关键词： Axons   Mathematical model   Couplings   Extracellular   Electric potential   Brain modeling   Ephaptic coupling   tract geometry   myelinated nerve fibers   action potential   synchronization  

摘要： The study of axon tracts is important in neurophysiology. In this paper, we study axon tracts with arbitrary geometry and present a governing equation for ephaptic coupling among the constituent axons. We derive this equation and simulate it, obtaining a novel, geometry-dependent, delayed coupling phenomenon. This has relevance for how an axon tract communicates data in a neurological network.

关键词： Primary Sjogren's syndrome   Facial pain   Autonomic neuropathy   Ataxic polyneuropathy   Cranial nerve neuropathy   Carpal tunnel syndrome   Myasthenia gravis   Multiple mononeuropathy   Motor neuron syndrome   Pure motor polyneuropathy   Non-ataxic painful polyneuropathy   Polyradiculoneuropathy   Pure sensory polyneuropathy   Sensorimotor polyneuropathy   Small fiber polyneuropathy   Sural neuropathy   Trigeminal affectation  

摘要： Keywords: Primary Sjogren's syndrome; Facial pain; Autonomic neuropathy; Ataxic polyneuropathy; Cranial nerve neuropathy; Carpal tunnel syndrome; Myasthenia gravis; Multiple mononeuropathy; Motor neuron syndrome; Pure motor polyneuropathy; Non-ataxic painful polyneuropathy; Polyradiculoneuropathy; Pure sensory polyneuropathy; Sensorimotor polyneuropathy; Small fiber polyneuropathy; Sural neuropathy; Trigeminal affectation EN Primary Sjogren's syndrome Facial pain Autonomic neuropathy Ataxic polyneuropathy Cranial nerve neuropathy Carpal tunnel syndrome Myasthenia gravis Multiple mononeuropathy Motor neuron syndrome Pure motor polyneuropathy Non-ataxic painful polyneuropathy Polyradiculoneuropathy Pure sensory polyneuropathy Sensorimotor polyneuropathy Small fiber polyneuropathy Sural neuropathy Trigeminal affectation *** *** 1 02/25/21 20210301 NES 210301 Facial pain is a challenging entity in medicine, and has a wide range of differential diagnoses. Primary Sjogren's syndrome, Facial pain, Autonomic neuropathy, Ataxic polyneuropathy, Cranial nerve neuropathy, Carpal tunnel syndrome, Myasthenia gravis, Multiple mononeuropathy, Motor neuron syndrome, Pure motor polyneuropathy, Non-ataxic painful polyneuropathy, Polyradiculoneuropathy, Pure sensory polyneuropathy, Sensorimotor polyneuropathy, Small fiber polyneuropathy, Sural neuropathy, Trigeminal affectation 95, 19 2016, e3632 10.1097/md.0000000000003632 28 J. Font, J. Valls, R. Cervera, A. Pou, M. Ingelmo, F. Graus, Pure sensory neuropathy in patients with primary Sjogren's syndrome: clinical, immunological, and electromyographic findings. Sjögren's Syndrome: an undiagnosed etiology for facial pain Case series with review of neurological manifestation of Sjögren syndrome. [Extracted from the article]

关键词： CAPE   neurological disorders   pinocembrin   propolis   psychiatric disorders  

摘要： Propolis consists of a honeybee product, with a complex mix of substances that have been widely used in traditional medicine. Among several compounds present in propolis, caffeic acid phenethyl ester (CAPE), and pinocembrin emerge as two principal bioactive compounds, with benefits in a variety of body systems. In addition to its well-explored pharmacological properties, neuropharmacological activities have been poorly discussed. In an unprecedented way, the present review addresses the current finding on the promising therapeutic purposes of propolis, focusing on CAPE and pinocembrin, highlighting its use on neurological disturbance, as cerebral ischemia, neuroinflammation, convulsion, and cognitive impairment, as well as psychiatric disorders, such as anxiety and depression. In addition, we provide a critical analysis, discussion, and systematization of the molecular mechanisms which underlie these central nervous system effects. We hypothesize that the pleiotropic action of CAPE and pinocembrin, per se or associated with other substances present in propolis may result in the therapeutic activities reported. Inhibition of the pro-inflammatory cascade, antioxidant activity, and positive neurotrophic modulatory effects consist of the main molecular targets attributed to CAPE and pinocembrin in health benefits.

关键词： Palliative care   pediatric   patient-centered outcome research   outcome measure   factor analysis   questionnaire design   multicenter study   prospective study  

摘要： Background: Comprehensive outcome measurement in pediatric palliative care focusing on the entire unit of care, that is, the affected child and its family, is crucial to depict treatment effects. Despite its increasing relevance, no appropriate multidimensional outcome measures exist for the largest patient group in this field, namely children with severe neurological impairments. Aim: The aim of this study was to develop and validate a family-centered multidimensional outcome measure for pediatric palliative care patients with severe neurological impairment that encompasses the entire unit of care. Design: Based on results of a qualitative study, the questionnaire was developed by consensus-based generation of questions. It was validated in a multicenter prospective study employing exploratory and confirmatory factor analyses as well as reliability and item analyses. Setting: A total of 11 pediatric palliative care teams across Germany aided in the recruitment of study participants. Questionnaires were answered by 149 parents of children with severe neurological impairment and 157 professional caregivers. Results: The exploratory factor analysis of parent data revealed a 6-factor structure of the questionnaire representing: symptoms, the child's social participation, normalcy, social support, coping with the disease and caregiver's competencies. This structure was verified by a confirmatory factor analysis of professional caregiver data. Five separate items regarding siblings, partners, and further symptoms not applicable for all patients were added to ensure full multidimensionality. Conclusion: A validated outcome tool for severely neurologically impaired pediatric palliative care patients, the FACETS-OF-PPC, now exists. Due to its concise length and appropriate psychometric properties, it is well suited for clinical application.

关键词： Autism spectrum disorders   Gene expression profiling   Gene regulation   Genetics of the nervous system   Molecular neuroscience  

摘要： Boulting et al. profile activity-dependent gene expression and regulatory elements in human induced pluripotent stem cell-derived GABAergic neurons and uncover a possible role for calcium-responsive gene promoters of these neurons in autism risk. Neuronal activity-dependent gene expression is essential for brain development. Although transcriptional and epigenetic effects of neuronal activity have been explored in mice, such an investigation is lacking in humans. Because alterations in GABAergic neuronal circuits are implicated in neurological disorders, we conducted a comprehensive activity-dependent transcriptional and epigenetic profiling of human induced pluripotent stem cell-derived GABAergic neurons similar to those of the early developing striatum. We identified genes whose expression is inducible after membrane depolarization, some of which have specifically evolved in primates and/or are associated with neurological diseases, including schizophrenia and autism spectrum disorder (ASD). We define the genome-wide profile of human neuronal activity-dependent enhancers, promoters and the transcription factors CREB and CRTC1. We found significant heritability enrichment for ASD in the inducible promoters. Our results suggest that sequence variation within activity-inducible promoters of developing human forebrain GABAergic neurons contributes to ASD risk.

关键词： coronavirus disease‐   2019   headache   neurological symptoms   severe acute respiratory syndrome coronavirus 2   trigeminal neuralgia  

摘要： Objective To describe a case report of trigeminal neuralgia (TN) due to coronavirus disease-2019 (COVID-19). Background In March 2020, the World Health Organization declared COVID-19 as a pandemic. Respiratory system manifestations are dominant in this new disease. However, numerous case series and reviews have been published on the neurological manifestations, highlighting the potential neurotropism of the new coronavirus. Methods We describe a clinical case of TN during COVID-19 and we discuss the differential diagnosis and the potential pathogenic mechanism according to the literature. Results A 65-year-old man with general malaise and typical respiratory symptoms of COVID-19, who presented with paroxysmal lancinating pain in the right V1 trigeminal territory without other neurological symptoms. General blood test and neuroimaging study were normal. A rapid test showed positive IgG and IgM serologies for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). The patient was diagnosed with TN secondary to a viral infection by SARS-CoV-2. Facial pain resolved with the improvement of COVID-19. Conclusions The new coronavirus SARS-CoV-2 is a possible etiology of secondary TN. Nevertheless, more studies are needed to elucidate the neuropathology of this viral infection.

关键词： Argininemia   Arginase deficiency   Urea cycle disorders   Spastic diplegia  

摘要： Argininemia or hyperargininemia is a urea cycle disorder caused by deficiency of the enzyme arginase 1. It is inherited in an autosomal recessive fashion. It commonly leads to spastic diplegia in childhood, but other important features include cognitive deterioration and epilepsy. Unlike other disorders of the urea cycle, hyperammonemia is not prominent. The authors report three siblings with genetically proven argininemia who presented with diverse phenotypes but with spasticity being a common feature. Sibling 1 developed motor regression in early childhood, sibling 2 developed delayed motor milestones from early infancy, whereas sibling 3 had global developmental delay in late infancy after a period of normal development. All siblings had mild hyperammonemia only. Early recognition is imperative, not only to initiate ammonia scavenging therapy which may lead to definite clinical improvement, but also to provide genetic counselling.

摘要： Aim To report the differential diagnosis in children with progressive intellectual and neurological deterioration (PIND) in the UK. Method Since 1997 the PIND Study has searched for variant Creutzfeldt-Jakob disease (vCJD) in children, using the British Paediatric Surveillance Unit to perform prospective surveillance of those younger than 16 years with PIND. Results From May 1997 to October 2019, 2255 children meeting PIND criteria had been notified, of whom 2008 (1085 males, 923 females) had underlying diagnoses. There were over 220 different diseases, including six cases of vCJD. The numbers presenting in four age groups were: <1 year, 805 (40%);1 to 4 years inclusive, 825 (41%);5 to 9 years inclusive, 264 (13%);and 10 to 15 years inclusive, 114 (6%). The two largest ethnic groups were White and Pakistani (58.2% and 17% of diagnosed cases). The most common diseases in these two ethnic groups are shown for the four age groups. The distribution of diseases varied with age but was quite similar in White and Pakistani children. Interpretation This paper provides a unique guide to the complex differential diagnosis of childhood PIND, showing considerable differences between four age groups, but similarities between ethnic groups. The PIND Study still provides the only systematic surveillance for vCJD in children in the UK.

关键词： Blood-brain barriers   Exosomes   Extracellular vesicles   Drug delivery systems   Neurological disorders  

摘要： Cells secrete extracellular vesicles (EVs) for intercellular communication. EVs are natural nanovesicles that are surrounded by lipid bilayer for delivery of assorted cargoes for therapeutic purposes. In addition to their therapeutic roles, these vesicles are potential drug delivery systems. Exosomes are the most studied EVs as the delivery carriers that can cross the blood-brain barrier (BBB) because of their nanosize. BBB is a diffusion barrier that is selective for small molecules to transit from blood to the brain. This barrier has been an obstacle for the delivery of drugs to the brain for the treatment of neurological disorders (NDs). For efficient drug delivery, synthetic vesicles such as liposomes have been employed as carriers for delivery of therapeutic molecules in clinical practice. However, these delivery systems are not without drawbacks. Among the limitations of these drug carriers include recognition by the body as foreign particles that encounter multiple defence systems that could recognize, neutralize and eliminate them. EVs are natural vesicles that may circumvent the body defence system to remain in systemic circulation for a long time. This unique property made them excellent drug delivery vehicles for clinical application. Here I discuss the progress, challenges and future directions of EVs (especially exosomes) as vehicles for targeted delivery of drug and at the same time deliver their cargoes for regenerative purposes in NDs. Recent developments in bioengineering and microfluidic technologies, which hold promise for clinical-grade production of EVs as drug delivery systems for NDs are also highlighted.