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https://www.readbyqxmd.com/read/27911409/measuring-progressive-neurological-disability-in-a-mouse-model-of-multiple-sclerosis
#1
Francesca Gilli, Darlene B Royce, Andrew R Pachner
After intracerebral infection with the Theiler's Murine Encephalomyelitis Virus (TMEV), susceptible SJL mice develop a chronic-progressive demyelinating disease, with clinical features similar to the progressive forms of multiple sclerosis (MS). The mice show progressive disability with loss of motor and sensory functions, which can be assessed with multiple apparatuses and protocols. Among them, the Rotarod performance test is a very common behavioral test, its advantage being that it provides objective measurements, but it is often used assuming that it is straightforward and simple...
November 14, 2016: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/27911122/an-update-on-the-use-of-cerebrospinal-fluid-analysis-as-a-diagnostic-tool-in-multiple-sclerosis
#2
Matteo Gastaldi, Elisabetta Zardini, Diego Franciotta
Intrathecal B-lymphocyte activation is a hallmark of multiple sclerosis (MS), a multi-factorial inflammatory-demyelinating disease of the central nervous system. Such activation has a counterpart in the cerebrospinal fluid (CSF) oligoclonal IgG bands (OCB), whose diagnostic role in MS has been downgraded within the current McDonald's criteria. With a theoretico-practical approach, the authors review the physiopathological basis of the CSF dynamics, and the state-of-the-art of routine CSF analysis and CSF biomarkers in MS...
December 2, 2016: Expert Review of Molecular Diagnostics
https://www.readbyqxmd.com/read/27909439/the-a-allele-of-the-single-nucleotide-polymorphism-rs630923-creates-a-binding-site-for-mef2c-resulting-in-reduced-cxcr5-promoter-activity-in-b-cell-lymphoblastic-cell-lines
#3
Nikita A Mitkin, Alisa M Muratova, Anton M Schwartz, Dmitry V Kuprash
Chemokine receptor CXCR5 is highly expressed in B-cells and under normal conditions is involved in their migration to specific areas of secondary lymphoid organs. B-cells are known to play an important role in various autoimmune diseases including multiple sclerosis (MS) where areas of demyelinating lesions attract B-cells by overexpressing CXCL13, the CXCR5 ligand. In this study, we aimed to determine the functional significance of single-nucleotide polymorphism rs630923 (A/C), which is located in cxcr5 gene promoter, and its common allele is associated with increased risk of MS...
2016: Frontiers in Immunology
https://www.readbyqxmd.com/read/27909192/the-more-the-merrier-scoring-statistics-and-animal-welfare-in-experimental-autoimmune-encephalomyelitis
#4
Pushpalatha Palle, Filipa M Ferreira, Axel Methner, Thorsten Buch
Experimental autoimmune encephalomyelitis (EAE) is a frequently used animal model for the investigation of autoimmune processes in the central nervous system. As such, EAE is useful for modelling certain aspects of multiple sclerosis, a human autoimmune disease that leads to demyelination and axonal destruction. It is an important tool for investigating pathobiology, identifying drug targets and testing drug candidates. Even though EAE is routinely used in many laboratories and is often part of the routine assessment of knockouts and transgenes, scoring of the disease course has not become standardized in the community, with at least 83 published scoring variants...
December 2016: Laboratory Animals
https://www.readbyqxmd.com/read/27909013/comparison-of-cerebrospinal-fluid-opening-pressure-in-children-with-demyelinating-disease-to-children-with-primary-intracranial-hypertension
#5
Bethanie Morgan-Followell, Shawn C Aylward
The authors aimed to compare the opening pressures of children with demyelinating disease to children with primary intracranial hypertension. Medical records were reviewed for a primary diagnosis of demyelinating disease, or primary intracranial hypertension. Diagnosis of demyelinating disease was made according to either the 2007 or 2012 International Pediatric Multiple Sclerosis Study Group criteria. Primary intracranial hypertension diagnosis was confirmed by presence of elevated opening pressure, normal cerebrospinal fluid composition and neuroimaging...
November 30, 2016: Journal of Child Neurology
https://www.readbyqxmd.com/read/27904983/vdbp-cyp27b1-and-25-hydroxyvitamin-d-gene-polymorphism-analyses-in-a-group-of-sicilian-multiple-sclerosis-patients
#6
L Agnello, C Scazzone, B Lo Sasso, C Bellia, G Bivona, S Realmuto, F Brighina, R Schillaci, P Ragonese, G Salemi, Marcello Ciaccio
Multiple sclerosis (MS) is a chronic demyelinating disease of central nervous system regarded as one of the most common causes of neurological disability in young adults. The exact etiology of MS is not yet known, although epidemiological data indicate that both genetic susceptibility and environmental exposure are involved. A poor vitamin D status has been proposed as the most attractive environmental factor. Several evidence have highlighted the importance of mutations in vitamin D-regulating genes for vitamin D status...
November 30, 2016: Biochemical Genetics
https://www.readbyqxmd.com/read/27904823/expression-of-gp91phox-and-p22phox-catalytic-subunits-of-nadph-oxidase-on-microglia-in-nasu-hakola-disease-brains
#7
Jun-Ichi Satoh, Yoshihiro Kino, Motoaki Yanaizu, Youhei Tosaki, Kenji Sakai, Tusyoshi Ishida, Yuko Saito
The superoxide-producing nicotinamide adenine dinucleotide phosphate (NADPH) oxidase complex of phagocytes (phox) plays a key role in production of reactive oxygen species (ROS) by microglia. The catalytic subunits of the NADPH oxidase are composed of p22phox and gp91phox. Nasu-Hakola disease (NHD) is a rare autosomal recessive disorder caused by a loss-of-function mutation of either TYROBP (DAP12) or TREM2. Pathologically, the brains of NHD patients exhibit extensive demyelination designated leukoencephalopathy, astrogliosis, accumulation of axonal spheroids, and remarkable activation of microglia predominantly in the white matter of frontal and temporal lobes...
November 2016: Intractable & Rare Diseases Research
https://www.readbyqxmd.com/read/27904492/hydroxycitric-acid-ameliorates-inflammation-and-oxidative-stress-in-mouse-models-of-multiple-sclerosis
#8
Mahdi Goudarzvand, Sanaz Afraei, Somaye Yaslianifard, Saleh Ghiasy, Ghazal Sadri, Mustafa Kalvandi, Tina Alinia, Ali Mohebbi, Reza Yazdani, Shahin Khadem Azarian, Abbas Mirshafiey, Gholamreza Azizi
Hydroxycitric acid (HCA) is derived primarily from the Garcinia plant and is widely used for its anti-inflammatory effects. Multiple sclerosis can cause an inflammatory demyelination and axonal damage. In this study, to validate the hypothesis that HCA exhibits therapeutic effects on multiple sclerosis, we established female C57BL/6 mouse models of multiple sclerosis, i.e., experimental autoimmune encephalomyelitis, using Complete Freund's Adjuvant (CFA) emulsion containing myelin oligodendrocyte glycoprotein (35-55)...
October 2016: Neural Regeneration Research
https://www.readbyqxmd.com/read/27904491/intravenous-transplantation-of-mouse-embryonic-stem-cells-attenuates-demyelination-in-an-icr-outbred-mouse-model-of-demyelinating-diseases
#9
Kidsadagon Pringproa, Anucha Sathanawongs, Chananthida Khamphilai, Sarocha Sukkarinprom, Apichart Oranratnachai
Induction of demyelination in the central nervous system (CNS) of experimental mice using cuprizone is widely used as an animal model for studying the pathogenesis and treatment of demyelination. However, different mouse strains used result in different pathological outcomes. Moreover, because current medicinal treatments are not always effective in multiple sclerosis patients, so the study of exogenous cell transplantation in an animal model is of great importance. The aims of the present study were to establish an alternative ICR outbred mouse model for studying demyelination and to evaluate the effects of intravenous cell transplantation in the present developed mouse model...
October 2016: Neural Regeneration Research
https://www.readbyqxmd.com/read/27903432/antigen-presentation-for-priming-t-cellsin-central-system
#10
Shaoni Dasgupta, Subhajit Dasgupta
Generation of myelin antigen-specific T cells is a major event in neuroimmune responses that causes demyelination. The antigen-priming of T cells and its location is important in chronic and acute inflammation. In autoimmune multiple sclerosis, the effector T cells are considered to generate in periphery. However, the reasons for chronic relapsing-remitting events are obscure. Considering mechanisms, a feasible aim of research is to investigate the role of antigen-primed T cells in lupus cerebritis. Last thirty years of investigations emphasize the relevance of microglia and infiltrated dendritic cells/macrophages as antigen presenting cells in the central nervous system...
November 26, 2016: International Journal of Biochemistry & Cell Biology
https://www.readbyqxmd.com/read/27903328/cognitive-and-behavioral-functioning-in-childhood-acquired-demyelinating-syndromes
#11
Christine Till, Austin Noguera, Leonard H Verhey, Julia O'Mahony, E Ann Yeh, Jean K Mah, Katia J Sinopoli, Brian L Brooks, Berengere Aubert-Broche, D Louis Collins, Sridar Narayanan, Douglas L Arnold, Brenda L Banwell
OBJECTIVES: The aim of this study was to describe cognitive, academic, and psychosocial outcomes after an incident demyelinating event (acquired demyelinating syndromes, ADS) in childhood and to investigate the contribution of brain lesions and confirmed MS diagnosis on outcome. METHODS: Thirty-six patients with ADS (mean age=12.2 years, SD=2.7, range: 7-16 years) underwent brain MRI scans at presentation and at 6-months follow-up. T2-weighted lesions on MRI were assessed using a binary classification...
November 2016: Journal of the International Neuropsychological Society: JINS
https://www.readbyqxmd.com/read/27902997/chronic-inflammatory-demyelinating-polyradiculoneuropathy-cidp-clinical-features-diagnosis-and-current-treatment-strategies
#12
Jacques Reynolds, George Sachs, Kara Stavros
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired immune-mediated disorder characterized by weakness and sensory deficits that can lead to significant neurological disability. The diagnosis is based on a combination of clinical examination findings, electrodiagnostic studies, and other supportive evidence. Recognizing CIDP and distinguishing it from other chronic polyneuropathies is important because many patients with CIDP are highly responsive to treatment with immunosuppressive or immunomodulatory therapies...
December 1, 2016: Rhode Island Medical Journal
https://www.readbyqxmd.com/read/27900773/systematic-review-of-immunoglobulin-use-in-paediatric-neurological-and-neurodevelopmental-disorders
#13
REVIEW
Jonathan Gadian, Emma Kirk, Kate Holliday, Ming Lim, Michael Absoud
AIM: A systematic literature review of intravenous immunoglobulin (IVIG) treatment of paediatric neurological conditions was performed to summarize the evidence, provide recommendations, and suggest future research. METHOD: A MEDLINE search for articles reporting on IVIG treatment of paediatric neuroinflammatory, neurodevelopmental, and neurodegenerative conditions published before September 2015, excluding single case reports and those not in English. Owing to heterogeneous outcome measures, meta-analysis was not possible...
November 30, 2016: Developmental Medicine and Child Neurology
https://www.readbyqxmd.com/read/27896893/an-acute-disseminated-encephalomyelitis-like-illness-in-the-elderly-neuroimaging-and-neuropathology-findings
#14
Ulrike W Kaunzner, Elliott Salamon, Elena Pentsova, Marc Rosenblum, Sasan Karimi, Nancy Nealon, Ehud Lavi, Dara G Jamieson
INTRODUCTION: Acute disseminated encephalomyelitis (ADEM) is a rare demyelinating disease of the central nervous system (CNS) that classically occurs in children and adolescents. It characteristically presents with acute inflammation, resulting in demyelination, often following an infectious disease. ADEM has been described in adult patients, but the incidence in the adult and especially elderly population is low. CASES: We describe five older adults (age 57 to 85) who presented with acute neurological symptoms...
November 29, 2016: Journal of Neuroimaging: Official Journal of the American Society of Neuroimaging
https://www.readbyqxmd.com/read/27894792/pyruvate-dehydrogenase-e1%C3%AE-deficiency-presenting-as-recurrent-acute-proximal-muscle-weakness-of-upper-and-lower-extremities-in-an-8-year-old-boy
#15
Bülent Kara, Hülya Maraş Genç, Emek Uyur-Yalçın, Ayfer Sakarya-Güneş, Uğur Topçu, Serap Mülayim, Serdar Ceylaner
The mitochondrial pyruvate dehydrogenase enzyme complex (PDHC) plays an important role in aerobic energy metabolism and acid-base equilibrium. PDHC contains of 5 enzymes, 3 catalytic (E1, E2, E3) and 2 regulatory, as well as 3 cofactors and an additional protein (E3-binding protein) encoded by nuclear genes. The clinical presentation of PDHC deficiency ranges from fatal neonatal lactic acidosis to chronic neurologic dysfunction without lactic acidosis. Paroxysmal neurologic problems such as intermittent ataxia, episodic weakness, exercise-induced dystonia and recurrent demyelination may also be seen although they are rare...
November 9, 2016: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/27894447/differentiating-familial-neuropathies-from-guillain-barr%C3%A3-syndrome
#16
REVIEW
Brett J Bordini, Priya Monrad
Differentiating Guillain-Barré syndrome (GBS) from inherited neuropathies and other acquired peripheral neuropathies requires understanding the atypical presentations of GBS and its variant forms, as well as historical and physical features suggestive of inherited neuropathies. GBS is typically characterized by the acute onset of ascending flaccid paralysis, areflexia, and dysesthesia secondary to peripheral nerve fiber demyelination. The disorder usually arises following a benign gastrointestinal or respiratory illness, is monophasic, reaches a nadir with several weeks, and responds to immunomodulatory therapy...
February 2017: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/27893883/use-of-magnetic-resonance-imaging-to-visualize-leptomeningeal-inflammation-in-patients-with-multiple-sclerosis-a-review
#17
Jonathan Zurawski, Hans Lassmann, Rohit Bakshi
Importance: Multiple sclerosis (MS) is a chronic demyelinating disease of the central nervous system traditionally characterized by an initial relapsing-remitting clinical course and focal inflammatory lesions that have a predilection for the periventricular white matter. Recently, however, histopathologic and imaging studies have illustrated a more complex pathologic substrate involving cortical demyelination, gray matter atrophy, and meningeal inflammation. Neuroimaging advances have facilitated improved detection of cortical pathology, but our understanding of the pathogenesis of cortical disease remains incomplete...
November 28, 2016: JAMA Neurology
https://www.readbyqxmd.com/read/27893410/characterization-of-variations-in-il23a-and-il23r-genes-possible-roles-in-multiple-sclerosis-and-other-neuroinflammatory-demyelinating-diseases
#18
Fei-Feng Li, Xi-Dong Zhu, Peng Yan, Mei-Hua Jin, Hui Yue, Qiong Zhang, Jin Fu, Shu-Lin Liu
Multiple sclerosis is among the most serious inflammatory demyelinating diseases (IDD). Interleukin-23A (IL23A) regulates and coordinates the activities of immune cells by interacting with its receptor IL23R and plays key roles in the pathogenesis of immune inflammatory diseases. IDD, deemed to be a kind of autoimmune diseases, may involve IL23A in the pathogenesis. The aim of this work was to validate the hypothesized involvement of IL-23A and its receptor in IDD. We sequenced the IL-23A and IL-23R genes for 206 Chinese Han IDD patients and evaluated SNPs within or near those genes...
November 26, 2016: Aging
https://www.readbyqxmd.com/read/27892518/brain-microbiota-disruption-within-inflammatory-demyelinating-lesions-in-multiple-sclerosis
#19
W G Branton, J Q Lu, M G Surette, R A Holt, J Lind, J D Laman, C Power
Microbial communities reside in healthy tissues but are often disrupted during disease. Bacterial genomes and proteins are detected in brains from humans, nonhuman primates, rodents and other species in the absence of neurological disease. We investigated the composition and abundance of microbiota in frozen and fixed autopsied brain samples from patients with multiple sclerosis (MS) and age- and sex-matched nonMS patients as controls, using neuropathological, molecular and bioinformatics tools. 16s rRNA sequencing revealed Proteobacteria to be the dominant phylum with restricted diversity in cerebral white matter (WM) from MS compared to nonMS patients...
November 28, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27890181/imaging-of-vascular-compression-syndromes
#20
REVIEW
Joseph H Donahue, David A Ornan, Sugoto Mukherjee
Trigeminal neuralgia, hemifacial spasm, vestibulocochlear neuralgia and glossopharyngeal neuralgia represent the most common neurovascular compression syndromes. Repeated vascular pulsations at the vulnerable transitional zone of the individual cranial nerves lead to focal axonal injury and demyelination. High-resolution 3-D T2-weighted MR imaging is essential in detecting and mapping neurovascular compression for directed therapy. Knowledge of the specific nerve root exit, the transitional zones, and the adjacent vasculature is critical in proper management...
January 2017: Radiologic Clinics of North America
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