Neuroprotective effects of ellorarxine in neuronal models of degeneration.

Introduction: Retinoic acid (RA) was first recognised to be important for the central nervous system (CNS) in its developmental regulatory role and, given this action, it has been proposed in the adult CNS to regulate plasticity and promote regeneration. These types of roles have included support of neurogenesis, induction of neurite outgrowth, and protection from neuronal death. These functions are predominantly mediated by the retinoic acid receptor (RAR) transcription factor, and hence agonists for the RARs have been tested in a variety of models of neurodegeneration.

Evaluation of a Synthetic Retinoid, Ellorarxine, in the NSC-34 Cell Model of Motor Neuron Disease.

Amyotrophic lateral sclerosis (ALS) is the most common motor neuron disease worldwide and is characterized by progressive muscle atrophy. There are currently two approved treatments, but they only relieve symptoms briefly and do not cure the disease. The main hindrance to research is the complex cause of ALS, with its pathogenesis not yet fully elucidated.

Operational description of rare diseases: a reference to improve the recognition and visibility of rare diseases.

Improving health and social equity for persons living with a rare disease (PLWRD) is increasingly recognized as a global policy priority. However, there is currently no international alignment on how to define and describe rare diseases. A global reference is needed to establish a mutual understanding to inform a wide range of stakeholders for actions.

Predicting Intervention Use in Youth with Rare Variants in Autism-Associated Genes.

Specialized multidisciplinary supports are important for long-term outcomes for autistic youth. Although family and child factors predict service utilization in autism, little is known with respect to youth with rare, autism-associated genetic variants, who frequently have increased psychiatric, developmental, and behavioral needs. We investigate the impact of family factors on service utilization to determine whether caregiver (autistic features, education, income) and child (autistic features, sex, age, IQ, co-occurring conditions) factors predicted service type (e.

Post-operative antibiotic prophylaxis in spine surgery patients with thoracolumbar drains: A meta analysis.

Objective: Closed-suction drains are commonly placed after thoracolumbar surgery to reduce the risk of post-operative hematoma and neurologic deterioration, and may stay in place for a longer period of time if output remains high. Prolonged maintenance of surgical site drains, however, is associated with an increased risk of surgical site infection (SSI). The present study aims to examine the literature regarding extended duration (≥24 h) prophylactic antibiotic use in patients undergoing posterior thoracolumbar surgery with closed-suction drainage.

Shared and divergent mental health characteristics of ADNP-, CHD8- and DYRK1A-related neurodevelopmental conditions.

Background: Neurodevelopmental conditions such as intellectual disability (ID) and autism spectrum disorder (ASD) can stem from a broad array of inherited and de novo genetic differences, with marked physiological and behavioral impacts. We currently know little about the psychiatric phenotypes of rare genetic variants associated with ASD, despite heightened risk of psychiatric concerns in ASD more broadly. Understanding behavioral features of these variants can identify shared versus specific phenotypes across gene groups, facilitate mechanistic models, and provide prognostic insights to inform clinical practice.

Stereoelectroencephalography in the setting of a previously implanted responsive neural stimulation device: illustrative case.

Background: Responsive neural stimulation (RNS) is a relatively novel procedure for drug-resistant epilepsy, which involves implantation of a device into the skull and brain. As more devices are implanted, there may be an increasing need to perform intracranial electrocorticography in implant patients with persistent seizures. Given the device location, imaging difficulties with implanted devices, and other technical hurdles, stereoelectroencephalography (SEEG) could be especially challenging.

Influence of CrossFit and Deep End Fitness training on mental health and coping in athletes.

Physical exercise is known to improve mental health. Athletes can experience unique physical and emotional stressors, which can deteriorate mental health and cognitive function. Training apathy can lead to cognitive dissonance and further degrade performance by promoting maladaptive, avoidance coping strategies.

Bullseye Analysis: A Fluorescence Microscopy Technique to Detect Local Changes in Intracellular Reactive Oxygen Species (ROS) Production.

Reactive oxygen species (ROS) are naturally produced compounds that play important roles in cell signaling, gene regulation, and biological defense, including involvement in the oxidative burst that is central to the anti-microbial actions of macrophages. However, these highly reactive, short-lived radical species also stimulate cells to undergo programmed cell death at high concentrations, as well as causing detrimental effects such as oxidation of macromolecules at more moderate levels. Imaging ROS is highly challenging, with many researchers working on the challenge over the past 10-15 years without producing a definitive method.

Per- and Polyfluoroalkyl Substances and Mercury in Arctic Alaska Coastal Fish of Subsistence Importance.

Per- and polyfluoroalkyl substances (PFAS) and mercury (Hg) are harmful compounds that are widely present in the environment, partly due to spills and atmospheric pollution. The presence of PFAS and Hg in the tissues of animals that are harvested by rural and Indigenous Alaskans is of great concern, yet fish in Arctic Alaska have not previously been assessed for concentrations of PFAS. Fish species of subsistence and recreational importance were collected from nearshore Beaufort and Chukchi Sea, Alaska habitats and assessed for PFAS and total mercury concentrations [THg].

Comparison of Ambulatory Quality Measures Between Shared Practice Panels and Independent Practice Panels.

Objective: To assess for differences in patient care outcomes in the primary care setting for patients assigned to an independent practice panel (IPP) or a shared practice panel (SPP).

Patients And Methods: We retrospectively reviewed the electronic health records of patients of 2 Mayo Clinic family medicine primary care clinics from January 1, 2019 to December 31, 2019. Patients were assigned to either an IPP (physician or advanced practice provider [APP]) or an SPP (physician and ≥1 APP).

A Mysterious Bleed: A Case of Spontaneous Spinal Epidural Hematoma After Atrial Fibrillation Ablation.

Spontaneous spinal epidural hematoma is an extraordinarily rare condition for which there are only a few hundred documented cases, none in relation to cardiac ablation procedures. This case describes a 71-year-old woman who develops lower extremity numbness and weakness due to spontaneous spinal epidural hematoma after an atrial fibrillation ablation. ().

Virtual Accessible Bilingual Conference Planning: The Parks Accessibility Conference.

Virtual events have become more popular recently, and while these events have the potential to be inclusive to a broader range of attendees, there is limited information available on how to plan and deliver a virtual, accessible, and bilingual event. The objective of this paper is to share how our team planned and delivered a virtual conference that was fully bilingual and accessible to individuals with disabilities by incorporating closed captions, sign language interpretation, language interpretation (audio), regularly scheduled breaks, and a multi-sensory experience. We describe our approaches to planning the conference, such as including individuals with disabilities in decision-making, selecting virtual conference platforms, captioners, and interpreters, and how we incorporated a multi-sensory experience.

Impact of restricted access to, and low awareness of, mexiletine on people with myotonia: a real-world European survey.

Although mexiletine effectively treats myotonia, supply disruptions affected Europe between 2008-2018. MyoPath was a mixed-methods, cross-sectional, market research survey conducted January-June 2018 to evaluate consequences of limited access to/awareness of mexiletine in people with myotonia. Part A: qualitative structured interviews (clinicians; advocates for adult patients); Part B: quantitative online questionnaire completed by people with self-reported history of myotonia.

Between a Rock and a Hard Place: COVID-19 Lessons Learned From Providers Rounding at Skilled Nursing Facilities in the Rural Midwest.

On March 11, 2020, coronavirus disease 2019 (COVID-19) was classified as a pandemic, setting in motion unprecedented practice changes across the healthcare industry. Never was this more evident than in Skilled Nursing Facilities (SNFs). SNFs were tested on multiple fronts, requiring innovation and perseverance at levels never before seen.

A Survey of the Challenges Faced by Individuals with Disabilities and Unpaid Caregivers during the COVID-19 Pandemic.

The COVID-19 pandemic negatively affected many individuals. In particular, it is likely that individuals with disabilities and unpaid caregivers were disproportionately affected, however, its exact impact is largely unknown. The primary objective of this work was to identify challenges faced by individuals with disabilities and unpaid caregivers.

A computational tool to accurately and quickly predict F NMR chemical shifts of molecules with fluorine-carbon and fluorine-boron bonds.

We report the evaluation of density-functional-theory (DFT) based procedures for predicting F NMR chemical shifts at modest computational cost for a range of molecules with fluorine bonds, to be used as a tool for assisting the characterisation of reaction intermediates and products and as an aid to identifying mechanistic pathways. The results for a balanced learning set of molecules were then checked using two further testing sets, resulting in the recommendation of the ωB97XD/aug-cc-pvdz DFT method and basis set as having the best combination of accuracy and computational time, with a RMS error of 3.57 ppm.

Systematic Review and Meta-Analysis of Responsive Neurostimulation in Epilepsy.

Background: Neuromodulatory implants provide promising alternatives for patients with drug-resistant epilepsy (DRE) in whom resective or ablative surgery is not an option. Responsive neurostimulation (RNS) operates a unique "closed-loop" system of electrocorticography-triggered stimulation for seizure control. A comprehensive review of the current literature would be valuable to guide clinical decision-making regarding RNS.

Contributions and perspectives of Indigenous Peoples to the study of mercury in the Arctic.

Arctic Indigenous Peoples are among the most exposed humans when it comes to foodborne mercury (Hg). In response, Hg monitoring and research have been on-going in the circumpolar Arctic since about 1991; this work has been mainly possible through the involvement of Arctic Indigenous Peoples. The present overview was initially conducted in the context of a broader assessment of Hg research organized by the Arctic Monitoring and Assessment Programme.

A Rare Case of Creutzfeldt-Jakob Disease With Alcohol Use Disorder and Review of Literature.

Sporadic Creutzfeldt-Jakob disease (CJD) is a rare neurodegenerative disorder, accounting for a majority of the sporadic prion disease burden. This disorder rapidly progresses and is often fatal with no known cure. Initial diagnosis may be delayed due to its varied presentations, which can include psychiatric changes (behavioural and mood variances), visual and auditory hallucinations, cerebellar dysfunction, and pain, occurring in isolation in many cases.

Cross-Sectional Analysis of US Health Insurance Payer Policies for Humanitarian Device Exemption Indications for Deep Brain Stimulation.

Background: The US Food and Drug Administration (FDA) has granted deep brain stimulation (DBS) approval under the humanitarian device exemption (HDE) pathway for both dystonia and obsessive-compulsive disorder (OCD). However, recent reports from the community of functional neurosurgeons suggest that insurance authorization remains a disproportionate barrier to OCD DBS implantation despite both conditions having similar support from the FDA. This cross-sectional study of health insurance policies quantifies the current payer landscape for these two interventions.

Heterogeneous ketonic decarboxylation of dodecanoic acid: studying reaction parameters.

Ketonic decarboxylation has gained significant attention in recent years as a pathway to reduce the oxygen content within biomass-derived oils, and to produce sustainable ketones. The reaction is base catalysed, with MgO an economic, accessible and highly basic heterogeneous catalyst. Here we use MgO to catalyse the ketonic decarboxylation of dodecanoic acid to form 12-tricosanone at moderate temperatures (250 °C, 280 °C and 300 °C) with low catalyst loads of 1% (w/w), 3% (w/w) and 5% (w/w) with respect to the dodecanoic acid, with a reaction time of 1 hour under batch conditions.

Synthetic Diphenylacetylene-Based Retinoids Induce DNA Damage in Chinese Hamster Ovary Cells without Altering Viability.

All-trans-retinoic acid (ATRA), the active metabolite of vitamin A, plays a pivotal role in cell differentiation, proliferation and embryonic development. It is an effective therapy for dermatological disorders and malignancies. ATRA is prone to isomerization and oxidation, which can affect its activity and selectivity.