Practical Approach to Longitudinal Neurologic Care of Adults With X-Linked Adrenoleukodystrophy and Adrenomyeloneuropathy.

Although X-linked adrenoleukodystrophy (ALD) has historically been considered a childhood disease managed by pediatric neurologists, it is one of the most common leukodystrophies diagnosed in adulthood. An increase in both male and female adults reaching diagnosis due to familial cases identified by state newborn screening panels and more widespread use of genetic testing results in a large cohort of presymptomatic or early symptomatic adults. This population is in urgent need of standardized assessments and follow-up care.

Student preference for and performance in fixed- versus mixed-duration schedules.

Duration schedules of reinforcement for continuous behavior abide by several preexisting operant behavioral economic equations for reinforcer cost, otherwise known as price, and consumption. Duration schedules require behaviors to occur for a set duration of time prior to accessing reinforcement, unlike interval schedules that produce reinforcement after the first instance of a behavior after a given period. Despite extensive examples of naturally occurring duration schedules, translational research regarding duration schedules is quite limited.

Hospitals That Serve Many Black Patients Have Lower Revenues and Profits: Structural Racism in Hospital Financing.

Background: Care for Black patients is concentrated at a relatively small proportion of all US hospitals. Some previous studies have documented quality deficits at Black-serving hospitals, which may be due to inequities in financial resources for care.

Objective: To assess disparities in funding between hospitals associated with the proportion of Black patients that they serve.

Abuse Potential of Lemborexant, a Dual Orexin Receptor Antagonist, Compared With Zolpidem and Suvorexant in Recreational Sedative Users.

Background: Lemborexant (LEM) is a dual orexin receptor antagonist approved for the treatment of insomnia in adults in multiple countries including the the United States, Japan, Canada, Australia and several Asian countries.

Procedures: This was a randomized, single-dose, single-center, double-blind, active-control, 6-way crossover study to evaluate LEM abuse potential. The study assessed oral doses of LEM 10 mg (LEM10), 20 mg (LEM20), and 30 mg (LEM30) compared with placebo (PBO), zolpidem (ZOL) immediate release 30 mg, and suvorexant (SUV) 40 mg.

Acute Cognitive Effects of the Dual Orexin Receptor Antagonist Lemborexant Compared With Suvorexant and Zolpidem in Recreational Sedative Users.

Purpose/background: As part of a human abuse potential (HAP) study of lemborexant (LEM), the effects of therapeutic (LEM 10 mg), and supratherapeutic doses of LEM 20 mg and LEM 30 mg on cognition and psychomotor performance were compared with placebo (PBO) and supratherapeutic doses of zolpidem (ZOL) 30 mg and suvorexant (SUV) 40 mg. Subjects (n = 32) were healthy, nondependent, recreational sedative users able to discriminate the effects of both SUV and ZOL from PBO on subjective drug measures.

Methods/procedures: The human abuse potential study was a single-dose, randomized, double-blind, PBO-controlled, 6-way crossover study.

Signatures of GVHD and relapse after posttransplant cyclophosphamide revealed by immune profiling and machine learning.

The key immunologic signatures associated with clinical outcomes after posttransplant cyclophosphamide (PTCy)-based HLA-haploidentical (haplo) and HLA-matched bone marrow transplantation (BMT) are largely unknown. To address this gap in knowledge, we used machine learning to decipher clinically relevant signatures from immunophenotypic, proteomic, and clinical data and then examined transcriptome changes in the lymphocyte subsets that predicted major posttransplant outcomes. Kinetics of immune subset reconstitution after day 28 were similar for 70 patients undergoing haplo and 75 patients undergoing HLA-matched BMT.

Advancing equity, diversity, and inclusion in the American College of Neuropsychopharmacology (ACNP): advances, challenges, and opportunities to accelerate progress.

It is increasingly accepted that higher levels of excellence and innovation in research can be achieved by organizations that promote equity, diversity, and inclusion across several domains including ethnicity and gender. The purpose of this commentary is to provide an overview of the methods used to increase diversity within ACNP, as well as recommendations for accelerating progress. Annual membership surveys confirm increases in female membership and leadership positions, slower but encouraging signals for “Asian” and “Hispanic” members, and less progress for African American and other ethnic populations.

Outcomes and Predictors of Wound Healing among Patients with Complex Diabetic Foot Wounds Treated with a Dermal Regeneration Template (Integra).

Background: The utility of dermal regeneration templates for treating high-risk diabetic foot wounds is unclear. The authors report wound healing and major amputation outcomes among a cohort of diabetic patients with complex diabetic foot wounds treated in a multidisciplinary setting.

Methods: All patients with complex diabetic foot wounds treated with a dermal regeneration template (March of 2013 to February of 2019) were captured in a prospective institutional database.

Characterization of Allele-Specific Regulation of Telomerase Reverse Transcriptase in Promoter Mutant Thyroid Cancer Cell Lines.

Telomerase reverse transcriptase () promoter mutations play a role in carcinogenesis and are found in both tumors and cancer cell lines. promoter methylation, transcription factor binding, chromatin remodeling, and alternative splicing are also known to play an integral role in regulation. Using nanopore Cas9 targeted sequencing, we characterized allele-specific methylation in thyroid cancer cell lines heterozygous for the promoter mutation.

Context influences preference for and level of physical activity of adolescents with intellectual and developmental disabilities.

The purpose of the current study was to replicate and extend Hustyi, Normand, Larson, and Morley (2012) by determining the effects of different contexts on physical activity displayed by adolescents with intellectual and developmental disabilities, and determining preference for various activities. Results indicated that an exergaming condition produced the highest levels of activity. Results of a preference assessment indicated that 2 out of 3 participants preferred the physical activity context to the sedentary.

Are we guilty of errors of omission on the potential role of electronic nicotine delivery systems as less harmful substitutes for combusted tobacco use?

Two of the more controversial tobacco control and regulatory strategies in recent years are the nicotine reduction and tobacco harm reduction (THR) strategies. They have become inextricably intertwined as a successful nicotine reduction policy might only be possible in an environment in which alternative, noncombusted forms of nicotine like electronic nicotine delivery systems (ENDS) are available to address the needs of those who were unable or unwilling to completely give up nicotine. Unfortunately, ENDS have emerged as particularly controversial, in part, because they are the first product to carry reduced risk potential while being broadly appealing to cigarette smokers across demographic groups and subpopulations, and to a much smaller extent nonsmokers including, and most controversial, adolescents.

Supports and Barriers to the Provision of Human Milk by Mothers of African American Preterm Infants.

Background: Mother's own milk (MOM) provides significant health benefits to very low birth-weight infants (VLBW, ≤1500 g). However, 60% of African American (AA) women initiate lactation, and less than 35% provide MOM 6 months following the birth of their infant. Previous research focuses on term infants and is not specific to AA mothers of VLBW infants.

Redox Mechanisms in Neurodegeneration: From Disease Outcomes to Therapeutic Opportunities.

Significance: Once considered to be mere by-products of metabolism, reactive oxygen, nitrogen and sulfur species are now recognized to play important roles in diverse cellular processes such as response to pathogens and regulation of cellular differentiation. It is becoming increasingly evident that redox imbalance can impact several signaling pathways. For instance, disturbances of redox regulation in the brain mediate neurodegeneration and alter normal cytoprotective responses to stress.

Comprehensive systematic review summary: Treatment of cerebellar motor dysfunction and ataxia: Report of the Guideline Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology.

Objective: To systematically review evidence regarding ataxia treatment.

Methods: A comprehensive systematic review was performed according to American Academy of Neurology methodology.

Conclusions: For patients with episodic ataxia type 2, 4-aminopyridine 15 mg/d probably reduces ataxia attack frequency over 3 months (1 Class I study).

Functional analysis and treatment of problem behavior related to mands for rearrangement.

This study is a systematic replication of a functional analysis (FA) of the relation between mands and problem behavior. We extended treatment approaches for this problem behavior function, and describe the treatment of problem behavior related to mands for rearrangement demonstrated by a 12-year-old girl with autism spectrum disorder and Smith-Magenis syndrome. The mands consisted of requests for others to change their body positioning or proximity, or rearrange items back to their original position.

Noncontingent reinforcement for the treatment of severe problem behavior: An analysis of 27 consecutive applications.

Noncontingent reinforcement (NCR) is a commonly used treatment for severe problem behavior displayed by individuals with intellectual and developmental disabilities. The current study sought to extend the literature by reporting outcomes achieved with 27 consecutive applications of NCR as the primary treatment for severe problem behavior. All applications of NCR were included regardless of treatment outcome to minimize selection bias favoring successful cases.

Telomere longitudinal shortening as a biomarker for dementia status of adults with Down syndrome.

Previous studies have suggested that Alzheimer's disease (AD) causes an accelerated shortening of telomeres, the ends of chromosomes consisting of highly conserved TTAGGG repeats that, because of unidirectional 5'-3' DNA synthesis, lose end point material with each cell division. Our own previous work suggested that telomere length of T-lymphocytes might be a remarkably accurate biomarker for "mild cognitive impairment" in adults with Down syndrome (MCI-DS), a population at dramatically high risk for AD. To verify that the progression of cognitive and functional losses due to AD produced this observed telomere shortening, we have now examined sequential changes in telomere length in five individuals with Down syndrome (3F, 2M) as they transitioned from preclinical AD to MCI-DS (N = 4) or dementia (N = 1).