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https://www.readbyqxmd.com/read/29904974/anti-glycine-receptor-antibody-related-disease-a-case-series-and-literature-review
#1
Andrew Swayne, Linda Tjoa, Simon Broadley, Sasha Dionisio, David Gillis, Leslie Jacobson, Mark R Woodhall, Andrew McNabb, Daniel Schweitzer, Ben Tsang, Angela Vincent, Sarosh R Irani, Richard Wong, Patrick Waters, Stefan Blum
INTRODUCTION: Antibodies to glycine receptors (GlyR-Abs) were first defined in progressive encephalopathy with rigidity and myoclonus (PERM), but subsequently identified in other clinical presentations. We aimed to assess the clinical associations of all patients identified with GlyR-Abs in Queensland, Australia between April 2014 to May 2017, and compared these to cases reported in the literature. METHODS: A literature review identified the clinical features of all published GlyR-Ab positive cases through online databases...
June 15, 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/29901009/a-case-of-primary-sj%C3%A3-gren-s-syndrome-presenting-as-mass-like-encephalitis-with-progression-to-neuromyelitis-optica-spectrum-disorder
#2
Hyun-Jung Lee, Sung Hae Chang, Eun Ha Kang, Yun Jong Lee, Yeong Wook Song, You-Jung Ha
Neuromyelitis optica is an idiopathic inflammatory demyelinating disease of the central nervous system (CNS) that predominantly affects the optic nerves and spinal cord. With the discovery of the pathogenic anti-aquaporin-4 (AQP4) antibody, the disease was recognized as part of a spectrum of autoimmune diseases that target AQP4, collectively referred to as neuromyelitis optica spectrum disorder (NMOSD). NMOSD consists of conditions that affect various parts of the CNS with the AQP4 antibody. In this article, we report a 43-year-old female patient who was initially diagnosed with primary Sjögren's syndrome (pSS) with CNS involvement, but was later diagnosed with overlapping pSS and NMOSD, which required more intensive treatment...
December 2017: Archives of Rheumatology
https://www.readbyqxmd.com/read/29882008/overview-of-therapeutic-plasma-exchange-in-pediatric-neurology-a-single-center-experience
#3
Murat Özkale, Ilknur Erol, Yasemin Özkale, İlknur Kozanoğlu
Therapeutic plasma exchange (TPE) is used in the treatment of neurological, hematological, renal and autoimmune diseases with known or suspected immune pathogenesis. In comparison with neurological diseases of adults, knowledge about the use of TPE in children is incomplete. We report our experience on TPE in children with neurological diseases in a single institution and describe the underlying etiology, clinical course, treatment and outcome. We retrospectively evaluated 22 consecutive children (12 girls, 10 boys, aged 2-16 years) who underwent TPE in the pediatric intensive care unit between January 2010 and January 2017...
June 7, 2018: Acta Neurologica Belgica
https://www.readbyqxmd.com/read/29873694/the-compartmentalized-inflammatory-response-in-the-multiple-sclerosis-brain-is-composed-of-tissue-resident-cd8-t-lymphocytes-and-b-cells
#4
Joana Machado-Santos, Etsuji Saji, Anna R Tröscher, Manuela Paunovic, Roland Liblau, Galina Gabriely, Christian G Bien, Jan Bauer, Hans Lassmann
Multiple sclerosis is an inflammatory demyelinating disease in which active demyelination and neurodegeneration are associated with lymphocyte infiltrates in the brain. However, so far little is known regarding the phenotype and function of these infiltrating lymphocyte populations. In this study, we performed an in-depth phenotypic characterization of T and B cell infiltrates in a large set of multiple sclerosis cases with different disease and lesion stages and compared the findings with those seen in inflammatory, non-inflammatory and normal human controls...
June 4, 2018: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/29728463/biochemically-altered-myelin-triggers-autoimmune-demyelination
#5
Andrew V Caprariello, James A Rogers, Megan L Morgan, Vahid Hoghooghi, Jason R Plemel, Adam Koebel, Shigeki Tsutsui, Jeffrey F Dunn, Lakshmi P Kotra, Shalina S Ousman, V Wee Yong, Peter K Stys
Although immune attack against central nervous system (CNS) myelin is a central feature of multiple sclerosis (MS), its root cause is unresolved. In this report, we provide direct evidence that subtle biochemical modifications to brain myelin elicit pathological immune responses with radiological and histological properties similar to MS lesions. A subtle myelinopathy induced by abbreviated cuprizone treatment, coupled with subsequent immune stimulation, resulted in lesions of inflammatory demyelination. The degree of myelin injury dictated the resulting immune response; biochemical damage that was too limited or too extensive failed to trigger overt pathology...
May 4, 2018: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29702605/advanced-glycation-endproducts-are-increased-in-the-animal-model-of-multiple-sclerosis-but-cannot-be-reduced-by-pyridoxamine-treatment-or-glyoxalase-1-overexpression
#6
Suzan Wetzels, Kristiaan Wouters, Toshio Miyata, Jean L J M Scheijen, Jerome J A Hendriks, Casper G Schalkwijk, Tim Vanmierlo
Multiple sclerosis (MS) is a demyelinating autoimmune disease of the central nervous system (CNS). The immune response in MS patients leads to the infiltration of immune cells in the CNS and their subsequent activation. Immune cell activation induces a switch towards glycolysis. During glycolysis, the dicarbonyl product methylglyoxal (MGO) is produced. MGO is a glycating agent that can rapidly form advanced glycation endproducts (AGEs). In turn, AGEs are able to induce inflammatory responses. The glyoxalase system is the endogenous defense system of the body to reduce the burden of MGO thereby reducing AGE formation...
April 27, 2018: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29670575/mog-igg-associated-optic-neuritis-encephalitis-and-myelitis-lessons-learned-from-neuromyelitis-optica-spectrum-disorder
#7
REVIEW
Giordani Rodrigues Dos Passos, Luana Michelli Oliveira, Bruna Klein da Costa, Samira Luisa Apostolos-Pereira, Dagoberto Callegaro, Kazuo Fujihara, Douglas Kazutoshi Sato
Antibodies against myelin oligodendrocyte glycoprotein (MOG-IgG) have been found in some cases diagnosed as seronegative neuromyelitis optica spectrum disorder (NMOSD). MOG-IgG allowed the identification of a subgroup with a clinical course distinct from that of NMOSD patients who are seropositive for aquaporin-4-IgG antibodies. MOG-IgG is associated with a wider clinical phenotype, not limited to NMOSD, with the majority of cases presenting with optic neuritis (ON), encephalitis with brain demyelinating lesions, and/or myelitis...
2018: Frontiers in Neurology
https://www.readbyqxmd.com/read/29661538/an-unusual-case-of-anti-mog-cns-demyelination-with-concomitant-mild-anti-nmdar-encephalitis
#8
Jiajia Zhou, Winwen Tan, Suyin Elaine Tan, Jing Hu, Zhongqin Chen, Kang Wang
We report the case of a patient who presented with progressive unsteadiness and narcoleptic attacks followed by behavioral change and psychosis, without visual disturbances or seizures. MRI revealed multiple areas of fluid attenuation inversion recovery (FLAIR) high-intensity lesions involving the cerebellum, brainstem, thalamus and third ventricular peri-ependymal region consistent with demyelination. Both the serum myelin oligodendrocyte glycoprotein-antibodies (MOG-Abs) and cerebral spinal fluid (CSF) anti-N-methyl-d-as-partate receptor (NMDAR) antibodies were positive using transfected cell based assays...
July 15, 2018: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/29629942/paediatric-multiple-sclerosis-and-other-acute-demyelinating-diseases
#9
Kevin Rostásy, Barbara Bajer-Kornek
PURPOSE OF REVIEW: Neuroimmunological diseases encompass a wide spectrum of diseases in children. Apart from the discovery of autoantibodies affecting primarily grey matter structures and the improved clinical characterization of rare entities such as N-methyl D-aspartate receptor-R- encephalitis, important strides have also been made in autoimmune-mediated white matter diseases, including paediatric multiple sclerosis (pedMS) and other acute demyelinating syndromes (ADS) often associated with antibodies (abs) against myelin-oligodendrocyte-glycoprotein (MOG)...
June 2018: Current Opinion in Neurology
https://www.readbyqxmd.com/read/29604982/hiv-neuropathology
#10
Susan Morgello
Primary human immunodeficiency virus (HIV) neuropathologies can affect all levels of the neuraxis and occur in all stages of natural history disease. Some, like HIV encephalitis, HIV myelitis, and diffuse infiltrative lymphocytosis of peripheral nerve, reflect productive infection of the nervous system; others, like vacuolar myelopathy, distal symmetric polyneuropathy, and central and peripheral nervous system demyelination, are not clearly related to regional viral replication, and reflect more complex cascades of dysregulated host immunity and metabolic dysfunction...
2018: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/29604979/hiv-and-spinal-cord-disease
#11
Seth N Levin, Jennifer L Lyons
The epidemiology of spinal cord disease in human immunodeficiency virus (HIV) infection is largely unknown due to a paucity of data since combination antiretroviral therapy (cART). HIV mediates spinal cord injury indirectly, by immune modulation, degeneration, or associated infections and neoplasms. The pathologies vary and range from cytotoxic necrosis to demyelination and vasculitis. Control of HIV determines the differential for all neurologic presentations in infected individuals. Primary HIV-associated acute transverse myelitis, an acute inflammatory condition with pathologic similarities to HIV encephalitis, arises in early infection and at seroconversion...
2018: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/29593631/acute-hemorrhagic-encephalitis-responding-to-combined-decompressive-craniectomy-intravenous-immunoglobulin-and-corticosteroid-therapies-association-with-novel-ranbp2-variant
#12
Abdulla Alawadhi, Christine Saint-Martin, Farhan Bhanji, Myriam Srour, Jeffrey Atkinson, Guillaume Sébire
Background: Acute hemorrhagic encephalomyelitis (AHEM) is considered as a rare form of acute disseminated encephalomyelitis characterized by fulminant encephalopathy with hemorrhagic necrosis and most often fatal outcome. Objective: To report the association with Ran Binding Protein ( RANBP2) gene variant and the response to decompressive craniectomy and high-dose intravenous methylprednisolone (IVMP) in life-threatening AHEM. Design: Single case study...
2018: Frontiers in Neurology
https://www.readbyqxmd.com/read/29574788/mechanisms-underlying-lesion-development-and-lesion-distribution-in-cns-autoimmunity
#13
REVIEW
Maren Lindner, Luisa Klotz, Heinz Wiendl
It is widely accepted that development of autoimmunity in the central nervous system (CNS) is triggered by autoreactive T cells, that are activated in the periphery and gain the capacity to migrate through endothelial cells at the blood-brain barrier (BBB) into the CNS. Upon local reactivation, an inflammatory cascade is initiated, that subsequently leads to a recruitment of additional immune cells ultimately causing demyelination and axonal damage. Even though the interaction of immune cells with the BBB has been in the focus of research for many years, the exact mechanisms of how immune cells enter and exit the CNS remains poorly understood...
March 25, 2018: Journal of Neurochemistry
https://www.readbyqxmd.com/read/29549218/clinical-and-pathogenic-significance-of-igg-iga-and-igm-antibodies-against-the-nmda-receptor
#14
Makoto Hara, Eugenia Martinez-Hernandez, Helena Ariño, Thais Armangué, Marianna Spatola, Mar Petit-Pedrol, Albert Saiz, Myrna R Rosenfeld, Francesc Graus, Josep Dalmau
OBJECTIVE: To determine the frequency and clinical relevance of immunoglobulin (Ig)G, IgA, and IgM N -methyl-d-aspartate receptor (NMDAR) antibodies in several diseases, and whether the IgG antibodies occur in disorders other than anti-NMDAR encephalitis. METHODS: Evaluation of IgG, IgA, and IgM NMDAR antibodies in serum of 300 patients with anti-NMDAR encephalitis, stroke, dementia, schizophrenia, or seronegative autoimmune encephalitis. Antibodies and their effect on cultured neurons were examined with cell-based assays and brain and live neuronal immunostaining...
April 17, 2018: Neurology
https://www.readbyqxmd.com/read/29542041/atx-ms-1467-induces-long-term-tolerance-to-myelin-basic-protein-in-dr2-%C3%A3-ob1-f1-mice-by-induction-of-il-10-secreting-itregs
#15
Adriano Luís Soares De Souza, Stefan Rudin, Rui Chang, Keith Mitchell, Timothy Crandall, Shuning Huang, Ji-Kyung Choi, Shinji L Okitsu, Danielle L Graham, Blake Tomkinson, Tammy Dellovade
INTRODUCTION: Antigen-specific immunotherapy could provide a targeted approach for the treatment of multiple sclerosis that removes the need for broad-acting immunomodulatory drugs. ATX-MS-1467 is a mixture of four peptides identified as the main immune-dominant disease-associated T-cell epitopes in myelin basic protein (MBP), an autoimmune target for activated autoreactive T cells in multiple sclerosis. Previous animal studies have shown that ATX-MS-1467 treatment prevented the worsening of signs of disease in experimental autoimmune encephalitis (EAE) in the humanized (DR2 × Ob1)F1 mouse in a dose-dependent fashion...
March 14, 2018: Neurology and Therapy
https://www.readbyqxmd.com/read/29526318/a-comparative-study-of-melatonin-and-immunomodulatory-therapy-with-interferon-beta-and-glatiramer-acetate-in-a-mouse-model-of-multiple-sclerosis
#16
E J Ramos González, L J Ramirez Jirano, D Z García Martínez, G G Ortiz, L F Jave Suárez, C A Leal Cortes, O K Bitzer Quintero
INTRODUCTION: Multiple sclerosis (MS) is a chronic, demyelinating, autoimmune disease of the central nervous system causing neuroinflammation. Experimental autoimmune encephalitis (EAE) is a model of the disease. MS is classically treated with interferon beta (IFN-β) and glatiramer acetate (GA). Melatonin (MLT) has been reported to modulate immune system responses. The aim of the present study is to analyse the effects of MLT administration in comparison with the first-line treatments for MS (IFN-β and GA)...
March 8, 2018: Neurología: Publicación Oficial de la Sociedad Española de Neurología
https://www.readbyqxmd.com/read/29459060/bickerstaff-s-brainstem-encephalitis-with-overlapping-guillain-barr%C3%A3-syndrome-usefulness-of-sequential-nerve-conduction-studies
#17
Choong Yi Fong, Hnin Wint Wint Aung, Arie Khairani, Chin Seng Gan, Nortina Shahrizaila, Khean Jin Goh
Bickerstaff's brainstem encephalitis (BBE) is a rare immune-mediated disorder characterized by ophthalmoplegia, ataxia and disturbance of consciousness, which may overlap with Guillain-Barré syndrome (GBS) if there is additional limb weakness. We report a 7-month-old boy presented with ophthalmoplegia followed by a rapidly ascending paralysis of all four limbs and disturbance of consciousness. The initial impression was BBE with overlapping GBS. This was supported by sequential nerve conduction study (NCS) findings compatible with an acute inflammatory demyelinating polyneuropathy (AIDP)...
June 2018: Brain & Development
https://www.readbyqxmd.com/read/29426675/the-use-of-cerebral-imaging-for-investigating-delirium-aetiology
#18
Zina Hijazi, Peter Lange, Rosie Watson, Andrea B Maier
BACKGROUND: This study aims to investigate the frequency and patterns of use of cerebral imaging in delirium and to describe pathological changes associated with delirium using computed tomography (CT) and magnetic resonance imaging (MRI). METHODS: This retrospective observational study included patients with delirium admitted to a tertiary hospital (The Royal Melbourne Hospital, Australia) between January 2015 and August 2016. Data on cerebral imaging was collected and positive imaging findings were defined as: Acute or subacute infarct, haemorrhage, abscess, neoplasm, vasculitis, posterior reversible encephalopathy syndrome, encephalitis, acute demyelination, or fat embolism...
February 6, 2018: European Journal of Internal Medicine
https://www.readbyqxmd.com/read/29423617/human-t-cell-lymphotropic-virus-htlv-associated-encephalopathy-an-under-recognised-cause-of-acute-encephalitis-case-series-and-literature-review
#19
Ania A Crawshaw, Divya Dhasmana, Brynmor Jones, Carolyn M Gabriel, Steve Sturman, Nicholas W S Davies, Graham P Taylor
Human T-cell lymphotropic virus (HTLV)-1-associated myelopathy (HAM) is well described. Clinical features are predominantly consistent with cord pathology, though imaging and autopsy studies also demonstrate brain inflammation. In general, this is subclinical; however, six cases have previously been reported of encephalopathy in HTLV-1-infected patients, without alternative identified aetiology. We describe three further cases of encephalitis in the UK HAM cohort (n = 142), whereas the annual incidence of acute encephalitis in the general population is 0...
April 2018: Journal of Neurology
https://www.readbyqxmd.com/read/29368307/hashimoto-s-encephalopathy-presenting-as-pseudobulbar-palsy
#20
Gokcen Oz Tuncer, Serap Teber, Muhammed Gültekin Kutluk, Pelin Albayrak, Gülhis Deda
INTRODUCTION: Hashimoto's encephalopathy (HE) is an autoimmune condition with varied neurological and psychiatric features. HE is very unusual as a cause of pseudobulbar palsy (PSP). CASE PRESENTATION: A 14-year-old male was admitted with right-sided weakness, dysphagia, speech disorder, and aggressiveness. Brain magnetic resonance imaging showed increased intensity in bilateral temporal, insular cortex, amygdala, and parahippocampal area on T2-weighted and fluid-attenuated inversion recovery images...
January 24, 2018: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
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