AANEM news and comments

Introduction: With clinical trials underway, our objective was to construct a composite score of global function that could discriminate among people with spinal muscular atrophy (SMA). Methods: Data were collected from 126 participants with SMA types 2 and 3. Scores from the Hammersmith Functional Motor Scale—Expanded and Upper Limb Module were expressed as a percentage of the maximum score and 6‐minute walk test as percent of predicted normal distance. A principal component analysis was performed on the correlation matrix for the 3 percentage scores. Results: The first principal component yielded a composite score with approximately equal weighting of the 3 components and accounted for 82% of the total variability. The SMA functional composite score, an unweighted average of the 3 individual percentage scores, correlated almost perfectly with the first principal component. Conclusions: This combination of measures broadens the spectrum of ability that can be quantified in type 2 and 3 SMA patients. Muscle Nerve 52 : 942–947, 2015     

Outcome reliability in non‐Ambulatory Boys/Men with duchenne muscular dystrophy

Introduction: Therapeutic trials in Duchenne muscular dystrophy (DMD) often exclude non‐ambulatory individuals. Here we establish optimal and reliable assessments in a multicenter trial. Methods: Non‐ambulatory boys/men with DMD (N = 91; 16.7 ± 4.5 years of age) were assessed by trained clinical evaluators. Feasibility (percentage completing task) and reliability [intraclass correlation coefficients (ICCs) between morning and afternoon tests] were measured. Results: Forced vital capacity (FVC), assessed in all subjects, showed a mean of 47.8 ± 22% predicted (ICC 0.98). Brooke Upper Extremity Functional Rating (Brooke) and Egen Klassifikation (EK) scales in 100% of subjects showed ICCs ranging from 0.93 to 0.99. Manual muscle testing, range of motion, 9‐hole peg test, and Jebsen‐Taylor Hand Function Test (JHFT) demonstrated varied feasibility (99% to 70%), with ICCs ranging from 0.99 to 0.64. We found beneficial effects of different forms of corticosteroids for the Brooke scale, percent predicted FVC, and hand and finger strength. Conclusions: Reliable assessment of non‐ambulatory boys/men with DMD is possible. Clinical trials will have to consider corticosteroid use. Muscle Nerve 51: 522–532, 2015     

Prospective study of muscle cramps in Charcot‐Marie‐Tooth disease

Introduction: This study aims to assess the frequency, location, severity, duration, and fluctuation over time of muscle cramps in Charcot‐Marie‐Tooth disease (CMT). Methods: Inherited Neuropathies Consortium Contact Registry participants recorded the occurrence and characteristics of muscle cramps using an 11‐question survey administered 3 times over 8 weeks. Results: A total of 110 adult patients with CMT completed the survey. Weekly cramp frequency was 9.3 (SD 12.3), and 23% had daily muscle cramps. Twenty‐two percent reported a significant impact on quality of life. Over 8 weeks, the daily frequency and severity of muscle cramps did not change significantly. Conclusions: Patients with CMT have muscle cramps that vary little over an 8‐week period, and they may interfere with quality of life. These data may be useful in the planning of clinical trials of agents to treat adults with CMT‐associated muscle cramps. Muscle Nerve 51: 485–488, 2015     

Old measures and new scores in spinal muscular atrophy patients

Introduction: A recent Rasch analysis performed on the Hammersmith Functional Motor Scale—Expanded (HFMSE) in patients with spinal muscular atrophy (SMA) identified issues impacting scale validity, redundant items, and disordered thresholds on some items. Methods: We modified the HMFSE scoring based on the Rasch analysis and on expert consensus to establish whether the traditional scoring overestimated the number of patients with changes within 2 points from baseline. Data were collected retrospectively from multicenter data sets in 255 type 2 and 3 SMA patients. Results: The mean 12‐month changes using the new and the traditional scoring system did not differ significantly (P > 0.05). The numbers of patients who improved or decreased by >2 points were also similar. Conclusions: The presence of outliers using the traditional scoring system was not due to overestimation of changes in activities that were tested bilaterally or to discrepancies in the scoring hierarchy of individual items. Muscle Nerve 52:435–437, 2015     

Recent progress in the treatment and prevention of cancer‐related lymphedema

Answer questions and earn CME/CNE This article provides an overview of the recent developments in the diagnosis, treatment, and prevention of cancer‐related lymphedema. Lymphedema incidence by tumor site is evaluated. Measurement techniques and trends in patient education and treatment are also summarized to include current trends in therapeutic and surgical treatment options as well as longer‐term management. Finally, an overview of the policies related to insurance coverage and reimbursement will give the clinician an overview of important trends in the diagnosis, treatment, and management of cancer‐related lymphedema. CA Cancer J Clin 2015;65:55–81. © 2014 American Cancer Society.     

A Spectral Analysis of Rotator Cuff Musculature Electromyographic Activity: Surface and Indwelling

Electromyography (EMG) of the shoulder girdle is commonly performed; however, EMG spectral properties of shoulder muscles have not been clearly defined. The purpose of this study was to determine the maximum power frequency, Nyquist rate, and minimum sampling rate for indwelling and surface EMG of the normal shoulder girdle musculature. EMG signals were recorded using indwelling electrodes for the rotator cuff muscles and surface electrodes for ten additional shoulder muscles in ten healthy volunteers. A fast Fourier transform was performed on the raw EMG signal collected during maximal isometric contractions to derive the power spectral density. The 95% power frequency was calculated during the ramp and plateau subphase of each contraction. Data were analyzed with analysis of variance (ANOVA) and paired t tests. Indwelling EMG signals had more than twice the frequency content of surface EMG signals (p < .001). Mean 95% power frequencies ranged from 495 to 560 Hz for indwelling electrodes and from 152 to 260 Hz for surface electrodes. Significant differences in the mean 95% power frequencies existed among muscles monitored with surface electrodes (p = .002), but not among muscles monitored with indwelling electrodes (p = .961). No significant differences in the 95% power frequencies existed among contraction subphases for any of the muscle–electrode combinations. Maximum Nyquist rate was 893 Hz for surface electrodes and 1,764 Hz for indwelling electrodes. Our results suggest that when recording EMG of shoulder muscles, the minimum sampling frequency is 1,340 Hz for surface electrodes and 2,650 Hz for indwelling electrodes. The minimum sampling recommendations are higher than the 1,000 Hz reported in many studies involving EMG of the shoulder.