Cultural Adaptation of the Functional External Memory Aid Tool for Use in the United Kingdom and Australia.

Purpose: The Functional External Memory Aid Tool (FEMAT) is a performance-based measure of applied cognitive-linguistic abilities and attempted use of compensatory aids and strategies (e.g., using a calendar to recall an appointment) through completion of simulated everyday living tasks.

Sexually dimorphic computational histopathological signatures prognostic of overall survival in high-grade gliomas via deep learning.

High-grade glioma (HGG) is an aggressive brain tumor. Sex is an important factor that differentially affects survival outcomes in HGG. We used an end-to-end deep learning approach on hematoxylin and eosin (H&E) scans to (i) identify sex-specific histopathological attributes of the tumor microenvironment (TME), and (ii) create sex-specific risk profiles to prognosticate overall survival.

Neuropathologically directed profiling of PRNP somatic and germline variants in sporadic human prion disease.

Creutzfeldt-Jakob Disease (CJD), the most common human prion disease, is associated with pathologic misfolding of the prion protein (PrP), encoded by the PRNP gene. Of human prion disease cases, < 1% were transmitted by misfolded PrP, ~ 15% are inherited, and ~ 85% are sporadic (sCJD). While familial cases are inherited through germline mutations in PRNP, the cause of sCJD is unknown.

A fibroblast-dependent TGF-β1/sFRP2 noncanonical Wnt signaling axis promotes epithelial metaplasia in idiopathic pulmonary fibrosis.

Reciprocal interactions between alveolar fibroblasts and epithelial cells are crucial for lung homeostasis, injury repair, and fibrogenesis, but underlying mechanisms remain unclear. To investigate, we administered the fibroblast-selective TGF-β1 signaling inhibitor epigallocatechin gallate (EGCG) to interstitial lung disease (ILD) patients undergoing diagnostic lung biopsy and conducted single-cell RNA-Seq on spare tissue. Biopsies from untreated patients showed higher fibroblast TGF-β1 signaling compared with nondisease donor or end-stage ILD tissues.

Neuropathologically-directed profiling of somatic and germline variants in sporadic human prion disease.

Creutzfeldt-Jakob Disease (CJD), the most common human prion disease, is associated with pathologic misfolding of the prion protein (PrP), encoded by the gene. Of human prion disease cases, ~1% were transmitted by misfolded PrP, ~15% are inherited, and ~85% are sporadic (sCJD). While familial cases are inherited through germline mutations in , the cause of sCJD is unknown.

SiX (X = S, Se) Single Chains and (Si-Ge)X Quaternary Alloys.

Layered or chain materials have received significant research attention owing to their interesting physical properties, which can dramatically change when the material is thinned from bulk (three-dimensional) to thin two-dimensional sheet or one-dimensional (1D) chain form. Materials with the stoichiometry AX with A = Si or Ge and X = S or Se form an especially intriguing semiconducting class. For example, bulk silicon dichalcogenides (SiX) consist of 1D chains held together by van der Waals forces.

Montreal Cognitive Assessment Scores Do Not Associate With Communication Challenges Reported by Adults With Alzheimer's Disease or Parkinson's Disease.

Purposes: Screening for cognitive-communication challenges in people with Alzheimer's disease (AD) or Parkinson's disease (PD) may benefit from multiple kinds of information about the client (e.g., patient-reported, performance-based).

Poststroke Communication Ability Predicts Patient-Informant Discrepancies in Reported Activities and Participation.

Background: has been identified by people with aphasia (PWA) as an ultimate outcome of rehabilitation and is often measured with patient-reported outcome measures (PROMs) or informant-reported measures. It is known that PWA and informants do not always produce similar scores on measures of activities and participation. However, systematic differences between PWA and informants and the causes of these differences are not well understood.

Continuous Positive Airway Pressure in the Treatment of Pediatric High Altitude Pulmonary Edema: A Case Study.

Treatment of high altitude pulmonary edema (HAPE) can be challenging and is further complicated in the pediatric patient in the prehospital environment. The following case presents a decompensating pediatric patient with HAPE in the prehospital aeromedical environment. It illustrates the potential benefit of continuous positive airway pressure (CPAP) as a treatment modality in the treatment of HAPE.

Exploring symptom clusters in mild cognitive impairment and dementia with the NIH Toolbox.

Objective: Symptom clustering research provides a unique opportunity for understanding complex medical conditions. The objective of this study was to apply a variable-centered analytic approach to understand how symptoms may cluster together, within and across domains of functioning in mild cognitive impairment (MCI) and dementia, to better understand these conditions and potential etiological, prevention, and intervention considerations.

Method: Cognitive, motor, sensory, emotional, and social measures from the NIH Toolbox were analyzed using exploratory factor analysis (EFA) from a dataset of 165 individuals with a research diagnosis of either amnestic MCI or dementia of the Alzheimer's type.

Differences in Primary Care Follow-up After Acute Care Discharge Within and Across Health Systems: a Retrospective Cohort Study.

Background: Timely primary care follow-up after acute care discharge may improve outcomes.

Objective: To evaluate whether post-discharge follow-up rates differ among patients discharged from hospitals directly affiliated with their primary care clinic (same-site), other hospitals within their health system (same-system), and hospitals outside their health system (outside-system).

Design: Retrospective cohort study.

Efficient transmission of human prion diseases to a glycan-free prion protein-expressing host.

It is increasingly evident that the association of glycans with the prion protein (PrP), a major post-translational modification, significantly impacts the pathogenesis of prion diseases. A recent bioassay study has provided evidence that the presence of PrP glycans decreases spongiform degeneration and disease-related PrP (PrPD) deposition in a murine model. We challenged (PRNPN181Q/197Q) transgenic (Tg) mice expressing glycan-free human PrP (TgGlyc-), with isolates from sporadic Creutzfeldt-Jakob disease subtype MM2 (sCJDMM2), sporadic fatal insomnia and familial fatal insomnia, three human prion diseases that are distinct but share histotypic and PrPD features.

Coats Plus Syndrome Presenting in an Adult.

To present a case of retinal vascular disease characterized primarily by capillary nonperfusion in an adult with Coats plus syndrome (CPS). : A case and its findings were analyzed. : A 38-year-old woman with a history of poliosis, thrombocytopenia, seizures, and white-matter brain lesions was referred for evaluation of bilateral blurred central vision.

1D Magnetic MX Single-Chains (M = Cr, V and X = Cl, Br, I).

Magnetic materials in reduced dimensions are not only excellent platforms for fundamental studies of magnetism, but they play crucial roles in technological advances. The discovery of intrinsic magnetism in monolayer 2D van der Waals systems has sparked enormous interest, but the single-chain limit of 1D magnetic van der Waals materials has been largely unexplored. This paper reports on a family of 1D magnetic van der Waals materials with composition MX (M = Cr, V, and X = Cl, Br, I), prepared in fully-isolated fashion within the protective cores of carbon nanotubes.

Pathological findings in autoimmune encephalitis autopsy specimens from cases of suspected prion disease.

Objective: The underlying pathology of autoimmune encephalitis is not well characterized due to the limited opportunities to study tissue specimens. Autopsy specimens available at prion surveillance centers from patients with suspected Creutzfeldt-Jakob disease offer a unique opportunity to study the pathology of autoimmune encephalitis. Our objective was to describe pathological findings of autoimmune encephalitis specimens submitted to the U.

Anisotropy and isotope effect in superconducting solid hydrogen.

Elucidating the phase diagram of solid hydrogen is a key objective in condensed matter physics. Several decades ago, it was proposed that at low temperatures and high pressures, solid hydrogen would be a metal with a high superconducting transition temperature. This transition to a metallic state can happen through the closing of the energy gap in the molecular solid or through a transition to an atomic solid.

Novel histotypes of sporadic Creutzfeldt-Jakob disease linked to 129MV genotype.

The MV1 and MV2 subtypes of sporadic Creutzfeldt-Jakob disease (sCJD) are linked to the heterozygous methionine (M)/valine (V) polymorphism at codon 129 of the prion protein (PrP) gene. MV2 is phenotypically heterogeneous, whereas MV1, due to its low prevalence, is one of the least well characterized subtypes. In this study, we investigated the biochemical properties of PrP and phenotypic expression of cases diagnosed as sCJD MV1 and MV2.

A fibroblast-dependent TGFβ1/sFRP2 noncanonical Wnt signaling axis underlies epithelial metaplasia in idiopathic pulmonary fibrosis.

Reciprocal interactions between alveolar fibroblasts and epithelial cells are crucial for lung homeostasis, injury repair, and fibrogenesis, but underlying mechanisms remain unclear. To investigate this, we administered the fibroblast-selective TGFβ1 signaling inhibitor, epigallocatechin gallate (EGCG), to Interstitial Lung Disease (ILD) patients undergoing diagnostic lung biopsy and conducted single-cell RNA sequencing on spare tissue. Unexposed biopsy samples showed higher fibroblast TGFβ1 signaling compared to non-disease donor or end-stage ILD tissues.

Development of consensus-based considerations for use of adult proxy reporting: an ISOQOL task force initiative.

Aims: Many large-scale population-based surveys, research studies, and clinical care allow for inclusion of proxy reporting as a strategy to collect outcomes when patients are unavailable or unable to provide reliable self-report. Prior work identified an absence of methodological guidelines regarding proxy reporting in adult populations, including who can serve as a proxy, and considerations for data collection, analysis, and reporting. The primary objective of this work by the ISOQOL Proxy Task Force was to review documents and clinical outcome assessment measures with respect to proxy reporting and to develop, through consensus, considerations for proxy reporting.

A novel subtype of sporadic Creutzfeldt-Jakob disease with PRNP codon 129MM genotype and PrP plaques.

The presence of amyloid kuru plaques is a pathological hallmark of sporadic Creutzfeldt-Jakob disease (sCJD) of the MV2K subtype. Recently, PrP plaques (p) have been described in the white matter of a small group of CJD (p-CJD) cases with the 129MM genotype and carrying resPrP type 1 (T1). Despite the different histopathological phenotype, the gel mobility and molecular features of p-CJD resPrP T1 mimic those of sCJDMM1, the most common human prion disease.

Severe Obesity Associated With Pituitary Corticotroph Hyperplasia and Neoplasia.

Objective: To investigate the incidence of corticotroph hyperplasia (CH) or lymphocyte infiltration in the pituitary of patients with obesity.

Methods: The pituitary and adrenal glands from 161 adult autopsies performed between 2010 and 2019 at our institution were reviewed. The clinical history, body mass index (BMI), and cause of death were recorded.

DementiaBank: Theoretical Rationale, Protocol, and Illustrative Analyses.

Purpose: Dementia from Alzheimer's disease (AD) is characterized primarily by a significant decline in memory abilities; however, language abilities are also commonly affected and may precede the decline of other cognitive abilities. To study the progression of language, there is a need for open-access databases that can be used to build algorithms to produce translational models sensitive enough to detect early declines in language abilities. DementiaBank is an open-access repository of transcribed video/audio data from communicative interactions from people with dementia, mild cognitive impairment (MCI), and controls.