Journal Article

Autoimmune psychosis (AP) and other autoimmune psychiatric syndromes (APS) are associated with central nervous system antibodies. This study investigated related magnetic resonance spectroscopic imaging (MRSI) signatures and their correlations with electroencephalography (EEG), cerebrospinal fluid (CSF), and psychometric/neuropsychological measures.Twenty-eight adults with suspected antibody-positive AP spectrum syndromes were compared with 28 matched healthy controls. [...]

The existence of linear cholesterol-recognition motifs in transmembrane domains has long been debated. Evolutionary molecular dynamics (Evo-MD) simulations-genetic algorithms guided by (coarse-grained) molecular force-fields-reveal that thermodynamic optimal cholesterol attraction in isolated alpha-helical transmembrane domains occurs when multiple consecutive lysine/arginine residues flank a short hydrophobic segment. [...]

Rehabilitation is widely recognized as an essential component in evaluating the effectiveness of a therapeutic intervention in animal models of translational spinal cord injury research. Ideally, the rehabilitation method used should be optimized for the specific injury model and severity to adequately compare the efficacy of the investigational therapy. [...]

Fatal familial insomnia (FFI) is a rare, autosomal dominant prion disease caused by a mutation in the PRNP gene, leading to the misfolding of the cellular prion protein (PrPC) into its pathogenic form (PrPSc). This results in neurodegeneration, particularly in the thalamus, a key region regulating sleep-wake cycles, which underlies the hallmark symptoms of FFI, including insomnia, autonomic dysfunctions, motor disturbances and cognitive decline. [...]

Prehospital stroke management on mobile stroke units (MSUs) shortens time to IV thrombolysis (IVT) and improves functional outcomes. Because IVT effects are time-dependent, optimizing workflows and dispatch-related processes may enhance MSU benefits. [...]

To gather comprehensive insights regarding current neuropsychological assessment practices in PRECISION-ALS, a pan-European research and industry consortium, to propose areas which can be harmonized and facilitate more robust cross-country comparisons.Representatives from PRECISION-ALS sites were surveyed with a semi-structured interview, gathering information on how people with ALS are assessed for cognitive/behavioral change, including how they are initially screened, classified as impaired/unimpaired, and followed up longitudinally. Assessment practices across PRECISION-ALS sites were summarized using descriptive analysis.Ten of the eleven PRECISION-ALS sites perform cognitive and/or behavioral screening at least once during the course of the disease, using the Edinburgh Cognitive and Behavioral ALS Screen, either for clinical or research purposes. [...]

The translation of mRNA into proteins in multicellular organisms needs to be carefully tuned to changing proteome demands in development and differentiation, while defects in translation often have a disproportionate impact in distinct cell types. Here we used inducible CRISPR interference screens to compare the essentiality of genes with functions in mRNA translation in human induced pluripotent stem cells (hiPS cells) and hiPS cell-derived neural and cardiac cells. [...]

Objective: To describe the development of a methodology to characterize hospitalizations and their relationship to amyotrophic lateral sclerosis (ALS) and provide results using this process in a phase 3 trial of reldesemtiv in ALS. Methods: ALS clinical trialists assisted in developing a classification system to determine if a hospitalization was related to ALS (HR-ALS), unrelated (HU-ALS), or if the relationship was indeterminate (HI-ALS) and this was applied by the investigators to hospitalizations in COURAGE-ALS. [...]

Background: Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) exhibit significant clinical, genetic and neuropathological abnormalities, and are regarded as belonging to a common disease spectrum, referred to as the ALS-FTD spectrum disorders. Our understanding of the underlying mechanisms of these diseases has advanced significantly, including molecular neuropathology, genetics and molecular pathophysiology. [...]