Abnormal Microstructural Development of the Cerebral Cortex in Neonates With Congenital Heart Disease Is Associated With Impaired Cerebral Oxygen Delivery.

Background Abnormal macrostructural development of the cerebral cortex has been associated with hypoxia in infants with congenital heart disease ( CHD ). Animal studies have suggested that hypoxia results in cortical dysmaturation at the cellular level. New magnetic resonance imaging techniques offer the potential to investigate the relationship between cerebral oxygen delivery and cortical microstructural development in newborn infants with CHD .

Management and 1-Year Outcomes of Patients With Newly Diagnosed Atrial Fibrillation and Chronic Kidney Disease: Results From the Prospective GARFIELD - AF Registry.

Background Using data from the GARFIELD - AF (Global Anticoagulant Registry in the FIELD -Atrial Fibrillation), we evaluated the impact of chronic kidney disease ( CKD ) stage on clinical outcomes in patients with newly diagnosed atrial fibrillation ( AF ). Methods and Results GARFIELD - AF is a prospective registry of patients from 35 countries, including patients from Asia (China, India, Japan, Singapore, South Korea, and Thailand). Consecutive patients enrolled (2013-2016) were classified with no, mild, or moderate-to-severe CKD , based on the National Kidney Foundation's Kidney Disease Outcomes Quality Initiative guidelines.

Species conservation profiles of a random sample of world spiders IV: Scytodidae to Zoropsidae.

Background: The IUCN Red List of Threatened Species is the most widely used information source on the extinction risk of species. One of the uses of the Red List is to evaluate and monitor the state of biodiversity and a possible approach for this purpose is the Red List Index (RLI). For many taxa, mainly hyperdiverse groups, it is not possible within available resources to assess all known species.

Two new species of Lafer (Carabidae, ) from China, with a key to all known Chinese species in this subgenus.

Two new species (Coleoptera, Carabidae) in the subgenus Feroperis Lafer, 1979 are described from Zhangguangcai Mountain, northeastern China: Pterostichus (Feroperis) silvestris Sun & Shi, and Pterostichus (Feroperis) maryseae Sun & Shi, Detailed descriptions and illustrations of the male endophallus and female reproductive tracts for these new species are provided, along with a key to the five known species of the subgenus in China.

The Outcome of Dopamine Dysregulation Syndrome in Parkinson's Disease: A Retrospective Postmortem Study.

Introduction: We have performed a retrospective analysis of the frequency and relation to treatment of dopamine dysregulation syndrome (DDS) using the Queen Square Brain Bank (QSBB) database.

Methods: A search of the QSBB database for consecutive cases donated between 2005 and 2016 with a pathological diagnosis of Parkinson's disease was performed.

Results: DDS was present in 8.

Clinical Rating Scales for Urinary Symptoms in Parkinson Disease: Critique and Recommendations.

Background: The prevalence of lower urinary tract symptoms (LUTS) is high in Parkinson's disease (PD). These problems negatively affect quality of life and include both storage and voiding problems. The International Parkinson and Movement Disorder Society established a task force to review clinical rating scales/questionnaires for the assessment of urinary symptoms in PD.

Species conservation profiles of a random sample of world spiders III: Oecobiidae to Salticidae.

Background: The IUCN Red List of Threatened Species is the most widely used information source on the extinction risk of species. One of the uses of the Red List is to evaluate and monitor the state of biodiversity and a possible approach for this purpose is the Red List Index (RLI). For many taxa, mainly hyperdiverse groups, it is not possible within available resources to assess all known species.

INCA (Peru) Study: Impact of Non-Invasive Cardiac Magnetic Resonance Assessment in the Developing World.

Background Advanced cardiac imaging permits optimal targeting of cardiac treatment but needs to be faster, cheaper, and easier for global delivery. We aimed to pilot rapid cardiac magnetic resonance ( CMR ) with contrast in a developing nation, embedding it within clinical care along with training and mentoring. Methods and Results A cross-sectional study of CMR delivery and clinical impact assessment performed 2016-2017 in an upper middle-income country.

Multimodal computational neocortical anatomy in pediatric hippocampal sclerosis.

Objective: In contrast to adult cohorts, neocortical changes in epileptic children with hippocampal damage are not well characterized. Here, we mapped multimodal neocortical markers of epilepsy-related structural compromise in a pediatric cohort of temporal lobe epilepsy and explored how they relate to clinical factors.

Methods: We measured cortical thickness, gray-white matter intensity contrast and intracortical FLAIR intensity in 22 patients with hippocampal sclerosis (HS) and 30 controls.

Male-biased gene expression resolves sexual conflict through the evolution of sex-specific genetic architecture.

Many genes are subject to contradictory selection pressures in males and females, and balancing selection resulting from sexual conflict has the potential to substantially increase standing genetic diversity in populations and thereby act as an important force in adaptation. However, the underlying causes of sexual conflict, and the potential for resolution, remains hotly debated. Using transcriptome-resequencing data from male and female guppies, we use a novel approach, combining patterns of genetic diversity and intersexual divergence in allele frequency, to distinguish the different scenarios that give rise to sexual conflict, and how this conflict may be resolved through regulatory evolution.

Presymptomatic white matter integrity loss in familial frontotemporal dementia in the GENFI cohort: A cross-sectional diffusion tensor imaging study.

Objective: We aimed to investigate mutation-specific white matter (WM) integrity changes in presymptomatic and symptomatic mutation carriers of the ,, and mutations by use of diffusion-weighted imaging within the Genetic Frontotemporal dementia Initiative (GENFI) study.

Methods: One hundred and forty mutation carriers (54 , 30 , 56 ), 104 presymptomatic and 36 symptomatic, and 115 noncarriers underwent 3T diffusion tensor imaging. Linear mixed effects models were used to examine the association between diffusion parameters and years from estimated symptom onset in ,, and mutation carriers versus noncarriers.

Navigational cue effects in Alzheimer's disease and posterior cortical atrophy.

Objective: Deficits in spatial navigation are characteristic and disabling features of typical Alzheimer's disease (tAD) and posterior cortical atrophy (PCA). Visual cues have been proposed to mitigate such deficits; however, there is currently little empirical evidence for their use.

Methods: The effect of visual cues on visually guided navigation was assessed within a simplified real-world setting in individuals with tAD ( = 10), PCA ( = 8), and healthy controls ( = 12).

Secular changes in severity of intellectual disability in tuberous sclerosis complex: A reflection of improved identification and treatment of epileptic spasms?

Tuberous sclerosis complex (TSC) is a multisystem genetic disorder caused by mutations in or . Epilepsy occurs in 80%-90% of affected individuals during their lifetime, and up to one-third of children with TSC will develop epileptic (infantile) spasms, for which vigabatrin has been shown to be particularly effective. Epilepsy severity and epileptic spasms are consistent markers of risk for the development of intellectual impairment in TSC.

Optimal clinical management of children receiving dietary therapies for epilepsy: Updated recommendations of the International Ketogenic Diet Study Group.

Ketogenic dietary therapies (KDTs) are established, effective nonpharmacologic treatments for intractable childhood epilepsy. For many years KDTs were implemented differently throughout the world due to lack of consistent protocols. In 2009, an expert consensus guideline for the management of children on KDT was published, focusing on topics of patient selection, pre-KDT counseling and evaluation, diet choice and attributes, implementation, supplementation, follow-up, side events, and KDT discontinuation.

Pathogenic potential of antibodies to the GABA receptor.

GABA receptor (GABAR) autoantibodies have been detected in the serum of immunotherapy-responsive patients with autoimmune encephalitis. This study aimed to investigate the effect of immunoglobulin G (IgG) from a patient with GABAR antibodies on primary neuronal cultures and acute slices of entorhinal cortex. Primary hippocampal neuronal cultures were incubated with serum immunoglobulin from patients with GABAR or AMPA receptor (AMPAR) antibodies for up to 72 h to investigate their effect on receptor surface expression.

Magnitude and factors associated with nonadherence to antiepileptic drug treatment in Africa: A cross-sectional multisite study.

Objectives: The epilepsy treatment gap is large in low- and middle-income countries, but the reasons behind nonadherence to treatment with antiepileptic drugs (AEDs) across African countries remain unclear. We investigated the extent to which AEDs are not taken and associated factors in people with active convulsive epilepsy (ACE) identified in cross-sectional studies conducted in five African countries.

Methods: We approached 2,192 people with a confirmed diagnosis of ACE for consent to give blood voluntarily.

Donepezil enhances understanding of degraded speech in Alzheimer's disease.

Auditory dysfunction under complex, dynamic listening conditions is a clinical hallmark of Alzheimer's disease (AD) but challenging to measure and manage. Here, we assessed understanding of sinewave speech (a paradigm of degraded speech perception) and general cognitive abilities in 17 AD patients, before and following a 10 mg dose of donepezil. Relative to healthy older individuals, patients had impaired sinewave speech comprehension that was selectively ameliorated by donepezil.

Tetrabenazine Versus Deutetrabenazine for Huntington's Disease: Twins or Distant Cousins?

Background: Tetrabenazine is the only US Food and Drug Administration-approved drug for Huntington's disease, and deutetrabenazine was recently tested against placebo. A switching-trial from tetrabenazine to deutetrabenazine is underway, but no head-to-head, blinded, randomized controlled trial is planned. Using meta-analytical methodology, the authors compared these molecules.

Neurite dispersion: a new marker of multiple sclerosis spinal cord pathology?

Objective: Conventional magnetic resonance imaging (MRI) of the multiple sclerosis spinal cord is limited by low specificity regarding the underlying pathological processes, and new MRI metrics assessing microscopic damage are required. We aim to show for the first time that (i.e.

Body Mass Index Decline Is Related to Spinocerebellar Ataxia Disease Progression.

Background: Spinocerebellar ataxias (SCAs) are dominantly inherited, progressive ataxia disorders. Disease progression could be preceded by weight loss.

Objectives: We aimed to study the course of weight loss in patients who had the most common SCAs (SCA1, SCA2 SCA3, and SCA6).

Characterizing QT interval prolongation in early clinical development: a case study with methadone.

Recently, we have shown how pharmacokinetic-pharmacodynamic (PKPD) modeling can be used to assess the probability of QT interval prolongation both in dogs and humans. A correlation between species has been identified for a drug-specific parameter, making it possible to prospectively evaluate nonclinical signals. Here, we illustrate how nonclinical data on methadone can be used to support the evaluation of dromotropic drug effects in humans.