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CSF and autoimmune disease

C G Bien
The paraneoplastic and autoimmune encephalitides are now well-established entities. Detection of neural autoantibodies enables specific diagnoses, provides information on the underlying disease pathophysiology, immunological treatability and the likelihood of a tumor being the underlying cause. This is true for the "high ranking" neural antibodies that have been established in the context of circumscribed clinical images and in consideration of large control groups, have been found in the same way by other laboratories and they respond to immunotherapy...
March 13, 2018: Der Nervenarzt
Monica Moresco, Mariangela Lecciso, Darina Ocadlikova, Marco Filardi, Silvia Melzi, Birgitte Rahbek Kornum, Elena Antelmi, Fabio Pizza, Emmanuel Mignot, Antonio Curti, Giuseppe Plazzi
BACKGROUND: Type 1 narcolepsy (NT1) is a central hypersomnia linked to the destruction of hypocretin-producing neurons. A great body of genetic and epidemiological data points to likely autoimmune disease aetiology. Recent reports have characterized peripheral blood T-cell subsets in NT1, whereas data regarding the cerebrospinal fluid (CSF) immune cell composition are lacking. The current study aimed to characterize the T-cell and natural killer (NK) cell subsets in NT1 patients with long disease course...
April 2018: Sleep Medicine
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
Yu Zhang, Mingqin Zhu, Lifang Wang, Miao Shi, Hui Deng
RATIONALE: Longitudinally extensive transverse myelitis (LETM) is characterized by contiguous inflammatory lesions of spinal cord extending to ≥3 vertebral segments. The etiology of LETM is complicated, including various infection, autoimmune disease, and so on. Neuromyelitis optic spectrum disorder (NMOSD) is the most common cause of LETM. Several case reports have suggested the associations between NMOSD and pulmonary tuberculosis (PTB). PATIENT CONCERNS: Patient 1, a 20-year-old woman who had a past history of PTB, presented with weakness, numbness, and pain in the limbs...
January 2018: Medicine (Baltimore)
Jie Li, Yan Xu, Haitao Ren, Yicheng Zhu, Bin Peng, Liying Cui
Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is a novel autoimmune disease. This is the first report of a case of autoimmune GFAP astrocytopathy after herpes simplex viral encephalitis (HSVE). A 35-year-old female patient presented with a combination of headache, fever, seizure and psychiatric/behavioral abnormalities. She had GFAP-IgG in both serum and cerebrospinal fluid (CSF). Magnetic resonance imaging (MRI) with gadolinium-enhancement revealed linear enhancement oriented radially to the ventricles...
February 19, 2018: Multiple Sclerosis and related Disorders
Jingtao Cui, Wenjuan Yan, Shaoxia Xu, Qiaofeng Wang, Weihong Zhang, Wenjing Liu, Anping Ni
BACKGROUND: Epstein-Barr virus (EBV) is associated with nasopharyngeal carcinoma (NPC) which is prevalent in South China, and its association with systemic lupus erythematosus (SLE) or other autoimmune diseases has not been studied in the mainland of China. The EBV serological tests have been performed on patients with various diseases or manifestations for years at our institution and their values need to be evaluated. METHODS: For routine medical purposes, anti-EB viral capsid antigen (VCA) IgG, IgA and IgM antibodies, anti-EBV diffuse early antigen (EA-D) IgA antibodies, and anti-EBV nuclear antigen-1(EBNA-1) IgG antibodies were tested with commercial enzyme-linked immunosorbent assay (ELISA) in patients visiting Peking Union Medical College Hospital between 2013 and 2017...
2018: PloS One
Natasha Warikoo, Samuel J Brunwasser, Ann Benz, Hong-Jin Shu, Steven M Paul, Michael Lewis, James Doherty, Michael Quick, Laura Piccio, Charles F Zorumski, Gregory S Day, Steven Mennerick
N-methyl-D-aspartate receptors (NMDARs) are ionotropic glutamate receptors important for synaptic plasticity, memory, and neuropsychiatric health. NMDAR hypofunction contributes to multiple disorders, including anti-NMDAR encephalitis (NMDARE), an autoimmune disease of the central nervous system associated with GluN1 antibody-mediated NMDAR internalization. Here we characterize the functional/pharmacological consequences of exposure to cerebrospinal fluid (CSF) from female human NMDARE patients on NMDAR function, and we characterize the effects of intervention with recently described positive allosteric modulators (PAMs) of NMDARs...
February 23, 2018: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
Kari M Gurtner, Eslam Shosha, Sandra C Bryant, Bruna D Andreguetto, David L Murray, Sean J Pittock, Maria Alice V Willrich
BACKGROUND: Cerebrospinal fluid (CSF) used in immunoglobulin gamma (IgG) index testing and oligoclonal bands (OCBs) are common laboratory tests used in the diagnosis of multiple sclerosis. The measurement of CSF free light chains (FLC) could pose as an alternative to the labor-intensive isoelectric-focusing (IEF) gels used for OCBs. METHODS: A total of 325 residual paired CSF and serum specimens were obtained after physician-ordered OCB IEF testing. CSF kappa (cKFLC) and lambda FLC (cLFLC), albumin and total IgG were measured...
February 19, 2018: Clinical Chemistry and Laboratory Medicine: CCLM
Joost M Costerus, Matthijs C Brouwer, Diederik van de Beek
Technological advances have changed the indications for and the way in which lumbar puncture is done. Suspected CNS infection remains the most common indication for lumbar puncture, but new molecular techniques have broadened CSF analysis indications, such as the determination of neuronal autoantibodies in autoimmune encephalitis. New screening techniques have increased sensitvity for pathogen detection and can be used to identify pathogens that were previously unknown to cause CNS infections. Evidence suggests that potential treatments for neurodegenerative diseases, such as Alzheimer's disease, will rely on early detection of the disease with the use of CSF biomarkers...
March 2018: Lancet Neurology
Nicolau Beckmann, Elisa Giorgetti, Anna Neuhaus, Stefan Zurbruegg, Nathalie Accart, Paul Smith, Julien Perdoux, Ludovic Perrot, Mark Nash, Sandrine Desrayaud, Peter Wipfli, Wilfried Frieauff, Derya R Shimshek
Multiple sclerosis (MS) is a chronic inflammatory disease affecting the central nervous system (CNS). While multiple effective immunomodulatory therapies for MS exist today, they lack the scope of promoting CNS repair, in particular remyelination. Microglia play a pivotal role in regulating myelination processes, and the colony-stimulating factor 1 (CSF-1) pathway is a key regulator for microglia differentiation and survival. Here, we investigated the effects of the CSF-1 receptor kinase inhibitor, BLZ945, on central myelination processes in the 5-week murine cuprizone model by non-invasive and longitudinal magnetic resonance imaging (MRI) and histology...
February 15, 2018: Acta Neuropathologica Communications
J Deng, F Fang, X H Wang, M Ge, L J He, N Zhang
Objective: To summarize the clinical and pathological features of small vessel-childhood primary angiitis of the central nervous system (SV-cPACNS), discuss the immune therapy and increase the attention to brain biopsy in SV-cPACNS. Methods: The clinical data, pathology of brain biopsy, treatment and outcome of an SV-cPACNS patient hospitalized in Beijing Children's Hospital in February 2016 were analyzed retrospectively. The cases reported at Pubmed, CNKI and Wanfang databases from 2007 to 2017 were searched, the clinical and pathological features, immunotherapy and prognosis of the disease were summarized according to the literature review...
February 2, 2018: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
Anupam Kumar, Basem Abdelmalak, Yoshikazu Inoue, Daniel A Culver
Pulmonary alveolar proteinosis (PAP) is a diffuse lung disease that results from the accumulation of lipoproteinaceous material in the alveoli and alveolar macrophages due to abnormal surfactant homoeostasis. Identification of the granulocyte-macrophage colony-stimulating factor (GM-CSF) as an indispensable mediator of macrophage maturation and surfactant catabolism was the key discovery leading to the current understanding of the pathogenesis of most forms of PAP. Impaired GM-CSF bioavailability due to anti-GM-CSF autoimmunity is the cause of approximately 90% of adult PAP cases...
January 31, 2018: Lancet Respiratory Medicine
Aoife M McGinley, Sarah C Edwards, Mathilde Raverdeau, Kingston H G Mills
Experimental autoimmune encephalomyelitis (EAE) is an animal model of multiple sclerosis (MS) that shares many features with the human disease. This review will focus on the role of IL-17-secreting CD4 and γδ T cells in EAE and MS, the plasticity of Th17 cells in vivo and the application of these findings to the understating of the pathogenesis and the development of new treatments for MS. There is convincing evidence that IL-17-secreting CD4 T cells (Th17 cells) and IL-17-secreting γδ T cells play a critical pathogenic role in central nervous system (CNS) inflammation in EAE and MS...
January 21, 2018: Journal of Autoimmunity
Christian Nusshag, Christian Morath, Martin Zeier, Markus A Weigand, Uta Merle, Thorsten Brenner
RATIONALE: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease entity primarily described in children, but not less relevant in adults. It is characterized by a misdirected activation of the immune system, resulting in an uncontrolled cytokine release from macrophages and cytotoxic T-cells (CTLs). Primary HLH relies on a genetic predisposition, whereas secondary HLH develops in the context of infections, malignancies or autoimmune diseases. However, the awareness and therapeutic knowledge for HLH in adulthood is limited...
December 2017: Medicine (Baltimore)
Alberto Vogrig, Bastien Joubert, Francois Ducray, Laure Thomas, Cristina Izquierdo, Kévin Decaestecker, Olivier Martinaud, Emmanuel Gerardin, Sylvie Grand, Jérome Honnorat
OBJECTIVE: To identify the clinical and radiological features that should raise suspicion for the autoimmune encephalitis (AE)-like presentation of glioblastoma. METHODS: This is an observational, retrospective case series of patients referred to the French National Reference Center on Paraneoplastic Neurological Diseases for suspected AE (possible, probable or definite, using the 2016 criteria) who later received a final diagnosis of glioblastoma according to 2016 WHO criteria...
January 30, 2018: Journal of Neurology
Aude Belbezier, Bastien Joubert, Gonzalo Montero-Martin, Marcelo Fernandez-Vina, Nicole Fabien, Véronique Rogemond, Emmanuel Mignot, Jérôme Honnorat
Objective: Neurologic autoimmune syndromes associated with anti-glutamate acid decarboxylase 65 antibodies (GAD65-Abs) are rare and mostly sporadic. Methods: We describe a niece and her aunt with GAD65-Abs neurologic syndromes. High-resolution HLA typing of Class I and Class II alleles was performed using next-generation sequencing. Results: The proband had cerebellar ataxia and probable limbic encephalitis features, whereas her niece had stiff-person syndrome...
January 2018: Neurology® Neuroimmunology & Neuroinflammation
Roy O Weller, Matthew M Sharp, Myron Christodoulides, Roxana O Carare, Kjeld Møllgård
Meninges that surround the CNS consist of an outer fibrous sheet of dura mater (pachymeninx) that is also the inner periosteum of the skull. Underlying the dura are the arachnoid and pia mater (leptomeninges) that form the boundaries of the subarachnoid space. In this review we (1) examine the development of leptomeninges and their role as barriers and facilitators in the foetal CNS. There are two separate CSF systems during early foetal life, inner CSF in the ventricles and outer CSF in the subarachnoid space...
January 24, 2018: Acta Neuropathologica
Belinda J Kaskow, Clare Baecher-Allan
Multiple sclerosis (MS) has long been considered a CD4 T-cell disease, primarily because of the findings that the strongest genetic risk for MS is the major histocompatibility complex (MHC) class II locus, and that T cells play a central role in directing the immune response. The importance that the T helper (Th)1 cytokine, interferon γ (IFN-γ), and the Th17 cytokine, interleukin (IL)-17, play in MS pathogenesis is indicated by recent clinical trial data by the enhanced presence of Th1/Th17 cells in central nervous system (CNS) tissue, cerebrospinal fluid (CSF), and blood, and by research on animal models of MS, such as experimental autoimmune encephalomyelitis (EAE)...
January 22, 2018: Cold Spring Harbor Perspectives in Medicine
Baozhu Liu, Zuoshan Xie, Guanghui Liu, Yong Gu, Suyue Pan, Honghao Wang
BACKGROUND: Anti‑N‑methyl‑d‑aspartate receptor (NMDAR) encephalitis is a relatively common autoimmune neurological disease of the central nervous system (CNS). Neuron-specific enolase (NSE) and S100 calcium-binding protein B (S100B) are structural proteins of the central nervous system (CNS). In patients with CNS injury accompanied by nervous tissue and cellular damage, these structural proteins are released from cells; their extracellular concentrations, including those in cerebrospinal fluid (CSF) and blood, subsequently increase...
January 9, 2018: Clinica Chimica Acta; International Journal of Clinical Chemistry
Abbas Shapouri Moghaddam, Saeed Mohammadian, Hossein Vazini, Mahdi Taghadosi, Seyed-Alireza Esmaeili, Fatemeh Mardani, Bita Seifi, Asadollah Mohammadi, Jalil Tavakol Afshari, Amirhossein Sahebkar
Macrophages are heterogeneous and their phenotype and functions are regulated by the surrounding micro-environment. Macrophages commonly exist in two distinct subsets: 1) Classically activated or M1 macrophages, which are pro-inflammatory and polarized by lipopolysaccharide (LPS) either alone or in association with Th1 cytokines such as IFN-γ, GM-CSF and produce pro-inflammatory cytokines such as interleukin-1β (IL-1β), IL-6, IL-12, IL-23 and TNF-α, and 2) Alternatively activated or M2 macrophages, which are anti-inflammatory and immunoregulatory and polarized by Th2 cytokines such as IL-4 and IL-13 and produce anti-inflammatory cytokines such as IL-10 and TGF-β...
January 10, 2018: Journal of Cellular Physiology
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