Search

Objective: Absent or truncated dystrophin in Duchenne (DMD) and Becker (BMD) muscular dystrophies results in impaired vasodilatory pathways and exercise induced muscle ischemia. Here, we used power Doppler sonography to quantify changes in intramuscular blood flow immediately following exercise in boys with D/BMD., Method: We quantified changes in intramuscular blood flow following exercise using power Doppler sonography in 14 boys with D/BMD and compared changes in muscle blood flow to disease severity and to historic controls., Result: Post exercise blood flow change in the anterior forearm muscles is lower in (1) DMD (median 0.25%; range -0.47 to 2.19%) than BMD (2.46%; 2.02-3.38%, p < 0.05) and historical controls (6.59%; 2.16-12.40%, p < 0.01); (2) in non-ambulatory (0.04%; -0.47 to 0.10%) than ambulatory DMD boys (0.71%; 0.07-2.19%, p < 0.05); and (3) in muscle with higher echointensity (r s  = -0.7253, p = 0.005). The tibialis anterior showed similar findings. We estimate that a single sample clinical trial would require 19 subjects to detect a doubling of blood flow to the anterior forearm after the intervention., Conclusion: Post-exercise blood flow is reduced in D/BMD and relates to disease severity., Significance: Our protocol for quantifying post-exercise intramuscular blood flow is feasible for clinical trials in D/BMD., (Copyright © 2019. Published by Elsevier B.V.)

Introduction: We evaluated the reliability of measuring muscle thickness with ultrasound in limbs and diaphragms of critically ill children and determined the sensitivity of these measures to quantitate muscle atrophy over time., Methods: An expert and trained novice sonographers prospectively measured limb and diaphragm muscle thickness in 33 critically ill children., Results: Expert and novice intrarater and interrater reliability were similar. Intraclass correlations (ICC) and coefficients of variation (CoV) were better in limbs (ICC > 0.9; CoV 3.57%-5.40%) than in diaphragm (ICC > 0.8; CoV novice 11.88%, expert, 12.28%). Mean relative difference in all muscles was small (1%-8%). Limits of agreement of the relative difference were smaller in limb (<13%-18%) than in diaphragm (<38%) muscles., Discussion: Muscle thickness is reliably measured with ultrasound by trained examiners in critically ill children. Our approach detects atrophy >13% in limb and >38% in diaphragm muscles. The smaller detectable change in limb muscles is likely due to their greater thickness. Muscle Nerve 59:88-94, 2019., (© 2018 Wiley Periodicals, Inc.)

Importance: ICU-acquired muscle atrophy occurs commonly and worsens outcomes in adults. The incidence and severity of muscle atrophy in critically ill children are poorly characterized., Objective: To determine incidence, severity and risk factors for muscle atrophy in critically ill children., Design, Setting and Participants: A single-center, prospective cohort study of 34 children receiving invasive mechanical ventilation for ≥48 hours. Patients 1 week- 18 years old with respiratory failure and without preexisting neuromuscular disease or skeletal trauma were recruited from a tertiary Pediatric Intensive Care Unit (PICU) between June 2015 and May 2016. We used serial bedside ultrasound to assess thickness of the diaphragm, biceps brachii/brachialis, quadriceps femoris and tibialis anterior. Serial electrical impedance myography (EIM) was assessed in children >1 year old. Medical records were abstracted from an electronic database., Exposures: Respiratory failure requiring endotracheal intubation for ≥48 hours., Main Outcome and Measures: The primary outcome was percent change in muscle thickness. Secondary outcomes were changes in EIM-derived fat percentage and "quality"., Results: Of 34 enrolled patients, 30 completed ≥2 ultrasound assessments with a median interval of 6 (IQR 6-7) days. Mean age was 5.42 years, with 12 infants <1 year (40%) and 18 children >1 year old (60%). In the entire cohort, diaphragm thickness decreased 11.1% (95%CI, -19.7% to -2.52%) between the first two assessments or 2.2%/day. Quadriceps thickness decreased 8.62% (95%CI, -15.7% to -1.54%) or 1.5%/day. Biceps (-1.71%; 95%CI, -8.15% to 4.73%) and tibialis (0.52%; 95%CI, -5.81% to 3.40%) thicknesses did not change. Among the entire cohort, 47% (14/30) experienced diaphragm atrophy (defined a priori as ≥10% decrease in thickness). Eighty three percent of patients (25/30) experienced atrophy in ≥1 muscle group, and 47% (14/30)-in ≥2 muscle groups. On multivariate linear regression, increasing age and traumatic brain injury (TBI) were associated with greater muscle loss. EIM revealed increased fat percentage and decreased muscle "quality"., Conclusions and Relevance: In children receiving invasive mechanical ventilation, diaphragm and other skeletal muscle atrophy is common and rapid. Increasing age and TBI may increase severity of limb muscle atrophy. Prospective studies are required to link muscle atrophy to functional outcomes in critically ill children., Competing Interests: The authors have declared that no competing interests exist.

Here we report the challenging case of a 41-year-old man with HIV complicated by AIDS and a history of prior neurologic injury from progressive multifocal leukoencephalopathy who presented with headache, fevers, lower extremity weakness, hyperreflexic upper extremities, and diminished lower extremity reflexes. We review the clinical decision-making and differential diagnosis for this presentation as the physical examination evolved and diagnostic testing changed over time., Competing Interests: Declaration of Conflicting Interests: The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Introduction: Duration of training to reliably measure nerve cross-sectional area with ultrasound is unknown., Methods: A retrospective review was performed of ultrasound data, acquired and recorded by 2 examiners-an expert and either a trainee with 2 months (novice) or a trainee with 12 months (experienced) of experience. Data on median, ulnar, and radial nerves were reviewed for 42 patients., Results: Interrater reliability was good and varied most with nerve site but little with experience. Coefficient of variation (CoV) range was 9.33%-22.5%. Intraclass correlation coefficient (ICC) was good to excellent (0.65-95) except ulnar nerve-wrist/forearm and radial nerve-humerus (ICC = 0.39-0.59). Interrater differences did not vary with nerve size or body mass index. Expert-novice and expert-experienced interrater differences and CoV were similar. The ulnar nerve-wrist expert-novice interrater difference decreased with time (r s  = -0.68, P = 0.001)., Discussion: A trainee with at least 2 months of experience can reliably measure upper limb nerves. Reliability varies by nerve and location and slightly improves with time. Muscle Nerve 57: 189-192, 2018., (© 2017 Wiley Periodicals, Inc.)

BACKGROUND Recovery from post-stroke aphasia is a long and complex process with an uncertain outcome. Various interventions have been proposed to augment the recovery, including constraint-induced aphasia therapy (CIAT). CIAT has been applied to patients suffering from post-stroke aphasia in several unblinded studies to show mild-to-moderate linguistic gains. The aim of the present study was to evaluate the neuroimaging correlates of CIAT in patients with chronic aphasia related to left middle cerebral artery stroke. MATERIAL AND METHODS Out of 24 patients recruited in a pilot randomized blinded trial of CIAT, 19 patients received fMRI of language. Eleven of them received CIAT (trained) and eight served as a control group (untrained). Each patient participated in three fMRI sessions (before training, after training, and 3 months later) that included semantic decision and verb generation fMRI tasks, and a battery of language tests. Matching healthy control participants were also included (N=38; matching based on age, handedness, and sex). RESULTS Language testing showed significantly improved performance on Boston Naming Test (BNT; p<0.001) in both stroke groups over time and fMRI showed differences in the distribution of the areas involved in language production between groups that were not present at baseline. Further, regression analysis with BNT indicated changes in brain regions correlated with behavioral performance (temporal gyrus, postcentral gyrus, precentral gyrus, thalamus, left middle and superior frontal gyri). CONCLUSIONS Overall, our results suggest the possibility of language-related cortical plasticity following stroke-induced aphasia with no specific effect from CIAT training.

Introduction: The radial nerve and posterior interosseous nerve (PIN) are prone to injury at multiple sites. Electrodiagnostic (EDx) studies may only identify the most proximal lesion. Nerve ultrasound could augment EDx by visualizing additional pathology., Methods: This investigation was a retrospective examination of ultrasound and EDx from 26 patients evaluated for posterior cord/radial/PIN lesions., Results: Eighteen of 26 patients had abnormalities on EDx (15 radial, 2 PIN, 1 posterior cord). Ultrasound identified 15 of 18 (83%) of the EDx abnormalities and provided additional diagnostic information. In 6 of 15 (40%) patients with EDx evidence of radial neuropathy, ultrasound identified both radial nerve enlargement and additional, unsuspected PIN enlargement (53% to 339% enlarged vs. unaffected side). Ultrasound also identified: nerve (dis)continuity at the trauma site (n = 8); and nerve tumor (n = 2; 1 with normal EDx)., Conclusion: In radial neuropathy, ultrasound often augments EDx studies and identifies a second lesion in the PIN. Further studies are required to determine the etiology and significance of this additional distal pathology., (© 2015 Wiley Periodicals, Inc.)

Background: To provide a preliminary estimate of efficacy of constraint-induced aphasia therapy (CIAT) when compared to no-intervention in patients with chronic (>1 year) post-stroke aphasia in order to plan an appropriately powered randomized controlled trial (RCT)., Material and Methods: We conducted a pilot single-blinded RCT. 24 patients were randomized: 14 to CIAT and 10 to no-intervention. CIAT groups received up to 4 hours/day of intervention for 10 consecutive business days (40 hours or therapy). Outcomes were assessed within 1 week of intervention and at 1 and 12 weeks after intervention and included several linguistic measures and a measure of overall subjective communication abilities (mini-Communicative Abilities Log (mini-CAL)). Clinicians treating patients (CIAT group) did not communicate with other team members to maintain blinding and the testing team members were blinded to treatment group assignment., Results: Overall, the results of this pilot RCT support the results of previous observational studies that CIAT may lead to improvements in linguistic abilities. At 12 weeks, the treatment group reported better subjective communication abilities (mini-CAL) than the no-intervention group (p=0.019). Other measures trended towards better performance in the CIAT group., Conclusions: In this pilot RCT intensive language therapy led to an improvement in subjective language abilities. The effects demonstrated allow the design of a definitive trial of CIAT in patients with a variety of post-stroke aphasia types. In addition, our experiences have identified important considerations for designing subsequent trial(s) of CIAT or other interventions for post-stroke aphasia.

Objective: Femoral neck fractures that are associated with femoral shaft fractures have historically been associated with high rates of missed diagnosis. Despite the potentially serious consequences of a missed femoral neck fracture, little work has been conducted to rigorously evaluate the ability of commonly used imaging studies to detect such fractures. Our hypothesis was that axial-view computed tomography is superior to plain radiography at detecting femoral neck fractures., Design: Blinded assessment of a randomized image set that included axial-view computed tomographic scans of the pelvis, anteroposterior-view radiographs of the pelvis, and anteroposterior-view radiographs of the femur., Setting: Academic trauma center., Patients: Twenty-eight patients who had femoral shaft fractures with femoral neck fractures and 60 patients who had femoral shaft fractures without femoral neck fractures., Intervention: Images that were stripped of demographic data were independently viewed in random order on computer workstations by 5 trauma fellowship-trained orthopaedic surgeons who were blinded to treatment and diagnosis., Main Outcome Measurements: Sensitivity, specificity, positive post-test probability, and 1 minus negative post-test probability were calculated for diagnosing femoral neck fracture based on each image type., Results: Interobserver reliability showed "substantial agreement" (kappa > 0.65) for all imaging modalities, indicating that the surgeons had a high level of agreement. All 3 imaging sets had high specificity (>94%) and 1 minus negative post-test probability (>95%) but had poor sensitivity (<65%) and positive post-test probability (<58%). False positives and false negatives were equally likely for all imaging modalities (P > 0.2)., Conclusions: Plain radiography and computed tomography have rates of missed femoral neck fractures that are similar and substantial, with a sensitivity of only 56%-64%. Our data emphasize the importance of intraoperative and postoperative imaging in detecting nondisplaced femoral neck fractures in association with femoral shaft fractures.