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Prospective memory (PM) deficits have been documented in multiple sclerosis (MS). This study aimed to explore the specific types of errors made by persons with MS (PwMS), including differences between PwMS and healthy controls (HC) and PwMS who do and do not have impairments in processing speed and/or verbal learning and memory. PwMS (n = 111) and HC (n = 75) completed the Memory for Intentions Test (MIST), an objective measure of PM that has five types of errors that can be coded (PM failure, task substitution, loss of content, loss of time, and random errors). The number and types of PM errors were calculated for the overall MIST and six subscales, which break down performance by types of delay (2-Minute and 15-Minute), cue (Time and Event), and response (Verbal and Action). Impairment was defined as performing < 1.5 SD on either the Symbol Digit Modalities Test (SDMT) or Rey Auditory Verbal Learning Test (RAVLT). Bivariate analyses were used to examine group differences, with post-hoc pairwise comparisons with Bonferroni corrections. Nearly 93% of PwMS made at least one PM error, compared to 76% of HC (V =.24, p =.001). The most commonly made PM error by PwMS was loss of content errors (45.0%). PwMS made significantly more task substitution errors (26.4% vs. 7.6%, p <.001) and fewer loss of time errors (9.5% vs. 21.2%, p <.001) than HC. Impaired PwMS made more errors than non-impaired PwMS, specifically PM failures on time-based tasks. PM errors are common in PwMS, particularly when there are longer delays and time-based cues. Not only do PwMS make more errors than demographically similar HC, but they exhibit different cognitive process failures. [ABSTRACT FROM AUTHOR]

Background: Muscle weakness is common and significantly affects persons with multiple sclerosis (PwMS), with dysfunction in upper limb (UL) muscle groups occurring in approximately 60% of PwMS., Objective: To develop gender-specific regression-based prediction equations, with 95% confidence intervals for maximal bilateral UL isometric strength (shoulder abduction and adduction, wrist flexion and extension) and hand grip strength in PwMS., Design: Cross-sectional study., Setting: Comprehensive MS center., Participants: 256 PwMS., Interventions: Not Applicable., Main Outcome Measures: Shoulder abduction and adduction and wrist flexion and extension isometric strength (Biodex System 4 Pro Dynamometer) and hand grip strength (Jamar handheld dynamometer) were measured. Disease characteristics (disability and disease duration) and demographics (age, height, and weight) were collected. Regression-based predictive equations were generated for the UL muscle groups for each gender and limb, using age, height, weight, disability, and disease duration as covariates. Variables were compared between genders using the Mann-Whitney U test. Maximal voluntary contraction (MVC) reference values (mean ± SD) were reported based on age (<30, 30-39, 40-49, 50-59, 60-69 years) and disability (mild, moderate, severe ambulant, and severe nonambulant) for each gender and limb., Results: Regression-based equations were developed for both genders' strongest and weakest limb, accounting for age, height, weight, disability, and disease duration. MVC was higher in men than women (p < .001) in all muscle groups. Overall, MVC was significantly related to age in 14, height in 5, weight in 6, disability in 14, and disease duration in none of the 20 models., Conclusion: This is the first study to provide regression-based prediction equations for strongest and weakest MVC of UL muscle groups and demonstrated an inverse relationship between MVC with disability and age. Regression-based reference strength values can help clinicians understand muscular strength along a spectrum of PwMS and can aid in goal setting and education for realistic outcomes., (© 2024 American Academy of Physical Medicine and Rehabilitation.)

Objectives: The study objectives were to analyze the outcomes of pediatric patients with heterotaxy syndrome undergoing cardiovascular surgery and to determine the predictors of mortality., Methods: A retrospective analysis of 82 patients diagnosed with heterotaxy syndrome who underwent cardiovascular surgery between January 2008 and December 2017 was performed. Univariate and multivariable Cox regression analyses to determine risk factors for mortality and Kaplan-Meier analysis for survival were performed., Results: Patient mortality in the cohort was 34% (28/82), including 36% (20/55) for single ventricle palliation and 30% (8/27) for biventricular repair. At 5 years, the probability of survival did not differ between the groups by log-rank testing ( P  = .829). Multivariable analysis found extracorporeal membrane oxygenation support (hazard ratio, 10.4; 95% confidence interval, 4.3-25.4; P  < .001), total anomalous pulmonary venous return (hazard ratio, 4.3; 95% confidence interval, 1.7-10.8; P  = .002), and birth weight 2.5 kg or less (hazard ratio, 2.4; 95% confidence interval, 1.0-5.4; P  = .041) to be independent risk factors for mortality in all-comers. Pulmonary vein stenosis was a univariate predictor of mortality among all patients with heterotaxy (hazard ratio, 3.0; 95% confidence interval, 1.4-6.4; P  = .005) and in the subgroup of patients with single ventricles (hazard ratio, 4.0; 95% confidence interval, 1.7-9.7; P  = .002). Overall survival was 66% (54/82) at a median follow-up time of 2.2 years (0.4-4.1) from the initial surgery., Conclusions: Outcomes of children with heterotaxy syndrome, irrespective of the operative pathway, remain suboptimal in the current era. Risk factors for mortality in this population include birth weight 2.5 kg or less, extracorporeal membrane oxygenation, pulmonary vein stenosis, and total anomalous pulmonary venous return, which may help to further optimize surgical decision making. Multiorgan system involvment is frequently encountered in these patients., (© 2022 The Author(s).)

Compared with acyanotic congenital heart disease (CHD), cyanotic CHD has an increased risk of lifelong mortality and morbidity. These adverse outcomes may be attributed to delayed cardiomyocyte maturation, since the transition from a hypoxic fetal milieu to oxygen-rich postnatal environment is disrupted. We established a rodent model to replicate hypoxic myocardial conditions spanning perinatal development, and tested the hypothesis that chronic hypoxia impairs cardiac development. Pregnant mice were housed in hypoxia beginning at embryonic day 16 . Pups stayed in hypoxia until postnatal day ( P ) 8 when cardiac development is nearly complete. Global gene expression was quantified at P8 and at P30 , after recovering in normoxia. Phenotypic testing included electrocardiogram, echocardiogram, and ex vivo electrophysiology study. Hypoxic P8 animals were 47% smaller than controls with preserved heart size. Gene expression was grossly altered by hypoxia at P8 (1,427 genes affected), but normalized after recovery ( P30 ). Electrocardiograms revealed bradycardia and slowed conduction velocity in hypoxic animals at P8 , with noticeable resolution after recovery ( P30 ). Notable differences that persisted after recovery ( P30 ) included a 65% prolongation in ventricular effective refractory period, sinus node dysfunction, 23% reduction in ejection fraction, and 16% reduction in fractional shortening in animals exposed to hypoxia. We investigated the impact of chronic hypoxia on the developing heart. Perinatal hypoxia was associated with changes in gene expression and cardiac function. Persistent changes to the electrophysiological substrate and contractile function warrant further investigation and may contribute to adverse outcomes observed in the cyanotic CHD population. NEW & NOTEWORTHY We utilized a new mouse model of chronic perinatal hypoxia to simulate the hypoxic myocardial conditions present in cyanotic congenital heart disease. Hypoxia caused numerous abnormalities in cardiomyocyte gene expression, the electrophysiologic substrate of the heart, and contractile function. Taken together, alterations observed in the neonatal period suggest delayed cardiac development immediately following hypoxia.