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https://www.readbyqxmd.com/read/28230504/peripheral-neuropathy-and-leptin-receptor-defect-demyelination-of-the-sciatic-nerve-of-the-obese-zucker-rat
#1
Jacques Gilloteaux, Kritika Subramanian, Nadia Solomon
No abstract text is available yet for this article.
January 2017: Ultrastructural Pathology
https://www.readbyqxmd.com/read/28230289/mice-lacking-bcas1-a-novel-myelin-associated-protein-display-hypomyelination-schizophrenia-like-abnormal-behaviors-and-upregulation-of-inflammatory-genes-in-the-brain
#2
Tetsuya Ishimoto, Kensuke Ninomiya, Ran Inoue, Masato Koike, Yasuo Uchiyama, Hisashi Mori
The abnormal expression and function of myelin-related proteins contribute to nervous system dysfunction associated with neuropsychiatric disorders; however, the underlying mechanism of this remains unclear. We found here that breast carcinoma amplified sequence 1 (BCAS1), a basic protein abundant in the brain, was expressed specifically in oligodendrocytes and Schwann cells, and that its expression level was decreased by demyelination. This suggests that BCAS1 is a novel myelin-associated protein. BCAS1 knockout mice displayed schizophrenia-like behavioral abnormalities and a tendency toward reduced anxiety-like behaviors...
February 23, 2017: Glia
https://www.readbyqxmd.com/read/28229840/-magnetic-resonance-imaging-conversion-predictors-of-clinically-isolated-syndrome-to-multiple-sclerosis
#3
REVIEW
Sara Peixoto, Pedro Abreu
INTRODUCTION: Clinically isolated syndrome may be the first manifestation of multiple sclerosis, a chronic demyelinating disease of the central nervous system, and it is defined by a single clinical episode suggestive of demyelination. However, patients with this syndrome, even with long term follow up, may not develop new symptoms or demyelinating lesions that fulfils multiple sclerosis diagnostic criteria. We reviewed, in clinically isolated syndrome, what are the best magnetic resonance imaging findings that may predict its conversion to multiple sclerosis...
November 2016: Acta Médica Portuguesa
https://www.readbyqxmd.com/read/28226691/structural-changes-of-cingulate-cortex-in-post-stroke-depression
#4
Chenfei Ye, Jun Wu, Xuhui Chen, Changle Zhang, Hengtong Li, Shuai Mao, Heather T Ma, Chenfei Ye, Jun Wu, Xuhui Chen, Changle Zhang, Hengtong Li, Shuai Mao, Heather T Ma, Shuai Mao, Heather T Ma, Jun Wu, Hengtong Li, Changle Zhang, Chenfei Ye, Xuhui Chen
Disruption of neural connections among regions regulating mood and cognition rather than cerebrovascular lesions may contribute to post-stroke depression (PSD). In this paper, we hypothesized that structural abnormalities in white matter structure like cingulate cortex induced by focal infarcts would play a role in mood regulation or depression after stroke onset. Various DTI coefficients including FA, RD and ADC with multiple signal distribution measurements were collected and statistically analyzed. The results identify significant differences in volume and multiple diffusion indices of DTI intensity distribution in cingulate cortex between PSD patients and the normal control...
August 2016: Conference Proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society
https://www.readbyqxmd.com/read/28225293/rituximab-associated-progressive-multifocal-leukoencephalopathy
#5
Juraj Sokol, Lenka Lisá, Jana Zeleňáková, Tomáš Balhárek, Ivana Plameňová, Ján Staško, Peter Kubisz
The definition "Progressive Multifocal Leukoencephalopathy" (PML) was first used in 1958 to describe a fatal demyelinating central nervous system (CNS) disease in patients with lymphoproliferative disorders. In 1971, the virus responsible for the disease was isolated and named John Cunningham virus (JCV). We present a rare case of a 62-year-old male with chronic lymphocytic leukemia and PML. In our work, we discuss the diagnostic and therapeutic challenges and offer suggestions for preventing PML development...
2017: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/28225274/a-rational-design-of-a-selective-inhibitor-for-kv1-1-channels-prevalent-in-demyelinated-nerves-that-improves-their-impaired-axonal-conduction
#6
Ahmed Al-Sabi, Declan Daly, Patrick Hoefer, Gemma K Kinsella, Charles P E Metais, Mark Pickering, Caroline Herron, Seshu Kumar Kaza, Kieran J Nolan, J Oliver Dolly
K(+) channels containing Kv1.1 α subunits, which become prevalent at internodes in demyelinated axons, may underlie their dysfunctional conduction akin to muscle weakness in multiple sclerosis. Small inhibitors were sought with selectivity for the culpable hyper-polarising K(+) currents. Modelling of interactions with the extracellular pore in a Kv1.1 deduced-structure identified a diaryldi-(2-pyrrolyl)methane (DPM) as a suitable scaffold, with optimised alkyl ammonium side chains. The resultant synthesised candidate [2,2'-((5,5'(di-p-topyldiaryldi-(2-pyrrolyl)methane)bis(2,2'carbonyl)bis (azanediyl)) diethaneamine...
February 22, 2017: Journal of Medicinal Chemistry
https://www.readbyqxmd.com/read/28225235/-multiple-sclerosis-a-mitochondria-mediated-disease
#7
Kristin N Varhaug, Christian A Vedeler, Charalampos Tzoulis, Laurence A Bindoff
BACKGROUND Mitochondria play an important role in the pathogenesis of various neurodegenerative disorders, including Parkinson's disease. Neurodegenerative changes occur early in the course of multiple sclerosis (MS). This article aims to present information on a possible association between mitochondrial dysfunction and multiple sclerosis.MATERIAL AND METHOD The article is based on original and review articles selected following a literature search in PubMed, restricted to articles written in English, and concluded in May 2016...
February 2017: Tidsskrift for Den Norske Lægeforening: Tidsskrift for Praktisk Medicin, Ny Række
https://www.readbyqxmd.com/read/28224702/early-platelet-and-leukocyte-decline-in-patients-with-neuroinflammatory-disorders-after-intravenous-immunoglobulins
#8
A Totzeck, M Stettner, T Hagenacker
BACKGROUND AND PURPOSE: Intravenous immunoglobulins (IVIGs) are a common therapy in patients with neuroinflammatory disorders, especially chronic inflammatory demyelinating polyradiculoneuropathy or Guillain-Barré syndrome. Hematological toxicities upon IVIG infusion are a known side effect and still an important subject of investigation. METHODS: Laboratory results and data for clinical efficacy and tolerability of 62 patients with neuroinflammatory disorders treated with IVIG (0...
February 22, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28222370/increased-postural-sway-in-persons-with-multiple-sclerosis-during-short-term-exposure-to-warm-ambient-temperatures
#9
Paula Y S Poh, Amy N Adams, Mu Huang, Dustin R Allen, Scott L Davis, Anna S Tseng, Craig G Crandall
BACKGROUND: Multiple sclerosis (MS) is a neurological disease marked by demyelination and axonal loss. Individuals with MS experience increases in clinical signs and symptoms during heat exposure. OBJECTIVE: To test the hypothesis that moderate heat exposure adversely affects postural sway in individuals with MS. METHODS: Ten individuals with relapsing-remitting MS (50±8y) and nine controls (47±10y) were examined under a Thermal and a Time Control trial...
February 8, 2017: Gait & Posture
https://www.readbyqxmd.com/read/28221307/utility-of-neurological-imaging-in-sensory-variant-of-chronic-inflammatory-demyelinating-polyneuropathy-a-case-report
#10
Alexander G Gevorgyan, Akshay Shah, Said R Beydoun
No abstract text is available yet for this article.
March 2017: Journal of Clinical Neuromuscular Disease
https://www.readbyqxmd.com/read/28217155/acute-bulbar-palsy-plus-syndrome-a-rare-variant-of-guillain-barre-syndrome
#11
Sanghamitra Ray, Prakash Chand Jain
Guillain-Barre syndrome (GBS) is the most common cause of acute flaccid paralysis worldwide both in adult and pediatric population. Although flaccid paralysis is the hallmark of this disease, there are some rare variants which may be easily missed unless suspected. Here, we present a very rare variant of GBS - acute bulbar palsy plus syndrome in a pediatric patient. A 13-year-old female child presented with right-sided lower motor neuron type of facial palsy and palsy of bilateral glossopharyngeal and vagus nerve of 2 weeks duration...
October 2016: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/28217021/central-pontine-myelinolysis-and-localized-fluorodeoxyglucose-uptake-seen-on-18-f-fdg-pet-ct
#12
Frederik Rønne, Peer Carsten Tfelt-Hansen, Lene Rørdam
Case report describing the finding of central pontine myelinolysis (CPM) using combined fluorine-18 ( (18)F)-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT). The patient was a known alcoholic who, during admission was under treatment for hyponatremia, showed a significant decline in both motor and cognitive function. Combined (18)F-FDG PET/CT showed localized FDG uptake in the pons, consistent with the finding of CPM observed on magnetic resonance imaging (MRI). CPM is a demyelinating lesion of the pons, resulting in several neurological symptoms...
January 2017: World Journal of Nuclear Medicine
https://www.readbyqxmd.com/read/28216651/deficiency-of-the-g-protein-g%C3%AE-q-ameliorates-experimental-autoimmune-encephalomyelitis-with-impaired-dc-derived-il-6-production-and-th17-differentiation
#13
Weiming Lai, Yingying Cai, Jinfeng Zhou, Shuai Chen, Chaoyan Qin, Cuixia Yang, Junling Liu, Xin Xie, Changsheng Du
Many G protein-coupled receptors (GPCRs) are reported to be involved in the pathogenesis of multiple sclerosis (MS), and ~40% of all identified GPCRs rely on the Gαq/11 G protein family to stimulate inositol lipid signaling. However, the function of Gα subunits in MS pathogenesis is still unknown. In this study, we attempted to determine the role of Gαq in the pathogenesis of experimental autoimmune encephalomyelitis (EAE), a well-known mouse model of MS. We discovered that compared with wild-type mice, Gαq-knockout mice exhibited less severe EAE symptoms, with lower clinical scores, reduced leukocyte infiltration and less extensive demyelination...
February 20, 2017: Cellular & Molecular Immunology
https://www.readbyqxmd.com/read/28216073/autoimmune-neuropathies-associated-to-rheumatic-diseases
#14
REVIEW
Alberto R M Martinez, Ingrid Faber, Anamarli Nucci, Simone Appenzeller, Marcondes C França
Systemic manifestations are frequent in autoimmune rheumatic diseases and include peripheral nervous system damage. Neuron cell body, axons and myelin sheath may all be affected in this context. This involvement results in severe and sometimes disabling symptoms. Sensory, motor and autonomic features may be present in different patterns that emerge as peculiar clinical pictures. Prompt recognition of these neuropathies is pivotal to guide treatment and reduce the risks of long term disability. In this review, we aim to describe the main immune-mediated neuropathies associated to rheumatic diseases: sensory neuronopathies, multiple mononeuropathies and chronic inflammatory demyelinating polyradiculoneuropathy, with an emphasis on clinical features and therapeutic options...
February 12, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28215668/potential-of-surfactant-coated-nanoparticles-to-improve-brain-delivery-of-arylsulfatase-a
#15
Tilman Schuster, Astrid Mühlstein, Claudia Yaghootfam, Olga Maksimenko, Elena Shipulo, Svetlana Gelperina, Jörg Kreuter, Volkmar Gieselmann, Ulrich Matzner
The lysosomal storage disorder (LSD) metachromatic leukodystrophy (MLD) is caused by a deficiency of the soluble, lysosomal hydrolase arylsulfatase A (ASA). The disease is characterized by accumulation of 3-O-sulfogalactosylceramide (sulfatide), progressive demyelination of the nervous system and premature death. Enzyme replacement therapy (ERT), based on regular intravenous injections of recombinant functional enzyme, is in clinical use for several LSDs. For MLD and other LSDs with central nervous system (CNS) involvement, however, ERT is limited by the blood-brain barrier (BBB) restricting transport of therapeutic enzymes from the blood to the brain...
February 16, 2017: Journal of Controlled Release: Official Journal of the Controlled Release Society
https://www.readbyqxmd.com/read/28215575/fibronectin-connecting-segment-1-peptide-inhibits-pathogenic-leukocyte-trafficking-and-inflammatory-demyelination-in-experimental-models-of-chronic-inflammatory-demyelinating-polyradiculoneuropathy
#16
Chaoling Dong, Kelsey M Greathouse, Rebecca L Beacham, Steven P Palladino, E Scott Helton, Eroboghene E Ubogu
The molecular determinants of pathogenic leukocyte migration across the blood-nerve barrier (BNB) in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) are unknown. Specific disease modifying therapies for CIDP are also lacking. Fibronectin connecting segment-1 (FNCS1), an alternatively spliced fibronectin variant expressed by microvascular endothelial cells at sites of inflammation in vitro and in situ, is a counterligand for leukocyte α4 integrin (also known as CD49d) implicated in pathogenic leukocyte trafficking in multiple sclerosis and inflammatory bowel disease...
February 16, 2017: Experimental Neurology
https://www.readbyqxmd.com/read/28214652/unilateral-oculomotor-palsy-in-charcot-marie-tooth-disease-1a-cmt-1a
#17
A Posa, A Emmer, M E Kornhuber
BACKGROUND: Charcot-Marie-Tooth disease (CMT) type 1A is the most common form of CMT 1 and one of the autosomal dominant demyelinating hereditary motor and sensory neuropathies (HMSN). Cranial nerves may be frequently subclinically affected in CMT disease. However manifest clinical signs of cranial nerve involvement are rare. METHODS: This case comprise neurological, ophthalmological, internal medicine and ear-nose-throat investigation, motor and sensory nerve conduction velocity, auditory evoked potentials and orbicularis-oculi reflex measurements, lumbar puncture and blood examination, inclusive molecular genetic testing, as well as electrocardiogram and cranial imaging such as computer tomography and magnetic resonance imaging RESULTS: The present case shows a Charcot-Marie-Tooth (CMT) 1A patient with complete unilateral oculomotor palsy in combination with predominant ipsilateral subclinical trigeminal demyelination...
February 13, 2017: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/28213912/diagnostic-red-flags-steroid-treated-malignant-cns-lymphoma-mimicking-autoimmune-inflammatory-demyelination
#18
Alonso Barrantes-Freer, Aylin Sophie Engel, Odir Antonio Rodríguez-Villagra, Anne Winkler, Markus Bergmann, Christian Mawrin, Tania Kuempfel, Hannah Pellkofer, Imke Metz, Annalen Bleckmann, Silvia Hernández-Durán, Sven Schippling, Elisabeth J Rushing, Stephan Frank, Markus Glatzel, Jakob Matschke, Christian Hartmann, Guido Reifenberger, Wolf Müller, Hans-Ulrich Schildhaus, Wolfgang Brück, Christine Stadelmann
The presence of inflammation and demyelination in a central nervous system (CNS) biopsy points towards a limited, yet heterogeneous group of pathologies, of which multiple sclerosis (MS) represents one of the principal considerations. Inflammatory demyelination has also been reported in patients with clinically suspected primary central nervous system lymphoma (PCNSL), especially when steroids had been administered prior to biopsy acquisition. The histopathological changes induced by corticosteroid treatment can range from mild reduction to complete disappearance of lymphoma cells...
February 18, 2017: Brain Pathology
https://www.readbyqxmd.com/read/28212925/reversible-opercular-syndrome-secondary-to-osmotic-demyelination
#19
Shuchit Pandey, Vikram V Holla, Imran Rizvi, Rakesh Shukla
Opercular syndrome (OPS) is characterized by weakness of facial, masticatory, pharyngeal, laryngeal, tongue and brachial muscles on voluntary command with preservation of emotional and reflexive movements. We report a case of 45year old female who developed the features of OPS due to lesions of bilateral frontal opercular region induced by osmotic demyelination secondary to hyperosmolar hyperglycaemia. On follow up at 6 months, she had complete recovery.
February 11, 2017: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/28212662/marked-central-nervous-system-pathology-in-cd59-knockout-rats-following-passive-transfer-of-neuromyelitis-optica-immunoglobulin-g
#20
Xiaoming Yao, Alan S Verkman
Neuromyelitis optica spectrum disorders (herein called NMO) is an inflammatory demyelinating disease of the central nervous system in which pathogenesis involves complement-dependent cytotoxicity (CDC) produced by immunoglobulin G autoantibodies targeting aquaporin-4 (AQP4-IgG) on astrocytes. We reported evidence previously, using CD59(-/-) mice, that the membrane-associated complement inhibitor CD59 modulates CDC in NMO (Zhang and Verkman, J. Autoimmun. 53:67-77, 2014). Motivated by the observation that rats, unlike mice, have human-like complement activity, here we generated CD59(-/-) rats to investigate the role of CD59 in NMO and to create NMO pathology by passive transfer of AQP4-IgG under conditions in which minimal pathology is produced in normal rats...
February 17, 2017: Acta Neuropathologica Communications
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