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https://www.readbyqxmd.com/read/29876600/-diagnosis-of-multiple-sclerosis-revision-of-the-mcdonald-criteria-2017
#1
REVIEW
O Aktas, M P Wattjes, M Stangel, H-P Hartung
Multiple sclerosis (MS) is the most common chronic autoimmune disorder of the central nervous system (CNS) largely affecting young adults. The diagnosis of MS is based on two pillars: 1) detection of the spatial and temporal dissemination of focal neurological deficits and 2) exclusion of important differential diagnoses. The current revision of the diagnostic criteria (McDonald 2017) also follows these principles, takes new data on magnetic resonance imaging (MRI) into account and reintroduces the role of cerebrospinal fluid (CSF) diagnostics for relapsing-remitting forms...
June 6, 2018: Der Nervenarzt
https://www.readbyqxmd.com/read/29788018/brain-immunohistopathology-in-a-patient-with-autoimmune-glial-fibrillary-acidic-protein-astrocytopathy
#2
Yaqing Shu, Youming Long, Yanyu Chang, Rui Li, Xiaobo Sun, Yuge Wang, Yinong Huang, Jing Li, Jianning Chen, Yu Yang, Zhengqi Lu, Xueqiang Hu, Allan G Kermode, Wei Qiu
BACKGROUND: Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is a novel meningoencephalomyelitis. However, the pathogenesis of this disease is unclear. We therefore examined a brain biopsy from a patient with autoimmune GFAP astrocytopathy by immunohistopathology. METHODS: We examined brain biopsy sections from a patient with autoimmune GFAP astrocytopathy using hematoxylin and eosin (HE) and Luxol fast blue (LFB) staining, and immunostaining with antibodies for CD4, CD8, CD3, CD20, CD68, CD138, Neu-N, GFAP, myelin oligodendrocyte glycoprotein (MOG), and aquaporin-4 (AQP4)...
May 22, 2018: Neuroimmunomodulation
https://www.readbyqxmd.com/read/29623106/defining-distinct-features-of-anti-mog-antibody-associated-central-nervous-system-demyelination
#3
REVIEW
Martin S Weber, Tobias Derfuss, Imke Metz, Wolfgang Brück
Extensive research over the last decades basically failed to identify a common cause of noninfectious inflammatory central nervous system (CNS) demyelinating disease. To a great extent, this may reflect that the group of inflammatory CNS demyelinating disorders likely contains multiple pathogenetically distinct disease entities. Indeed, the greatest success so far in deciphering the pathogenesis of a CNS demyelinating disorder resulted from the discovery of anti-aquaporin (AQP)-4 antibodies (ab), which allowed progressive delineation of neuromyelitis optica (NMO), formerly considered a variant of the most common CNS demyelinating disorder, multiple sclerosis (MS), as a distinct disease...
2018: Therapeutic Advances in Neurological Disorders
https://www.readbyqxmd.com/read/29465432/mechanisms-for-lesion-localization-in-neuromyelitis-optica-spectrum-disorders
#4
Monika Bradl, Markus Reindl, Hans Lassmann
PURPOSE OF REVIEW: Neuromyelitis optica spectrum disorders (NMOSD) are severe inflammatory diseases of the central nervous system (CNS), with the presence of aquaporin 4 (AQP4)-specific serum antibodies in the vast majority of patients, and with the presence of myelin oligodendrocyte glycoprotein (MOG)-specific antibodies in approximately 40% of all AQP4-antibody negative NMOSD patients. Despite differences in antigen recognition, the preferred sites of lesions are similar in both groups of patients: They localize to the spinal cord and to the anterior visual pathway including retina, optic nerves, chiasm, and optic tracts, and - to lesser extent - also to certain predilection sites in the brain...
June 2018: Current Opinion in Neurology
https://www.readbyqxmd.com/read/28640199/the-role-of-peripheral-cns-directed-antibodies-in-promoting-inflammatory-cns-demyelination
#5
REVIEW
Silke Kinzel, Martin S Weber
In central nervous system (CNS) demyelinating disorders, such as multiple sclerosis (MS), neuromyelitis optica (NMO) and related NMO-spectrum disorders (NMO-SD), a pathogenic role for antibodies is primarily projected into enhancing ongoing CNS inflammation by directly binding to target antigens within the CNS. This scenario is supported at least in part, by antibodies in conjunction with complement activation in the majority of MS lesions and by deposition of anti-aquaporin-4 (AQP-4) antibodies in areas of astrocyte loss in patients with classical NMO...
June 22, 2017: Brain Sciences
https://www.readbyqxmd.com/read/28326889/aquaporin-4-autoimmunity-in-patients-with-systemic-lupus-erythematosus-a-predominantly-population-based-study
#6
Nasrin Asgari, Sven Jarius, Helle Laustrup, Hanne Pb Skejoe, Soeren T Lillevang, Brian G Weinshenker, Anne Voss
BACKGROUND: Serum immunoglobulin G targeting the astrocyte water channel aquaporin-4 (AQP4) in the central nervous system (CNS) is a biomarker for neuromyelitis optica spectrum disease (NMOSD). Co-existence of NMOSD with systemic lupus erythematosus (SLE) putatively suggests susceptibility to antibody-mediated autoimmune disease. OBJECTIVE: To estimate the prevalence of NMOSD in SLE and investigate the immunogenetic background for an association of NMOSD and SLE...
March 2018: Multiple Sclerosis: Clinical and Laboratory Research
https://www.readbyqxmd.com/read/26587556/fulminant-demyelinating-encephalomyelitis-insights-from-antibody-studies-and-neuropathology
#7
Franziska Di Pauli, Romana Höftberger, Markus Reindl, Ronny Beer, Paul Rhomberg, Kathrin Schanda, Douglas Sato, Kazuo Fujihara, Hans Lassmann, Erich Schmutzhard, Thomas Berger
OBJECTIVES: Antibodies to myelin oligodendrocyte glycoprotein (MOG) are detectable in inflammatory demyelinating CNS diseases, and MOG antibody-associated diseases seem to have a better prognosis despite occasionally severe presentations. METHODS: We report the case of a 71-year-old patient with acute visual and gait disturbance that dramatically worsened to bilateral amaurosis, tetraplegia, and respiratory insufficiency within a few days. RESULTS: MRI showed multiple progressive cerebral and spinal lesions with diffusion restriction (including both optic nerves) and marginal contrast enhancement...
December 2015: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/25635259/does-mog-ig-positive-aqp4-seronegative-opticospinal-inflammatory-disease-justify-a-diagnosis-of-nmo-spectrum-disorder
#8
REVIEW
Scott S Zamvil, Anthony J Slavin
While neuromyelitis optica (NMO) immunoglobulin (Ig) G is considered the hallmark serologic marker of NMO, its association is not absolute, as NMO IgG is not detected in approximately one-fourth of the patients diagnosed with NMO spectrum disorder (NMOSD). Thus, the recent discovery that antibodies to myelin oligodendrocyte glycoprotein (MOG) are detected in some NMO IgG-seronegative patients manifesting clinical and neuroimaging signs of NMO or NMOSD has created tremendous excitement. However, it may be premature to classify this subgroup as NMOSD...
February 2015: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/25518368/-clinical-concept-etiology-and-pathology-of-neuromyelitis-optica
#9
REVIEW
Kazuhiro Kurosawa, Kazuo Fujihara
More than a century has passed since the first description of neuromyelitis optica (NMO) or Devic's disease. The relation between NMO and multiple sclerosis (MS) had long been debated, but the discovery of anti-aquaporin-4(AQP4) antibody, an NMO-specific autoantibody has accelerated clinical and experimental research of NMO, and contributed to estabilishing NMO spectrum disorder(NMOSD), a wider disease spectrum than a prototypic opticospinal phenotype and a new disease concept: autoimmune astrocytopathic disease...
November 2014: Nihon Rinsho. Japanese Journal of Clinical Medicine
https://www.readbyqxmd.com/read/25305252/idiopathic-aquaporin-4-antibody-negative-longitudinally-extensive-transverse-myelitis
#10
Jae-Won Hyun, Su-Hyun Kim, So-Young Huh, Woojun Kim, Junglim Yun, AeRan Joung, Douglas Kazutoshi Sato, Kazuo Fujihara, Ho Jin Kim
BACKGROUND: Longitudinally extensive transverse myelitis (LETM) is a characteristic manifestation of neuromyelitis optica (NMO). However, not all patients with LETM are positive for aquaporin-4 (AQP4) antibodies. We evaluated the characteristics of idiopathic isolated LETM negative for AQP4 antibodies. METHODS: From the National Cancer Center registry of inflammatory diseases of the central nervous system, patients with LETM as an initial manifestation and follow-up for at least two years were enrolled...
May 2015: Multiple Sclerosis: Clinical and Laboratory Research
https://www.readbyqxmd.com/read/25257613/severe-demyelination-but-no-astrocytopathy-in-clinically-definite-neuromyelitis-optica-with-anti-myelin-oligodendrocyte-glycoprotein-antibody
#11
Kensuke Ikeda, Naoki Kiyota, Hiroshi Kuroda, Douglas Kazutoshi Sato, Shuhei Nishiyama, Toshiyuki Takahashi, Tatsuro Misu, Ichiro Nakashima, Kazuo Fujihara, Masashi Aoki
We report a patient with neuromyelitis optica (NMO) presenting anti-myelin-oligodendrocyte glycoprotein (MOG)-seropositive, in whom biomarkers of demyelination and astrocyte damage were measured during an acute attack. A 31-year-old man developed right optic neuritis followed by longitudinally extensive transverse myelitis, fulfilling the criteria for definite NMO. He was anti-MOG-seropositive and anti-aquaporin-4 seronegative. The myelin basic protein level was markedly elevated whereas glial fibrillary acidic protein was not detectable in cerebrospinal fluid during an acute attack...
April 2015: Multiple Sclerosis: Clinical and Laboratory Research
https://www.readbyqxmd.com/read/24685353/neuromyelitis-optica-mog-igg-causes-reversible-lesions-in-mouse-brain
#12
Samira Saadoun, Patrick Waters, Gregory P Owens, Jeffrey L Bennett, Angela Vincent, Marios C Papadopoulos
INTRODUCTION: Antibodies against myelin oligodendrocyte glycoprotein (MOG-IgG) are present in some neuromyelitis optica patients who lack antibodies against aquaporin-4 (AQP4-IgG). The effects of neuromyelitis optica MOG-IgG in the central nervous system have not been investigated in vivo. We microinjected MOG-IgG, obtained from patients with neuromyelitis optica, into mouse brains and compared the results with AQP4-IgG. RESULTS: MOG-IgG caused myelin changes and altered the expression of axonal proteins that are essential for action potential firing, but did not produce inflammation, axonal loss, neuronal or astrocyte death...
March 31, 2014: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/24597481/extensive-dysregulations-of-oligodendrocytic-and-astrocytic-connexins-are-associated-with-disease-progression-in-an-amyotrophic-lateral-sclerosis-mouse-model
#13
Yiwen Cui, Katsuhisa Masaki, Ryo Yamasaki, Shihoko Imamura, Satoshi O Suzuki, Shintaro Hayashi, Shinya Sato, Yuko Nagara, Mami F Kawamura, Jun-ichi Kira
BACKGROUND: Non-cell-autonomous motor neuronal death is suggested in a mutant Cu/Zn superoxide dismutase 1 (mSOD1)-mediated amyotrophic lateral sclerosis (ALS) model, in which glial cells play significant roles in disease progression. Connexins (Cxs) form homotypic or heterotypic gap junctions (GJs) and allow direct intercellular communications among nervous tissue cells. The role of Cxs in motor neuron disease has never been investigated; therefore, we aimed to evaluate alterations of Cxs in mSOD1-transgenic (mSOD1-Tg) mice in comparison with their non-transgenic (non-Tg) littermates at the same ages...
March 6, 2014: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/23631257/-optic-neuritis-immunological-approach-to-elucidate-pathogenesis-and-develop-innovative-therapy
#14
REVIEW
Takeshi Kezuka
The pathogenesis of optic neuritis developed rapidly from the end of the 19th century to the beginning of the 20th century accompanying progress in morpho-anatomy and physiology. Thereafter, the pathology of the disease continues to be clarified with the advances in medicine and clinical peripheral devices. The analysis of optic neuritis is about to enter a new phase, triggered by the advent of molecular immunology and genetic engineering. This article describes the results of recent studies on the pathogenetic mechanism of optic neuritis and the potential of utilizing these new findings in the development of novel therapies...
March 2013: Nippon Ganka Gakkai Zasshi
https://www.readbyqxmd.com/read/22438105/extensive-loss-of-connexins-in-bal%C3%A3-s-disease-evidence-for-an-auto-antibody-independent-astrocytopathy-via-impaired-astrocyte-oligodendrocyte-myelin-interaction
#15
Katsuhisa Masaki, Satoshi O Suzuki, Takuya Matsushita, Tomomi Yonekawa, Takeshi Matsuoka, Noriko Isobe, Kyoko Motomura, Xiao-Mu Wu, Takeshi Tabira, Toru Iwaki, Jun-ichi Kira
Extensive aquaporin-4 (AQP4) loss without perivascular deposition of either activated complement or immunoglobulins is a characteristic of Baló's disease. Our aim in this study was to investigate the relationship between astrocytopathy and demyelination in Baló's disease, focusing on connexins (Cx), which form gap junctions among glial cells and myelin. Autopsied specimens from four cases that provided seven actively demyelinating concentric lesions infiltrated with numerous CD68(+) macrophages were immunohistochemically examined for the astrocyte markers glial fibrillary acidic protein (GFAP), AQP4, Cx43, Cx30 and megalencephalic leukoencephalopathy with subcortical cyst 1 (MLC1)...
June 2012: Acta Neuropathologica
https://www.readbyqxmd.com/read/22157536/relationship-between-nmo-antibody-and-anti-mog-antibody-in-optic-neuritis
#16
COMPARATIVE STUDY
Takeshi Kezuka, Yoshihiko Usui, Naoyuki Yamakawa, Yoshimichi Matsunaga, Ryusaku Matsuda, Masayuki Masuda, Hiroya Utsumi, Keiko Tanaka, Hiroshi Goto
BACKGROUND: Damage to astrocytes by anti-aquaporin-4 antibody (AQP4-Ab), also known as NMO antibody, has been implicated as the cause of neuromyelitis optica. Myelin oligodendrocyte glycoprotein (MOG) is well known as the causative protein of multiple sclerosis (MS). MOG antigen is currently considered as a cause of optic neuritis (ON) associated with MS because immunization with MOG antigen derived from oligodendrocytes induces murine ON with myelitis. We investigated the relationship between NMO antibody (NMO-Ab) and anti-MOG antibody (MOG-Ab) and potential in patients with ON for recovery of vision...
June 2012: Journal of Neuro-ophthalmology: the Official Journal of the North American Neuro-Ophthalmology Society
https://www.readbyqxmd.com/read/21257712/proinflammatory-role-of-aquaporin-4-in-autoimmune-neuroinflammation
#17
Lihua Li, Hua Zhang, Michel Varrin-Doyer, Scott S Zamvil, A S Verkman
Aquaporin-4 (AQP4) deficiency in mice reduces neuroinflammation in experimental autoimmune encephalomyelitis (EAE) produced by active immunization with myelin oligodendrocyte glycoprotein peptide (MOG). Potential mechanisms for the protective effect of AQP4 deficiency were investigated, including AQP4-dependent leukocyte and microglia cell function, immune cell entry in the central nervous system (CNS), intrinsic neuroinflammation, and humoral immune response. As we found with active-immunization EAE, neuroinflammation was greatly reduced in AQP4-knockout mice in adoptive-transfer EAE...
May 2011: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
https://www.readbyqxmd.com/read/19660138/greatly-attenuated-experimental-autoimmune-encephalomyelitis-in-aquaporin-4-knockout-mice
#18
Lihua Li, Hua Zhang, A S Verkman
BACKGROUND: The involvement of astrocyte water channel aquaporin-4 (AQP4) in autoimmune diseases of the central nervous system has been suggested following the identification of AQP4 autoantibodies in neuromyelitis optica, an inflammatory demyelinating disease. RESULTS: We investigated the involvement of AQP4 in disease severity in an established mouse model of experimental autoimmune encephalomyelitis (EAE) produced by immunization with myelin oligodendrocyte glycoprotein (MOG 35-55) peptide...
August 6, 2009: BMC Neuroscience
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