Acquired amusia after a right middle cerebral artery infarction - a case study.

A 62-year-old musician-MM-developed amusia after a right middle-cerebral-artery infarction. Initially, MM showed melodic deficits while discriminating pitch-related differences in melodies, musical memory problems, and impaired sensitivity to tonal structures, but normal pitch discrimination and spectral resolution thresholds, and normal cognitive and language abilities. His rhythmic processing was intact when pitch variations were removed.

Light switching for product selectivity control in photocatalysis.

Artificial switchable catalysis is a new, rapidly expanding field that offers great potential advantages for both homogeneous and heterogeneous catalytic systems. Light irradiation is widely accepted as the best stimulus to artificial switchable chemical systems. In recent years, tremendous progress has been made in the synthesis and application of photo-switchable catalysts that can control when and where bond formation and dissociation take place in reactant molecules.

Factors Associated With Early Motor Function Trajectories in DMD After Glucocorticoid Initiation: Post Hoc Analysis of the FOR-DMD Trial.

Background And Objectives: Clinical trials in Duchenne muscular dystrophy (DMD) require 3-6 months of stable glucocorticoids, and the primary outcome is explored at 48-52 weeks. The factors that influence the clinical outcome assessment (COA) trajectories soon after glucocorticoid initiation are relevant for the design and analysis of clinical trials of novel drugs. We describe early COA trajectories, associated factors, and the time from glucocorticoid initiation to COA peak.

Cu@CuO/WO with photo-regulated singlet oxygen and oxygen adatoms generation for selective photocatalytic aromatic amines to imines.

Photocatalysts can absorb light and activate molecular O under mild conditions, but the generation of unsuitable reactive oxygen species often limits their use in synthesizing fine chemicals. To address this issue, we disperse 1 wt% copper on tungsten trioxide (WO) support to create an efficient catalyst for selective oxidative coupling of aromatic amines to imines under sunlight irradiation at room temperature. Copper consists of a metallic copper core and an oxide shell.

A longitudinal study of disease progression in facioscapulohumeral muscular dystrophy (FSHD).

Introduction/aims: In preparation for clinical trials, it is important to better understand how disease burden changes over time in facioscapulohumeral muscular dystrophy (FSHD) and to assess the capability of select metrics to detect these changes. This study aims to evaluate FSHD disease progression over 1 year and to examine the sensitivity of several outcome measures in detecting changes during this interval.

Methods: We conducted a 12-month prospective observational study of 41 participants with FSHD.

Reference curves of motor function outcomes in young steroid-naïve males with Duchenne muscular dystrophy.

Aim: To investigate functional motor performance in a large cohort of young steroid-naïve males with Duchenne muscular dystrophy (DMD) and typically developing males, and to develop specific reference curves for both groups. Also, to describe associations between anthropometric values and functional motor outcomes.

Method: Cross-sectional data of 196 steroid-naïve males with DMD aged 4 to 8 years and 497 typically developing males aged 2 years 6 months to 8 years were included.

Lean tissue mass measurements by dual-energy X-ray absorptiometry and associations with strength and functional outcome measures in facioscapulohumeral muscular dystrophy.

Facioscapulohumeral muscular dystrophy (FSHD) is a slowly progressive disease of skeletal muscle. Dual energy X-ray absorptiometry (DEXA) is a widely available, cost-effective and sensitive technique for measuring whole body and regional lean tissue mass and has been used in prior clinical trials in neuromuscular diseases. The Clinical Trial Readiness to Solve Barriers to Drug Development in FSHD (ReSolve) study is a prospective, longitudinal, observational multisite study.

Unraveling the Mechanism of Alkali Metal Fluoride Post-Treatment of SnO for Efficient Planar Perovskite Solar Cells.

The facile synthesis and beneficial properties of tin oxide have driven the development of efficient planar perovskite solar cells (PSCs). To increase the PSC performance, alkali salts are used to treat the SnO surface to minimize the defect states. However, the underlying mechanism of alkali cations' role in the PSCs needs further exploration.

Assessing Bulbar Function in Spinal Muscular Atrophy Using Patient-Reported Outcomes.

Background: Novel Spinal Muscular Atrophy (SMA) treatments have demonstrated improvements on motor measures that are clearly distinct from the natural history of progressive decline. Comparable measures are needed to monitor bulbar function, which is affected in severe SMA.

Objective: To assess bulbar function with patient-reported outcome measures (PROs) and determine their relationships with clinical characteristics.

High Nitrile Yields of Aerobic Ammoxidation of Alcohols Achieved by Generating O and Br Radicals over Iron-Modified TiO Photocatalysts.

Catalytic ammoxidation of alcohols into nitriles is an essential reaction in organic synthesis. While highly desirable, conducting the synthesis at room temperature is challenging, using NH as the nitrogen source, O as the oxidant, and a catalyst without noble metals. Herein, we report robust photocatalysts consisting of Fe(III)-modified titanium dioxide (Fe/TiO) for ammoxidation reactions at room temperature utilizing oxygen at atmospheric pressure, NH as the nitrogen source, and NHBr as an additive.

Milestones of progression in myotonic dystrophy type 1 and type 2.

Introduction/aims: Disease progression in myotonic dystrophy (DM) is marked by milestone events when functional thresholds are crossed. DM type 2 (DM2) is considered less severe than DM type 1 (DM1), but it is unknown whether this applies uniformly to all features. We compared the age-dependent risk for milestone events in DM1 and DM2 and tested for associations with age of onset and sex.

The influence of earnings management on bank efficiency: the case of frontier markets.

Frontier market banks fill key funding gaps in the markets they serve, resulting in increased evaluation (a positive) and earnings management (EM) (a negative). Examination of a large sample of banks in 22 frontier market countries from 2001 to 2018 reveals a downward trend in efficiency, indicating that loan quality issues persist despite increasing economic growth in the respective countries. Using stochastic frontier analysis to quantify efficiency and random effects and truncated regression to investigate the EM-efficiency relation, this study demonstrates that efficiency is negatively associated with EM.

Clinical and Genetic Characteristics in Young, Glucocorticoid-Naive Boys With Duchenne Muscular Dystrophy.

Background And Objectives: Duchenne muscular dystrophy (DMD) is a pediatric neuromuscular disorder caused by mutations in the dystrophin gene. Genotype-phenotype associations have been examined in glucocorticoid-treated boys, but there are few data on the young glucocorticoid-naive DMD population. A sample of young glucocorticoid-naive DMD boys is described, and genotype-phenotype associations are investigated.

High efficient arsenic removal by In-layer sulphur of layered double hydroxide.

High-risk arsenic contamination found in aqueous system is reported across the world and causing severe environmental issues. In this study, the Mg-Al Layered Double Hydroxide (LDH) modified by sulphur species (LDH-S) was found exhibiting high effectivity and selectivity in As(V) removal owing to the strong interaction between embedded HS and AsO. The LDH-S with Mg to Al ratio 2-1 give the best performance with As(V) adsorption capacity 40.

Health related quality of life in young, steroid-naïve boys with Duchenne muscular dystrophy.

Knowledge of health related quality of life (HRQOL) in the immediate phase following DMD diagnosis has not been well-characterized. It is important to understand HRQOL early in disease for both clinical care and studies of treatment. The relationship between parent-proxy and child self-report HRQOL and their associations with medical, psycho-social and behavioral symptoms deserve study.

Nusinersen Treatment in Adults With Spinal Muscular Atrophy.

Objective: To determine changes in motor and respiratory function after treatment with nusinersen in adults with spinal muscular atrophy (SMA) during the first two years of commercial availability in the USA.

Methods: Data were collected prospectively on adult (age >17 years at treatment initiation) SMA participants in the Pediatric Neuromuscular Clinical Research (PNCR) Network. Baseline assessments of SMA outcomes including the Expanded Hammersmith Functional Rating Scale (HFMSE), Revised Upper Limb Module (RULM), and 6-Minute Walk Test (6MWT) occurred <5 months before treatment, and post-treatment assessments were made up to 24 months after nusinersen initation.

Psychometric properties of the PEDI-CAT for children and youth with spinal muscular atrophy.

Purpose: The purpose of this study was to examine the psychometric properties of the Pediatric Evaluation of Disability Inventory-Computer Adaptive Test (PEDI-CAT) in children and youth with Spinal Muscular Atrophy (SMA).

Methods: In this prospective cross-sectional study, caregivers of children and youth with SMA completed the PEDI-CAT Daily Activities and Mobility domains. A subset of caregivers completed a questionnaire about the measure.

Nusinersen in pediatric and adult patients with type III spinal muscular atrophy.

Objective: We report longitudinal data from 144 type III SMA pediatric and adult patients treated with nusinersen as part of an international effort.

Methods: Patients were assessed using Hammersmith Functional Motor Scale Expanded (HFMSE), Revised Upper Limb Module (RULM), and 6-Minute Walk Test (6MWT) with a mean follow-up of 1.83 years after nusinersen treatment.

Mexiletine in Myotonic Dystrophy Type 1: A Randomized, Double-Blind, Placebo-Controlled Trial.

Objective: To assess mexiletine's long-term safety and effect on 6-minute walk distance in a well-defined cohort of patients with myotonic dystrophy type 1 (DM1).

Methods: We performed a randomized, double-blind, placebo-controlled trial of mexiletine (150 mg 3 times daily) to evaluate its efficacy and safety in a homogenous cohort of adult ambulatory patients with DM1. The primary outcome was change in 6-minute walk distance at 6 months.

Scoliosis Surgery Significantly Impacts Motor Abilities in Higher-functioning Individuals with Spinal Muscular Atrophy1.

Background: Weakness affects motor performance and causes skeletal deformities in spinal muscular atrophy (SMA). Scoliosis surgery decision-making is based on curve progression, pulmonary function, and skeletal maturity. Benefits include quality of life, sitting balance, and endurance.

Improved dark ambient degradation of organic pollutants by cerium strontium cobalt perovskite.

This work investigates the effect of cerium substation into strontium cobalt perovskites (CeSrCoO) for the oxidative degradation of Orange II (OII) in dark ambient conditions without the aid of any external stimulants such as light, heating or chemical additives. The OII degradation rate by CeSrCoO reached 65% in the first hour, whilst for the blank sample without cerium (SrCoO) took over 2 hr to reach the same level of OII degradation. Hence, the cerium substitution improved the catalytic activity of the perovskite material, mainly associated with the CeSrCoO perovskite phase.

Reproductive Cancer Risk Factors in Women With Myotonic Dystrophy (DM): Survey Data From the US and UK DM Registries.

Recent evidence demonstrates that women with myotonic dystrophy type 1 are at increased risk of reproductive organ tumors. However, studies of reproductive cancer risk factors in those patients are lacking. Using questionnaires, we collected and analyzed personal history information related to cancer risk factors from women enrolled in a UK and US registry for myotonic dystrophy (; DM) patients.

Relationships between mutations and neurodevelopment in dystrophinopathy.

Objective: We performed a prospective, cross-sectional analysis of neurodevelopmental concerns and psychosocial adjustment in relation to mutations in young steroid-naive boys with dystrophinopathy.

Methods: We evaluated 196 steroid-naive boys with dystrophinopathy who were enrolled in the Finding the Optimal Regimen for Duchenne Muscular Dystrophy trial. The neurodevelopmental concerns and psychosocial adjustment challenges were analyzed in relation to mutation.

Ankle bracing practices in ambulatory, corticosteroid-naive boys with Duchenne muscular dystrophy.

Introduction: Loss of ambulation in Duchenne muscular dystrophy presages scoliosis, respiratory failure, and death. Strategies to maintain ankle range of motion are employed, but little evidence exists to support these approaches and limited information is available concerning current practice.

Methods: In this study we assessed baseline bracing data from 187 boys participating in a multicenter, international clinical trial.