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Background and Purpose. Simulation-based learning is commonly used in physical therapy education. However, given the financial cost and resources required to provide high-fidelity simulation (HFS)-based learning, it is necessary to determine its effectiveness. Therefore, the purpose of this study was to investigate the effect of HFSbased learning using computerized mannequins on clinical performance and preparedness of physical therapy students and physical therapists in acute cardiorespiratory physical therapy. Methods. We undertook a mixed-methods systematic review andmeta-analysis. Search strategies were used to find articles in electronic databases. Reference lists of studies were also searched. Included studies investigated the effect of HFS-based learning on physical therapy students' and physical therapists' clinical performance and preparedness in acute cardiorespiratory physical therapy and included the use of computerized mannequins. Both quantitative and qualitative studies were considered. Results. A total of 12 trials from 11 articles were included. Quantitative findings of pooled studies indicated a small, but not statistically significant, effect of simulation training on clinical performance of physical therapy students' when compared with traditional learning methods (Hedges' g = 0.18; 95% confidence interval, 20.18 to 0.64; P = .07; I2 = 57%). Qualitative data were synthesized into 4 themes in which HFS positively impacted students' preparedness: familiarization with the clinical setting, real-world learning in a controlled environment, learning about patient safety, and self-efficacy and confidence. Only the theme of self-efficacy and confidence were able to be integrated with quantitative data, demonstrating a positive effect of HFS on students' and physical therapists' self-efficacy and confidence. Discussion and Conclusion. High- fidelity simulation involving the use of computerized mannequins improves students' preparedness for clinical practice but does not improve students' clinical performance, although limited evidence exists for physical therapists' preparedness and clinical performance, indicating that further research of physical therapy clinicians is required. [ABSTRACT FROM AUTHOR]

BACKGROUND: In this article, the authors explore functional connectivity and network topology in electroencephalography recordings of patients with delirium after cardiac surgery, aiming to improve the understanding of the pathophysiology and phenomenology of delirium. The authors hypothesize that disturbances in attention and consciousness in delirium may be related to alterations in functional neural interactions. METHODS: Electroencephalography recordings were obtained in postcardiac surgery patients with delirium (N = 25) and without delirium (N = 24). The authors analyzed unbiased functional connectivity of electroencephalography time series using the phase lag index, directed phase lag index, and functional brain network topology using graph analysis. RESULTS: The mean phase lag index was lower in the [alpha] band (8 to 13 Hz) in patients with delirium (median, 0.120; interquartile range, 0.113 to 0.138) than in patients without delirium (median, 0.140; interquartile range, 0.129 to 0.168; P < 0.01). Network topology in delirium patients was characterized by lower normalized weighted shortest path lengths in the [alpha] band (t = -2.65; P = 0.01). [delta] Band-directed phase lag index was lower in anterior regions and higher in central regions in delirium patients than in nondelirium patients (F = 4.53; P = 0.04, and F = 7.65; P < 0.01, respectively). CONCLUSIONS: Loss of [alpha] band functional connectivity, decreased path length, and increased [delta] band connectivity directed to frontal regions characterize the electroencephalography during delirium after cardiac surgery. These findings may explain why information processing is disturbed in delirium. [ABSTRACT FROM AUTHOR]

Sleep deprivation is common in intensive care unit (ICU) patients. The criterion standard for sleep monitoring, polysomnography, is impractical in ICU. Actigraphy (a wrist watch indicating amount of sleep) proved to be a good alternative in non-ICU patients, but not in prolonged mechanically ventilated patients, probably due to ICU-acquired weakness. Short-stay ICU patients do not suffer from ICU-acquired weakness. However, the accuracy of actigraphy is unknown in these patients. Therefore, we compared actigraphy to polysomnography in short-stay ICU patients. Sleep measurements were conducted in 7 postcardiothoracic surgery patients. The sensitivity (percentage of actigraphy data that agreed with sleep determined using polysomnography) and specificity (percentage of actigraphy data that agreed with awake determined using polysonmography) were calculated. The result showed that actigraphy underestimated the amount of wake time and overestimated the amount of sleep. The median specificity for actigraphy was always less than 19% and sensitivity more than 94%. Therefore, actigraphy is not reliable for sleep monitoring in short-stay ICU patients. [ABSTRACT FROM AUTHOR]

Objective: To clarify the prevalence, long-term natural history, and severity determinants of SEPN1 -related myopathy (SEPN1-RM), we analyzed a large international case series., Methods: Retrospective clinical, histologic, and genetic analysis of 132 pediatric and adult patients (2-58 years) followed up for several decades., Results: The clinical phenotype was marked by severe axial muscle weakness, spinal rigidity, and scoliosis (86.1%, from 8.9 ± 4 years), with relatively preserved limb strength and previously unreported ophthalmoparesis in severe cases. All patients developed respiratory failure (from 10.1±6 years), 81.7% requiring ventilation while ambulant. Histopathologically, 79 muscle biopsies showed large variability, partly determined by site of biopsy and age. Multi-minicores were the most common lesion (59.5%), often associated with mild dystrophic features and occasionally with eosinophilic inclusions. Identification of 65 SEPN1 mutations, including 32 novel ones and the first pathogenic copy number variation, unveiled exon 1 as the main mutational hotspot and revealed the first genotype-phenotype correlations, bi-allelic null mutations being significantly associated with disease severity ( p = 0.017). SEPN1-RM was more severe and progressive than previously thought, leading to loss of ambulation in 10% of cases, systematic functional decline from the end of the third decade, and reduced lifespan even in mild cases. The main prognosis determinants were scoliosis/respiratory management, SEPN1 mutations, and body mass abnormalities, which correlated with disease severity. We propose a set of severity criteria, provide quantitative data for outcome identification, and establish a need for age stratification., Conclusion: Our results inform clinical practice, improving diagnosis and management, and represent a major breakthrough for clinical trial readiness in this not so rare disease., (© 2020 American Academy of Neurology.)

Objectives: Cardiac involvement has been reported in carriers of dystrophin mutations giving rise to Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD). The progress of these abnormalities during long-term follow-up is unknown. We describe the long-term follow-up of dilated cardiomyopathy (DCM) in DMD/BMD carriers., Methods: A long-term follow-up study was performed among Dutch DMD/BMD carriers first analyzed in 1995. A cardiac history was taken, and all carriers were assigned a functional score to assess skeletal muscle involvement. Electrocardiography and M-mode and 2-D echocardiography were performed. DCM was defined as an enlarged left ventricle with a global left ventricle dysfunction or fractional shortening less than 28%. Slow vital capacity of the lung was measured by a hand-held spirometer., Results: Ninety-nine carriers were monitored with a median follow-up of 9 years (range 7.0-10.6 years). Eleven carriers with DCM (10 DMD, 1 BMD) were identified. Nine of them developed DCM in the follow-up period. One of the patients with DCM reported in the 1995 study died of cardiac failure at age 57 years. DCM was more frequently found in carriers who were functionally symptomatic., Conclusion: Cardiac abnormalities in DMD/BMD carriers are progressive, as in patients with DMD/BMD.

The authors report the clinical and histologic phenotypes of a LGMD1B family including a newborn child with a homozygous LMNA nonsense mutation (Y259X). At the heterozygous state the nonsense mutation leads to a classic LGMD1B phenotype; the homozygous LMNA nonsense mutation causes a lethal phenotype.