1,224,301 results match your criteria Encephalopathy Hypertensive


Therapeutic Hypothermia in Brazil: A MultiProfessional National Survey.

Am J Perinatol 2018 Dec 15. Epub 2018 Dec 15.

Department of Pediatrics, Neonatal Division, McGill University Health Center, Montreal, Quebec, Canada.

Objective:  To determine the rate of therapeutic hypothermia (TH) use, current practices, and long-term follow-up.

Study Design:  Prospective cross-sectional national survey with 19 questions related to the assessment of hypoxic-ischemic encephalopathy (HIE) and TH practices. An online questionnaire was made available to health care professionals working in neonatal care in Brazil. Read More

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December 2018
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Primary cultured neuronal networks and type 2 diabetes model mouse fatty liver tissues in aqueous liquid observed by atmospheric SEM (ASEM): Staining preferences of metal solutions.

Micron 2018 Nov 23;118:9-21. Epub 2018 Nov 23.

Biomedical Research Institute, National Institute of Industrial Science and Technology (AIST), Higashi 1-1-1, Tsukuba, Ibaraki, 305-8566, Japan. Electronic address:

Neural networking, including axon targeting and synapse formation, is the basis of various brain functions, including memory and learning. Diabetes-mellitus affects peripheral nerves and is known to cause fatty liver disease. Electron microscopy (EM) provides the resolution required to observe changes in fine subcellular structures caused by such physiological and pathological processes, but samples are observed in vacuum. Read More

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November 2018

Patch spaces and fusion strategies in patch-based label fusion.

Comput Med Imaging Graph 2018 Dec 6;71:79-89. Epub 2018 Dec 6.

DTIC, Universitat Pompeu Fabra, Barcelona, Spain.

In the field of multi-atlas segmentation, patch-based approaches have shown promising results in the segmentation of biomedical images. In the most common approach, registration is used to warp the atlases to the target space and then the warped atlas labelmaps are fused into a consensus segmentation based on local appearance information encoded in form of patches. The registration step establishes spatial correspondence, which is important to obtain anatomical priors. Read More

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December 2018

Autoimmune encephalitis following alemtuzumab treatment of multiple sclerosis.

Mult Scler Relat Disord 2018 Dec 3;28:31-33. Epub 2018 Dec 3.

Department of Neurology, St Vincent's Hospital, 390 Victoria St, Darlinghurst, Sydney, NSW 2010, Australia.

Secondary autoimmune disorders are a recognised complication of alemtuzumab treatment for multiple sclerosis. We report a case of autoimmune encephalitis manifesting as a polymorphic epilepsia partialis continua / status epilepticus seven months after the second course of alemtuzumab in a patient with previous autoimmune hypothyroidism and immune thrombocytopenic purpura. An MRI revealed multifocal cortical abnormalities and neuronal loss was evident on biopsy. Read More

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December 2018
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An enzyme-based electrochemical biosensor probe with sensitivity to detect astrocytic versus glioma uptake of glutamate in real time in vitro.

Biosens Bioelectron 2018 Nov 16;126:751-757. Epub 2018 Nov 16.

Biomedical Engineering, PO Box 10157, Ruston, LA 71272, USA; Center for Biomedical Engineering and Rehabilitation Sciences, Louisiana Tech University, PO Box 10157, Ruston, LA 71272, USA. Electronic address:

Glutamate, a major excitatory neurotransmitter in the central nervous system, is essential for regulation of thought, movement, memory, and other higher functions controlled by the brain. Dysregulation of glutamate signaling is associated with severe neuropathological conditions, such as epilepsy, and glioma, a form of brain cancer. Glutamate signals are currently detected by several types of neurochemical probes ranging from microdialysis-based to enzyme-based carbon fiber microsensors. Read More

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November 2018
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Reduction of cerebral blood flow in community-based adults with subclinical cerebrovascular atherosclerosis: A 3.0T magnetic resonance imaging study.

Neuroimage 2018 Dec 12. Epub 2018 Dec 12.

Center for Biomedical Imaging Research, Department of Biomedical Engineering, School of Medicine, Tsinghua University, Beijing, China. Electronic address:

Reduction in cerebral blood flow (CBF), one of the major metrics for cerebral perfusion, is associated with many brain disorders. Therefore, early characterization of CBF prior to occurrence of symptoms is essential for prevention of cerebral ischemic disorders. We hypothesized that large artery atherosclerosis might be a potential indicator for decline in cerebral perfusion. Read More

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December 2018
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Alpha-synuclein is highly prone to distribution in the hippocampus and midbrain in tree shrews, and its fibrils seed Lewy body-like pathology in primary neurons.

Exp Gerontol 2018 Dec 12. Epub 2018 Dec 12.

Institute of Medical Biology, Chinese Academy of Medical Sciences, Kunming 650118, China; Medical Primate Research Center & Neuroscience Center, Chinese Academy of Medical Sciences, Beijing 100005, China; Yunnan Key Laboratory of Vaccine Research Development on Severe Infectious Diseases, Kunming 650118, China. Electronic address:

The Chinese tree shrew (TS) has many unique advantages that make it suitable for use as an experimental animal model for human disease including moderate body size, low cost of feeding, short reproductive cycle and lifespan, and close phylogenetic relationship to primates. Our previous studies have shown that TS treated with the mitochondrial inhibitor MPTP displayed classic Parkinsonian symptoms. Additionally, the structure of TS alpha-synuclein (α-syn) is highly homologous to that found in humans. Read More

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December 2018

Human olfactory stem cells: As a promising source of dopaminergic neuron-like cells for treatment of Parkinson's disease.

Neurosci Lett 2018 Dec 12. Epub 2018 Dec 12.

Applied Biophotonics Research Center, Science and Research Branch, Islamic Azad University, Tehran, Iran. Electronic address:

The production of dopaminergic (DA) neurons from stem cells holds a great promise for future clinical treatment of neurodegenerative diseases, such as Parkinson's disease (PD). Olfactory ecto-mesenchymal stem cells (OE-MSCs) derived from the adult human olfactory mucosa can be easily isolated and expanded in culture while maintaining their immense plasticity. In addition to reduced ethical concerns, OE-MSCs could be transplanted across allogeneic barriers, making them desirable stem cells for clinical applications. Read More

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December 2018

Nasal insulin administration does not affect hepatic glucose production at systemic fasting insulin levels.

Diabetes Obes Metab 2018 Dec 15. Epub 2018 Dec 15.

Institute for Diabetes Research and Metabolic Diseases of the Helmholtz Center Munich at the University of Tuebingen, Tuebingen, Germany.

Aim: Pharmacologic administration of insulin as nasal spray in humans has demonstrated a modulation of endogenous glucose production via the brain when systemic insulin levels are high. Results are conflicting under fasting insulin concentrations. To evaluate effects of brain insulin on endogenous glucose production in fasting humans with focus on hepatic glucose release, we performed a randomized, placebo controlled, blinded, cross-over experiment. Read More

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December 2018
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Pediatric encephalic ultrasonography: the essentials.

J Ultrasound 2018 Dec 14. Epub 2018 Dec 14.

Radiology Department, "Santobono-Pausilipon" Children Hospital, Naples, Italy.

Nowadays, cranial ultrasonography (US) of the newborn represents the first imaging method in brain damage study and its possible outcomes. This exam is performed using the natural fontanelles, especially the anterior one. It is fast, non-invasive and does not produce any side effect. Read More

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December 2018

Recent Progress on Functional Genomics Research of Enterovirus 71.

Virol Sin 2018 Dec 14. Epub 2018 Dec 14.

Beijing Key Laboratory of Antimicrobial Agents, Institute of Medicinal Biotechnology, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100050, China.

Enterovirus 71 (EV71) is one of the main pathogens that causes hand-foot-and-mouth disease (HFMD). HFMD caused by EV71 infection is mostly self-limited; however, some infections can cause severe neurological diseases, such as aseptic meningitis, brain stem encephalitis, and even death. There are still no effective clinical drugs used for the prevention and treatment of HFMD. Read More

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December 2018
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Thymosin beta 4-Induced Autophagy Increases Cholinergic Signaling in PrP (106-126)-Treated HT22 Cells.

Neurotox Res 2018 Dec 15. Epub 2018 Dec 15.

Department of Laboratory Animal Medicine, College of Veterinary Medicine, Chonbuk National University, 79 Gobongro, Iksan, 54596, Republic of Korea.

Prion protein peptide (PrP) has been associated with neurotoxicity in brain cells and progression of prion diseases due to spongiform degeneration and accumulation of the infectious scrapie prion protein (PrP). Autophagy has been shown to provide protective functions for neurodegenerative diseases, including prion disease. Thymosin beta 4 (Tβ) plays a key role in the nervous system, providing a neuronal growth effect that includes motility, neurite outgrowth, and proliferation. Read More

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December 2018
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Therapeutic potentials of the Rho kinase inhibitor Fasudil in experimental autoimmune encephalomyelitis and the related mechanisms.

Metab Brain Dis 2018 Dec 14. Epub 2018 Dec 14.

Institute of Brain Science, Shanxi Datong University, Datong, China.

Multiple sclerosis (MS), Parkinson's disease (PD), Alzheimer's disease (AD), and other neurodegenerative diseases of central nervous system (CNS) disorders are serious human health problems. Rho-kinase (ROCK) is emerging as a potentially important therapeutic target relevant to inflammatory neurodegeneration diseases. This is supported by studies showing the beneficial effects of fasudil, a ROCK inhibitor, in inflammatory neurodegeneration diseases. Read More

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December 2018

Time-course study of high fat diet induced alterations in spatial memory, hippocampal JNK, P38, ERK and Akt activity.

Metab Brain Dis 2018 Dec 14. Epub 2018 Dec 14.

Department of Physiology, Faculty of Medicine, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

Consumption of high fat diet (HFD) is a health concern in modern societies, which participate in wide range of diseases. One underlying mechanism in the HFD mediated pathologies is disruption of insulin signaling activity. It is believed that HFD activates several stress signaling molecules such as MAPKs signaling pathway and these molecules participate in harmful effects in different cell populations including hippocampal cells. Read More

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December 2018

The role of recessive inheritance in early-onset epileptic encephalopathies: a combined whole-exome sequencing and copy number study.

Eur J Hum Genet 2018 Dec 14. Epub 2018 Dec 14.

Institute of Medical Genetics, University of Zurich, Schlieren-Zurich, 8952, Switzerland.

Early-onset epileptic encephalopathy (EE) and combined developmental and epileptic encephalopathies (DEE) are clinically and genetically heterogeneous severely devastating conditions. Recent studies emphasized de novo variants as major underlying cause suggesting a generally low-recurrence risk. In order to better understand the full genetic landscape of EE and DEE, we performed high-resolution chromosomal microarray analysis in combination with whole-exome sequencing in 63 deeply phenotyped independent patients. Read More

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December 2018

Spectrum and time course of epilepsy and the associated cognitive decline in duplication syndrome.

Neurology 2018 Dec 14. Epub 2018 Dec 14.

From the Departments of Neurology (D.M., B.S., R.S., D.G., V.N.P., A.M.G.) and Pediatrics (R.S., D.G.), Baylor College of Medicine, Houston, TX.

Objective: We characterized the epilepsy features and contribution to cognitive regression in 47 patients with duplication syndrome (MDS) and reviewed these characteristics in over 280 MDS published cases.

Methods: The institutional review board approved this retrospective review of medical records and case histories of patients with MDS.

Results: The average age at enrollment was 10 ± 7 years. Read More

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December 2018

Role of diffusional kurtosis imaging in grading of brain gliomas: a protocol for systematic review and meta-analysis.

BMJ Open 2018 Dec 14;8(12):e025123. Epub 2018 Dec 14.

Lysholm Department of Neuroradiology, The National Hospital for Neurology and Neurosurgery UCL Hospitals NHS Trust, London, UK.

Introduction: Central nervous system (CNS) gliomas are the most common primary intra-axial brain tumours and pose variable treatment response according to their grade, therefore, precise staging is mandatory. Histopathological analysis of surgical tumour samples is still deemed as the state-of-the-art staging technique for gliomas due to the moderate specificity of the available non-invasive imaging modalities. A recently evolved analysis of the tissue water diffusion properties, known as diffusional kurtosis imaging (DKI), is a dimensionless metric, which quantifies water molecules' degree of non-Gaussian diffusion, hence reflects tissue microenvironment's complexity by means of non-invasive diffusion-weighted MRI acquisitions. Read More

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December 2018

Phase-dependent suppression of beta oscillations in Parkinson's disease patients.

J Neurosci 2018 Dec 14. Epub 2018 Dec 14.

MRC Brain Network Dynamics Unit, Department of Pharmacology, University of Oxford, Oxford OX1 3TH, United Kingdom

Synchronized oscillations within and between brain areas facilitate normal processing, but are often amplified in disease. A prominent example is the abnormally sustained beta-frequency (∼20Hz) oscillations recorded from the cortex and subthalamic nucleus of Parkinson's disease patients. Computational modelling suggests that the amplitude of such oscillations could be modulated by applying stimulation at a specific phase. Read More

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December 2018

APOPT1/COA8 assists COX assembly and is oppositely regulated by UPS and ROS.

EMBO Mol Med 2018 Dec 14. Epub 2018 Dec 14.

MRC-Mitochondrial Biology Unit, University of Cambridge, Cambridge, UK

Loss-of-function mutations in , a gene exclusively found in higher eukaryotes, cause a characteristic type of cavitating leukoencephalopathy associated with mitochondrial cytochrome oxidase (COX) deficiency. Although the genetic association of APOPT1 pathogenic variants with isolated COX defects is now clear, the biochemical link between APOPT1 function and COX has remained elusive. We investigated the molecular role of APOPT1 using different approaches. Read More

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December 2018

Cortical networks are disturbed in people with cirrhosis even in the absence of neuropsychometric impairment.

Clin Neurophysiol 2018 Nov 26. Epub 2018 Nov 26.

UCL Institute for Liver & Digestive Health, Division of Medicine, Royal Free Campus, University College London, Hampstead, London, UK. Electronic address:

Objective: Hepatic encephalopathy is a common complication of cirrhosis; it is characterised by neuropsychometric/neurophysiological abnormalities. Its pathophysiology is complex but glial neuronal communication is likely to be disrupted and to impact on oscillatory networks and cortical connectivity. The aim of this study was to use multichannel electroencephalography (EEG) to investigate functional connectivity, as a surrogate for cortical networks, in patients with cirrhosis. Read More

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November 2018

The Genetic Diagnosis of Neurodegenerative Diseases and Therapeutic Perspectives.

Brain Sci 2018 Dec 13;8(12). Epub 2018 Dec 13.

Evidence-Based Therapeutics Group, Department of Clinical Pharmacology, Universidad de La Sabana, Chía 140013, Colombia.

Genetics has led to a new focus regarding approaches to the most prevalent diseases today. Ascertaining the molecular secrets of neurodegenerative diseases will lead to developing drugs that will change natural history, thereby affecting the quality of life and mortality of patients. The sequencing of candidate genes in patients suffering neurodegenerative pathologies is faster, more accurate, and has a lower cost, thereby enabling algorithms to be proposed regarding the risk of neurodegeneration onset in healthy persons including the year of onset and neurodegeneration severity. Read More

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December 2018

The exploration of novel Alzheimer's therapeutic agents from the pool of FDA approved medicines using drug repositioning, enzyme inhibition and kinetic mechanism approaches.

Biomed Pharmacother 2019 Jan 3;109:2513-2526. Epub 2018 Dec 3.

College of Natural Science, Department of Biological Sciences, Kongju National University, Gongju, 32588, South Korea. Electronic address:

Novel drug development is onerous, time consuming and overpriced process with particularly low success and relatively high enfeebling rates. To overcome this burden, drug repositioning approach is being used to predict the possible therapeutic effects of FDA approved drugs in different diseases. Herein, we designed a computational and enzyme inhibitory mechanistic approach to fetch the promising drugs from the pool of FDA approved drugs against AD. Read More

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January 2019

Moringa oleifera extract attenuates the CoCl induced hypoxia of rat's brain: Expression pattern of HIF-1α, NF-kB, MAO and EPO.

Biomed Pharmacother 2019 Jan 20;109:1688-1697. Epub 2018 Nov 20.

Biochemistry Department, Faculty of Science, Jeddah University, KSA, Egypt; Department of Biochemistry, Faculty of Veterinary Medicine, Zagazig University, Zagazig, 44511, Egypt.

Hypoxia-induced oxidative stress and apoptosis are the major hallmark explanations underlying brain dysfunction. Hypoxia in the current study was induced by Cobalt chloride (CoCl) treatment in rats. The aim of this experiment was to explore the potential ameliorative potency of Moringa oleifera ethanolic extract (MO) against experimentally induced hypoxia on the structure and function of the rat's brain. Read More

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January 2019
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Network localization of heterogeneous neuroimaging findings.

Brain 2018 Dec 14. Epub 2018 Dec 14.

Berenson-Allen Center for Noninvasive Brain Stimulation, Department of Neurology, Beth Israel Deaconess Medical Center, Harvard Medical Center, Boston, MA, USA.

Studies of the same disease often implicate different brain regions, contributing to a perceived reproducibility crisis in neuroimaging. Here, we leverage the normative human brain connectome to test whether seemingly heterogeneous neuroimaging findings localize to connected brain networks. We use neurodegenerative disease, and specifically Alzheimer's disease, as our example as it is one of the diseases that has been studied the most using neuroimaging. Read More

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December 2018

Acoustic prepulse inhibition in male and female prairie voles: Implications for models of neuropsychiatric illness.

Behav Brain Res 2018 Dec 11. Epub 2018 Dec 11.

Oregon Health & Science University, Portland, OR, United States; VA Portland Health Care System, Portland, OR. United States. Electronic address:

Sensory gating, the ability to suppress sensory information of irrelevant stimuli, is affected in several neuropsychiatric diseases, notably schizophrenia and autism. It is currently unclear how these deficits interact with other hallmark symptoms of these disorders, such as social withdrawal and difficulty with interpersonal relationships. The highly affiliative prairie vole (Microtus ochrogaster) may be an ideal model organism to study the neurobiology underlying social behavior. Read More

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December 2018

TLR4 absence reduces neuroinflammation and inflammasome activation in Parkinson's diseases in vivo model.

Brain Behav Immun 2018 Dec 11. Epub 2018 Dec 11.

Department of Chemical, Biological, Pharmacological and Environmental Sciences, University of Messina, Messina, Italy; Department of Pharmacological and Physiological Science, Saint Louis University School of Medicine, USA. Electronic address:

Parkinson's disease (PD) is a progressive, disabling neurodegenerative disorder. It has been shown Toll like receptor (TLR) 4-deficient mice protect against MPTP toxicity, suggesting that dopaminergic cell death is TLR4-dependent. The aim of this study was to demonstrate, in an in vivo model of PD, how TLR4 plays its important role in the pathogenesis of PD by using MPTP neurotoxin model (4 × 20 mg/kg, 2 hr apart, i. Read More

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December 2018

Sex-Specific Association between Prenatal Life Stress Exposure and Infant Pro-Inflammatory Cytokine Levels during Acute Respiratory Infection.

Brain Behav Immun 2018 Dec 11. Epub 2018 Dec 11.

Department of Medicine, Division of Allergy, Pulmonary, and Critical Care Medicine, Vanderbilt University Medical Center, Nashville, Tennessee, 37232-2650, USA.

Background: Prenatal life stress exposure is linked to dysregulated immune function and chronic inflammatory disease in offspring, but we know little about its effects on infant immune response during viral infection.

Method: To address this issue, we examined associations between prenatal life stress exposure and infant upper-airway inflammatory markers during acute respiratory infection (ARI) using data from a prospective, population-based birth-cohort study (N=180). Infant inflammation was measured as a continuous latent factor within a structural equation modeling framework using nasal wash concentrations of interleukin-1β, interleukin-6, and tumor necrosis factor-α. Read More

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December 2018

Cell Biology and Dynamics of Neuronal Na/K-ATPase in Health and Diseases.

Neuropharmacology 2018 Dec 11. Epub 2018 Dec 11.

CNRS Neurodegenerative Diseases Laboratory (UMR9199), MiRCen, CEA Fontenay-aux-Roses, 18 route du Panorama, 92265, France.

Neuronal Na/K-ATPase is responsible for the maintenance of ionic gradient across plasma membrane. In doing so, in a healthy brain, Na/K-ATPase activity accounts for nearly half of total brain energy consumption. The α3-subunit containing Na/K-ATPase expression is restricted to neurons. Read More

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December 2018

Overestimation of an outbreak of Enterobacter cloacae in a neonatal intensive care unit in Germany, 2015.

Pediatr Infect Dis J 2018 Dec 13. Epub 2018 Dec 13.

Department for Infectious Disease Epidemiology, Robert Koch-Institute, Germany.

Background: In August 2015 seventeen neonates with Enterobacter (E.) cloacae colonisation were identified in a neonatal intensive care unit (NICU) in Germany. Two developed severe brain abscesses. Read More

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December 2018
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Morbidity and mortality after transjugular intrahepatic portosystemic shunt placement in patients with cirrhosis.

Eur J Gastroenterol Hepatol 2018 Dec 13. Epub 2018 Dec 13.

Hepatology and Liver Transplant Unit, Department of Medical Area (DAME).

Objectives: Transjugular intrahepatic portosystemic shunt (TIPS) is adopted to treat refractory complications of portal hypertension, such as variceal bleeding and ascites. This study aimed to assess predictors of hepatic encephalopathy (HE) development and cumulative transplant-free survival after TIPS placement in patients with cirrhosis complicated by refractory ascites and major gastroesophageal bleeding.

Materials And Methods: Sixty-three cirrhotic patients who underwent TIPS positioning as a secondary prophylaxis of major upper gastroesophageal bleeding (N=30) or to control refractory ascites (N=33) were enrolled. Read More

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December 2018

Gene mutations in a Han Chinese Alzheimer's disease cohort.

Brain Behav 2018 Dec 14:e01180. Epub 2018 Dec 14.

Department of Neurology, Xinxiang Medical University, Xinxiang, China.

Objective: Alzheimer's disease (AD) is the most common form of dementia characterized by memory loss at disease onset. The gene mutations in the amyloid precursor protein (APP), presenilin 1 (PSEN1), and presenilin 2 (PSEN2) are the frequent causes of AD. However, the clinical and genetic features of AD overlap with other neurodegenerative diseases. Read More

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December 2018

Biallelic CCM3 mutations cause a clonogenic survival advantage and endothelial cell stiffening.

J Cell Mol Med 2018 Dec 13. Epub 2018 Dec 13.

Department of Human Genetics, University Medicine Greifswald and Interfaculty Institute for Genetics and Functional Genomics, University of Greifswald, Greifswald, Germany.

CCM3, originally described as PDCD10, regulates blood-brain barrier integrity and vascular maturation in vivo. CCM3 loss-of-function variants predispose to cerebral cavernous malformations (CCM). Using CRISPR/Cas9 genome editing, we here present a model which mimics complete CCM3 inactivation in cavernous endothelial cells (ECs) of heterozygous mutation carriers. Read More

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December 2018
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Research advancements in the neurological presentation of flaviviruses.

Rev Med Virol 2018 Dec 12:e2021. Epub 2018 Dec 12.

Guangdong Provincial Key Laboratory of Tropical Disease Research, School of Public Health, Southern Medical University, Guangzhou, China.

Owing to the large-scale epidemic of Zika virus disease and its association with microcephaly, properties that allow flaviviruses to cause nervous system diseases are an important area of investigation. At present, although potential pathogenic mechanisms of flaviviruses in the nervous system have been examined, they have not been completely elucidated. In this paper, we review the possible mechanisms of blood-brain barrier penetration, the pathological effects on neurons, and the association between virus mutations and neurotoxicity. Read More

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December 2018

Heart and Brain Diseases: Two Different Worlds.

Authors:
Ivano Dones

Neuromodulation 2018 Dec 12. Epub 2018 Dec 12.

Department of Neurosurgery, Unit for the Surgical Treatment of Spasticity and Pain, Fondazione Istituto Neurologico "C. Besta", Milano, Italy.

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December 2018

The insights into molecular pathways of hypoxia-inducible factor in the brain.

J Neurosci Res 2018 Dec 12. Epub 2018 Dec 12.

Departments of Anesthesiology and Physiology, School of Medicine, Loma Linda University, Loma Linda, California.

The objectives of this present work were to review recent developments on the role of hypoxia-inducible factor (HIF) in the survival of cells under normoxic versus hypoxic and inflammatory brain conditions. The dual nature of HIF effects appears well established, based on the accumulated evidence of HIF playing both the role of adaptive factor and mediator of cell demise. Cellular HIF responses depend on pathophysiological conditions, developmental phase, comorbidities, and administered medications. Read More

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December 2018
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Novel variants in SPTAN1 without epilepsy: An expansion of the phenotype.

Am J Med Genet A 2018 Dec 11. Epub 2018 Dec 11.

Office of the Clinical Director, NHGRI, and NIH Undiagnosed Diseases Program, Office of the Director, National Institutes of Health, Bethesda, Maryland.

We describe two unrelated children with de novo variants in the non-erythrocytic alpha-II-spectrin (SPTAN1) gene who have hypoplastic brain structures, intellectual disability, and both fine and gross motor impairments. Using agnostic exome sequencing, we identified a nonsense variant creating a premature stop codon in exon 21 of SPTAN1, and in a second patient we identified an intronic substitution in SPTAN1 prior to exon 50 creating a new donor acceptor site. Neither of these variants has been described previously. Read More

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December 2018

Neuroinflammation in the central nervous system: Symphony of glial cells.

Glia 2018 Dec 11. Epub 2018 Dec 11.

Institute of Neuroscience, State Key Laboratory of Neuroscience, CAS Center for Excellence in Brain Science and Intelligence Technology, Chinese Academy of Sciences, Shanghai, China.

Neuroinflammation in the central nervous system (CNS) is an important subject of neuroimmunological research. Emerging evidence suggests that neuroinflammation is a key player in various neurological disorders, including neurodegenerative diseases and CNS injury. Neuroinflammation is a complex and well-orchestrated process by various groups of glial cells in CNS and peripheral immune cells. Read More

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December 2018
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TREM2 triggers microglial density and age-related neuronal loss.

Glia 2018 Dec 11. Epub 2018 Dec 11.

Neural Regeneration, Institute of Reconstructive Neurobiology, University Hospital of Bonn, University of Bonn, Bonn, Germany.

The microglial triggering receptor expressed on myeloid cells 2 (TREM2) signals via the activatory membrane adaptor molecule TYROBP. Genetic variants or mutations of TREM2 or TYROBP have been linked to inflammatory neurodegenerative diseases associated with aging. The typical aging process goes along with microglial changes and mild neuronal loss, but the exact contribution of TREM2 is still unclear. Read More

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December 2018

Curcumin: A new candidate for retinal disease therapy?

J Cell Biochem 2018 Dec 11. Epub 2018 Dec 11.

Research Center for Biochemistry and Nutrition in Metabolic Diseases, Kashan University of Medical Sciences, Kashan, Iran.

The retina is the neural portion and light-sensitive layer of the eye, which has been observed in most of the vertebrates. The retina is composed of light-sensitive cells that absorb light and convert it into neural signals. These signals are sent to the brain for visual recognition. Read More

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December 2018
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The sonic hedgehog pathway mediates Tongxinluo capsule-induced protection against blood-brain barrier disruption after ischemic stroke in mice.

Basic Clin Pharmacol Toxicol 2018 Dec 7. Epub 2018 Dec 7.

Key Laboratory of State Administration of TCM (Cardio-Cerebral Vessel Collateral Disease), National Key Laboratory of Collateral Disease Research and Innovative Chinese Medicine, Hebei Yiling Chinese Medicine Research Institute, Shijiazhuang, China.

Tongxinluo capsule (TXL), a Chinese prescription, has been extensively used for treating ischaemic cerebrovascular diseases in China. Studies have demonstrated that TXL protects the blood-brain barrier (BBB) after cerebral ischaemia. However, the underlying protective mechanisms are not fully elucidated. Read More

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December 2018
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Preparation of lyophilized recombinant prion protein for TSE diagnosis by RT-QuIC.

BMC Res Notes 2018 Dec 14;11(1):895. Epub 2018 Dec 14.

Virus and Prion Research Unit, National Animal Disease Center, United States Department of Agriculture, Agricultural Research Service, Ames, IA, 50010, USA.

Objective: Transmissible spongiform encephalopathies (TSEs) are a group of fatal neurodegenerative diseases, often referred as prion diseases. TSEs result from the misfolding of the cellular prion protein (PrP) into a pathogenic form (PrP) that accumulates in the brain and lymphatic tissue. Amplification based assays such as real-time quaking induced conversion allow us to assess the conversion of PrP to PrP. Read More

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December 2018

MRI phenotypes of the brain are related to future stroke and mortality in patients with manifest arterial disease: The SMART-MR study.

J Cereb Blood Flow Metab 2018 Dec 14:271678X18818918. Epub 2018 Dec 14.

1 Department of Radiology, University Medical Center Utrecht, and Utrecht University, Utrecht, The Netherlands.

Neurodegenerative and neurovascular diseases lead to heterogeneous brain abnormalities. A combined analysis of these abnormalities by phenotypes of the brain might give a more accurate representation of the underlying aetiology. We aimed to identify different MRI phenotypes of the brain and assessed the risk of future stroke and mortality within these subgroups. Read More

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December 2018

Advances in quantitative assessment of parkinsonian motor symptoms with wearable devices.

Sci China Life Sci 2018 Dec 3. Epub 2018 Dec 3.

Department of Neurology, Fujian Institute of Geriatrics, Fujian Medical University Union Hospital, Fuzhou, 350001, China.

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December 2018

Glucose metabolic brain patterns to discriminate amyotrophic lateral sclerosis from Parkinson plus syndromes.

EJNMMI Res 2018 Dec 13;8(1):110. Epub 2018 Dec 13.

Department of Nuclear Medicine and Molecular Imaging, Division of Nuclear Medicine, KU Leuven, Herestraat 49, 3000, Leuven, Belgium.

Background: F-FDG brain PET measures metabolic changes in neurodegenerative disorders and may discriminate between different diseases even at an early stage. The objective of this study was to classify patients with amyotrophic lateral sclerosis (ALS) and Parkinson plus syndromes (PP). To this end, different approaches were evaluated using generalized linear models and corresponding glucose metabolic brain patterns. Read More

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December 2018

[CARs, CRS and neurotoxicity: severe complications after administration of immunotherapy : Essentials for intensivists].

Med Klin Intensivmed Notfmed 2018 Dec 13. Epub 2018 Dec 13.

Klinik I für Innere Medizin, Uniklinik Köln, Kerpener Str. 62, 50937, Köln, Deutschland.

Background: The development of chimeric antigen receptor (CAR) T‑cells has shown promising results in relapsed/refractory B‑cell acute lymphoblastic leukemia/lymphoma (B-ALL) and diffuse large cell B‑cell lymphoma. Complications, especially cytokine release syndrome (CRS) and CAR T‑cell related encephalopathy syndrome (CRES), can be life threatening. The management of both plays a key role in CAR T‑cell therapy. Read More

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December 2018

The Role of Anticoagulation in Treating Portal Hypertension.

Curr Hepatol Rep 2018 18;17(3):200-208. Epub 2018 Jun 18.

1Division of Gastroenterology, Azienda Ospedaliero-Universitaria di Modena and University of Modena and Reggio Emilia, Modena, Italy.

Purpose Of Review: To revise experimental and clinical data supporting a less traditional role of anticoagulation for treating portal hypertension in patients with cirrhosis.

Recent Findings: Portal hypertension is the main driver of complications such as ascites, variceal hemorrhage, and hepatic encephalopathy, with inflammation as a key component. The traditional view of cirrhosis as a pro-hemorrhagic condition has recently changed, prothrombotic complications being recognized as frequently as the hemorrhagic ones. Read More

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Identification of PD-L2, B7-H3 and CTLA-4 immune checkpoint proteins in genetic subtypes of meningioma.

Oncoimmunology 2019 5;8(1):e1512943. Epub 2018 Sep 5.

Project neuroArm, Department of Clinical Neurosciences, Cumming School of Medicine, University of Calgary, Calgary, AB, Canada.

Meningioma is the most common brain tumor in adults. Surgical resection remains the primary treatment. No chemotherapy exists. Read More

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September 2018
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A Case of Mistaken Identity: Glutaric Aciduria Type I Masquerading as Postmeningitic Hydrocephalus.

J Clin Imaging Sci 2018 15;8:50. Epub 2018 Nov 15.

Department of Radiology and Imaging, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India.

We report the characteristic neuroimaging features of a rare metabolic leukodystrophy in an 8-year-old boy, born of consanguineous parenthood. The child presented with macrocrania, regression of milestones, and dystonia. The patient was referred for magnetic resonance imaging with a clinical diagnosis of postmeningitic hydrocephalus. Read More

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November 2018

Epigenetic modulation during hippocampal development.

Biomed Rep 2018 Dec 18;9(6):463-473. Epub 2018 Oct 18.

Department of Pharmacology, Medical School of Yangtze University, Jingzhou, Hubei 434023, P.R. China.

The hippocampus is located in the limbic system and is vital in learning ability, memory formation and emotion regulation, and is associated with depression, epilepsy and mental retardation in an abnormal developmental situation. Several factors have been found to modulate the development of the hippocampus, and epigenetic modification have a crucial effect in this progress. The present review summarizes the epigenetic modifications, including DNA methylation, histone acetylation, and non-coding RNAs, regulating all stages of hippocampal development, focusing on the growth of Ammons horn and the dentate gyrus in humans and rodents. Read More

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December 2018

Late adult-onset adrenomyeloneuropathy evolving with atypical severe frontal lobe syndrome: Importance of neuroimaging.

Radiol Case Rep 2019 Mar 5;14(3):309-314. Epub 2018 Dec 5.

Department of Medical, Surgical, Neurological, Metabolic Sciences, and Aging, 2nd Division of Neurology, Center for Rare Diseases and InterUniversity Center for Research in Neurosciences, University of Campania "Luigi Vanvitelli", Via Sergio Pansini, 80131 Naples, Italy.

X-linked adrenoleukodystrophy (X-ALD) is a rare inherited metabolic disease affecting the nervous system and the adrenal glands. It is caused by a mutation of the gene, resulting in the impaired degradation of very long-chain fatty acids and their subsequent accumulation in several organs and tissues. X-ALD is notable for its high phenotypical variability, that includes isolated adrenocortical insufficiency, slowly progressive myelopathy with paraparesis, ataxia, and peripheral neuropathy to severe childhood cerebral forms. Read More

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