction (SF) <28% or ejection fraction (EF) <55%. Results 231 participants reported a prior clinical echocardiogram study, and 174 had data for SF or EF. The prevalence of cardiomyopathy was 27% (47/174), and it was significantly associated with age and clinical stage. The association of cardiomyopathy with age and clinical stage was not changed by glucocorticoid use as a covariate (P>0.68). In patients with cardiomyopathy, 57 % (27/47) reported not taking any cardiac medications. Cardiac medications were used in 12% (15/127) of patients without cardiomyopathy. Discussion Echocardiograms were underutilized, and cardiomyopathy was undertreated in this DMD natural history cohort.

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The purpose of the study is to further assess the usefulness of short TI (time to inversion) recovery (STIR) magnetic resonance imaging (MRI) in detecting denervation of skeletal muscle compared to needle electromyography (EMG). Ninety subjects with clinical evidence of peripheral nerve injury or radiculopathy underwent STIR MRI and EMG of the affected limb. In 74 (82%) of these subjects, a positive correlation was found between STIR MRI and EMG (P < 0.009). STIR MRI has a relative sensitivity of 84% and specificity of 100% for detecting denervation. A subset of 28 subjects underwent quantitative assessments of signal intensity ratio (SIR) from the STIR MRI. The rank order correlation coefficient between the SIR and abnormal spontaneous activity on EMG was 0.70 (P < 0.001). Increased signal intensity on STIR MRI corresponds closely with spontaneous activity on EMG in denervated muscle. Although less sensitive than EMG in detecting muscle denervation, STIR MRI may be a useful adjunctive diagnostic tool in this setting. © 2000 John Wiley & Sons, Inc. Muscle Nerve 23: 1431‐1434, 2000