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Author Sonja Hochmeister

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A Fulminant Case of Demyelinating Encephalitis With Extensive Cortical Involvement Associated With Anti-MOG Antibodies.

Sonja Hochmeister, Thomas Gattringer, Martin Asslaber, Verena Stangl, Michaela Tanja Haindl, Christian Enzinger, Romana Höftberger,

Anti-myelin oligodendrocyte glycoprotein (MOG) antibodies (MOG-Abs) are commonly associated with clinical presentations as acute disseminated encephalomyelitis (ADEM) in both adults and children and anti-aquaporin 4 antibody-seronegative neuromyelitis optica spectrum disorder (NMOSD) and related syndromes such as optic neuritis, myelitis, and brainstem encephalitis. Most often, the presence of MOG-Abs is associated ... Read more >>

Front Neurol (Frontiers in neurology)
[2020, 11:31]

Cited: 0 times

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The pathology of central nervous system inflammatory demyelinating disease accompanying myelin oligodendrocyte glycoprotein autoantibody.

Romana Höftberger, Yong Guo, Eoin P Flanagan, A Sebastian Lopez-Chiriboga, Verena Endmayr, Sonja Hochmeister, Damir Joldic, Sean J Pittock, Jan Mendelt Tillema, Mark Gorman, Hans Lassmann, Claudia F Lucchinetti,

We sought to define the pathological features of myelin oligodendrocyte glycoprotein (MOG) antibody associated disorders (MOGAD) in an archival autopsy/biopsy cohort. We histopathologically analyzed 2 autopsies and 22 brain biopsies from patients with CNS inflammatory demyelinating diseases seropositive for MOG-antibody by live-cell-based-assay with full length MOG in its conformational form. MOGAD autopsies ... Read more >>

Acta Neuropathol. (Acta neuropathologica)
[2020, 139(5):875-892]

Cited: 0 times

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The formation of a glial scar does not prohibit remyelination in an animal model of multiple sclerosis.

Michaela Tanja Haindl, Ulrike Köck, Milena Zeitelhofer-Adzemovic, Franz Fazekas, Sonja Hochmeister,

The role of astrocytes in the pathophysiology of multiple sclerosis (MS) is discussed controversially. Especially the formation of the glial scar is often believed to act as a barrier for remyelination. At the same time, astrocytes are known to produce factors that influence oligodendrocyte precursor cell (OPC) survival. To explore ... Read more >>

Glia (Glia)
[2019, 67(3):467-481]

Cited: 2 times

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Systematic Review: Syndromes, Early Diagnosis, and Treatment in Autoimmune Encephalitis.

Christina Hermetter, Franz Fazekas, Sonja Hochmeister,

In recent years, new antibodies have been discovered which mediate autoimmune encephalitis. This immunological response can be triggered by an infection or a tumor. Classical onconeuronal antibodies are directed against intracellular neuronal agents but recently, a novel group of antibodies to neuronal cell-surface and synaptic antigens associated with different CNS-syndromes, ... Read more >>

Front Neurol (Frontiers in neurology)
[2018, 9:706]

Cited: 9 times

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Widespread cortical demyelination of both hemispheres can be induced by injection of pro-inflammatory cytokines via an implanted catheter in the cortex of MOG-immunized rats.

Muammer Üçal, Michaela Tanja Haindl, Milena Z Adzemovic, Johannes Strasser, Lisa Theisl, Manuel Zeitelhofer, Klaus Kraitsy, Stefan Ropele, Ute Schäfer, Franz Fazekas, Sonja Hochmeister,

Cortical demyelination is a common finding in patients with chronic multiple sclerosis (MS) and contributes to disease progression and overall disability. The exact pathomechanism that leads to cortical lesions is not clear. Research is limited by the fact that standard animal models of multiple sclerosis do not commonly affect the ... Read more >>

Exp. Neurol. (Experimental neurology)
[2017, 294:32-44]

Cited: 1 time

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Functional genomics analysis of vitamin D effects on CD4+ T cells in vivo in experimental autoimmune encephalomyelitis ‬.

Manuel Zeitelhofer, Milena Z Adzemovic, David Gomez-Cabrero, Petra Bergman, Sonja Hochmeister, Marie N'diaye, Atul Paulson, Sabrina Ruhrmann, Malin Almgren, Jesper N Tegnér, Tomas J Ekström, André Ortlieb Guerreiro-Cacais, Maja Jagodic,

Vitamin D exerts multiple immunomodulatory functions and has been implicated in the etiology and treatment of several autoimmune diseases, including multiple sclerosis (MS). We have previously reported that in juvenile/adolescent rats, vitamin D supplementation protects from experimental autoimmune encephalomyelitis (EAE), a model of MS. Here we demonstrate that this protective ... Read more >>

Proc. Natl. Acad. Sci. U.S.A. (Proceedings of the National Academy of Sciences of the United States of America)
[2017, 114(9):E1678-E1687]

Cited: 11 times

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Lipocalin-2 as an Infection-Related Biomarker to Predict Clinical Outcome in Ischemic Stroke.

Sonja Hochmeister, Odilo Engel, Milena Z Adzemovic, Thomas Pekar, Paul Kendlbacher, Manuel Zeitelhofer, Michaela Haindl, Andreas Meisel, Franz Fazekas, Thomas Seifert-Held,

OBJECTIVES:From previous data in animal models of cerebral ischemia, lipocalin-2 (LCN2), a protein related to neutrophil function and cellular iron homeostasis, is supposed to have a value as a biomarker in ischemic stroke patients. Therefore, we examined LCN2 expression in the ischemic brain in an animal model and measured plasma ... Read more >>

PLoS ONE (PloS one)
[2016, 11(5):e0154797]

Cited: 6 times

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Highly encephalitogenic aquaporin 4-specific T cells and NMO-IgG jointly orchestrate lesion location and tissue damage in the CNS.

Bleranda Zeka, Maria Hastermann, Sonja Hochmeister, Nikolaus Kögl, Nathalie Kaufmann, Kathrin Schanda, Simone Mader, Tatsuro Misu, Paulus Rommer, Kazuo Fujihara, Zsolt Illes, Fritz Leutmezer, Douglas Kazutoshi Sato, Ichiro Nakashima, Markus Reindl, Hans Lassmann, Monika Bradl,

In neuromyelitis optica (NMO), astrocytes become targets for pathogenic aquaporin 4 (AQP4)-specific antibodies which gain access to the central nervous system (CNS) in the course of inflammatory processes. Since these antibodies belong to a T cell-dependent subgroup of immunoglobulins, and since NMO lesions contain activated CD4(+) T cells, the question ... Read more >>

Acta Neuropathol. (Acta neuropathologica)
[2015, 130(6):783-798]

Cited: 29 times

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Blocking stroke-induced immunodeficiency increases CNS antigen-specific autoreactivity but does not worsen functional outcome after experimental stroke.

Christine Römer, Odilo Engel, Katarzyna Winek, Sonja Hochmeister, Tian Zhang, Georg Royl, Juliane Klehmet, Ulrich Dirnagl, Christian Meisel, Andreas Meisel,

Stroke-induced immunodepression (SIDS) is an essential cause of poststroke infections. Pharmacological inhibition of SIDS appears promising in preventing life-threatening infections in stroke patients. However, SIDS might represent an adaptive mechanism preventing autoreactive immune responses after stroke. To address this, we used myelin oligodendrocyte glycoprotein (MOG) T-cell receptor transgenic (2D2) mice ... Read more >>

J. Neurosci. (The Journal of neuroscience : the official journal of the Society for Neuroscience)
[2015, 35(20):7777-7794]

Cited: 20 times

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Long-term implanted cOFM probe causes minimal tissue reaction in the brain.

Thomas Birngruber, Arijit Ghosh, Sonja Hochmeister, Martin Asslaber, Thomas Kroath, Thomas R Pieber, Frank Sinner,

This study investigated the histological tissue reaction to long-term implanted cerebral open flow microperfusion (cOFM) probes in the frontal lobe of the rat brain. Most probe-based cerebral fluid sampling techniques are limited in application time due to the formation of a glial scar that hinders substance exchange between brain tissue ... Read more >>

PLoS ONE (PloS one)
[2014, 9(3):e90221]

Cited: 3 times

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Antibody-mediated inhibition of TNFR1 attenuates disease in a mouse model of multiple sclerosis.

Sarah K Williams, Olaf Maier, Roman Fischer, Richard Fairless, Sonja Hochmeister, Aleksandar Stojic, Lara Pick, Doreen Haar, Sylvia Musiol, Maria K Storch, Klaus Pfizenmaier, Ricarda Diem,

Tumour necrosis factor (TNF) is a proinflammatory cytokine that is known to regulate inflammation in a number of autoimmune diseases, including multiple sclerosis (MS). Although targeting of TNF in models of MS has been successful, the pathological role of TNF in MS remains unclear due to clinical trials where the ... Read more >>

PLoS ONE (PloS one)
[2014, 9(2):e90117]

Cited: 20 times

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Maternal neurofascin-specific autoantibodies bind to structures of the fetal nervous system during pregnancy, but have no long term effect on development in the rat.

Sonja Hochmeister, Thomas Pekar, Maren Lindner, Maja Kitic, Michaela Haindl, Maria Storch, Franz Fazekas, Christopher Linington,

Neurofascin was recently reported as a target for axopathic autoantibodies in patients with multiple sclerosis (MS), a response that will exacerbate axonal pathology and disease severity in an animal model of multiple sclerosis. As transplacental transfer of maternal autoantibodies can permanently damage the developing nervous system we investigated whether intrauterine ... Read more >>

PLoS ONE (PloS one)
[2014, 9(1):e85393]

Cited: 1 time

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Efficacy of vitamin D in treating multiple sclerosis-like neuroinflammation depends on developmental stage.

Milena Z Adzemovic, Manuel Zeitelhofer, Sonja Hochmeister, Sven A Gustafsson, Maja Jagodic,

The association of vitamin D deficiency with higher prevalence, relapse rate and progression of multiple sclerosis (MS) has stimulated great interest in using vitamin D supplementation as a preventative measure and even a therapy for established MS. However, there is a considerable lack of evidence when it comes to an ... Read more >>

Exp. Neurol. (Experimental neurology)
[2013, 249:39-48]

Cited: 17 times

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Neurofascin 186 specific autoantibodies induce axonal injury and exacerbate disease severity in experimental autoimmune encephalomyelitis.

Maren Lindner, Judy King Man Ng, Sonja Hochmeister, Edgar Meinl, Christopher Linington,

Axonal injury is considered the major cause of chronic disability in multiple sclerosis (MS) patients, however the mechanisms behind remain still unclear. Recently, it was demonstrated that autoantibodies against Neurofascin, a cell adhesion molecule within the adult nervous system, can contribute to the development of axonal pathology in some patients. ... Read more >>

Exp. Neurol. (Experimental neurology)
[2013, 247:259-266]

Cited: 15 times

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Intrastriatal injection of interleukin-1 beta triggers the formation of neuromyelitis optica-like lesions in NMO-IgG seropositive rats.

Maja Kitic, Sonja Hochmeister, Isabella Wimmer, Jan Bauer, Tatsuro Misu, Simone Mader, Markus Reindl, Kazuo Fujihara, Hans Lassmann, Monika Bradl,

Neuromyelitis optica (NMO) is a severe, disabling disease of the central nervous system (CNS) characterized by the formation of astrocyte-destructive, neutrophil-dominated inflammatory lesions in the spinal cord and optic nerves. These lesions are initiated by the binding of pathogenic aquaporin 4 (AQP4)-specific autoantibodies to astrocytes and subsequent complement-mediated lysis of ... Read more >>

Acta Neuropathol Commun (Acta neuropathologica communications)
[2013, 1:5]

Cited: 29 times

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Preclinical retinal neurodegeneration in a model of multiple sclerosis: The Journal of Neuroscience 2012; 32(16):5585-5597.

Richard Fairless, Sarah K Williams, Dorit B Hoffmann, Aleksandar Stojic, Sonja Hochmeister, Frank Schmitz, Maria K Storch, Ricarda Diem,

Ann Neurosci (Annals of neurosciences)
[2012, 19(3):121-122]

Cited: 1 time

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Expression of Ccl11 associates with immune response modulation and protection against neuroinflammation in rats.

Milena Z Adzemovic, Johan Öckinger, Manuel Zeitelhofer, Sonja Hochmeister, Amennai Daniel Beyeen, Atul Paulson, Alan Gillett, Melanie Thessen Hedreul, Ruxandra Covacu, Hans Lassmann, Tomas Olsson, Maja Jagodic,

Multiple sclerosis (MS) is a polygenic disease characterized by inflammation and demyelination in the central nervous system (CNS), which can be modeled in experimental autoimmune encephalomyelitis (EAE). The Eae18b locus on rat chromosome 10 has previously been linked to regulation of beta-chemokine expression and severity of EAE. Moreover, the homologous ... Read more >>

PLoS ONE (PloS one)
[2012, 7(7):e39794]

Cited: 23 times

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Preclinical retinal neurodegeneration in a model of multiple sclerosis.

Richard Fairless, Sarah K Williams, Dorit B Hoffmann, Aleksandar Stojic, Sonja Hochmeister, Frank Schmitz, Maria K Storch, Ricarda Diem,

Neurodegeneration plays a major role in multiple sclerosis (MS), in which it is thought to be the main determinant of permanent disability. However, the relationship between the immune response and the onset of neurodegeneration is still a matter of debate. Moreover, recent findings in MS patients raised the question of ... Read more >>

J. Neurosci. (The Journal of neuroscience : the official journal of the Society for Neuroscience)
[2012, 32(16):5585-5597]

Cited: 32 times

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Role of n-type voltage-dependent calcium channels in autoimmune optic neuritis.

Ivana Gadjanski, Susann Boretius, Sarah K Williams, Paul Lingor, Johanna Knöferle, Muriel B Sättler, Richard Fairless, Sonja Hochmeister, Kurt-Wolfram Sühs, Thomas Michaelis, Jens Frahm, Maria K Storch, Mathias Bähr, Ricarda Diem,

OBJECTIVE: The aim of this study was to investigate the role of voltage-dependent calcium channels (VDCCs) in axon degeneration during autoimmune optic neuritis. METHODS: Calcium ion (Ca(2+)) influx into the optic nerve (ON) through VDCCs was investigated in a rat model of optic neuritis using manganese-enhanced magnetic resonance imaging and ... Read more >>

Ann. Neurol. (Annals of neurology)
[2009, 66(1):81-93]

Cited: 23 times

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The "window of susceptibility" for inflammation in the immature central nervous system is characterized by a leaky blood-brain barrier and the local expression of inflammatory chemokines.

Lucia Schoderboeck, Milena Adzemovic, Eva-Maria Nicolussi, Claudia Crupinschi, Sonja Hochmeister, Marie-Therese Fischer, Hans Lassmann, Monika Bradl,

Early in postnatal development, the immature central nervous system (CNS) is more susceptible to inflammation than its adult counterpart. We show here that this "window of susceptibility" is characterized by the presence of leaky vessels in the CNS, and by a global chemokine expression profile which is clearly distinct from ... Read more >>

Neurobiol. Dis. (Neurobiology of disease)
[2009, 35(3):368-375]

Cited: 11 times

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The susceptibility to experimental autoimmune encephalomyelitis is not related to dysferlin-deficiency.

Sonja Hochmeister, Reginald E Bittner, Harald Höger, Hans Lassmann, Monika Bradl,

Recent observations suggested that dysferlin might play a role in the development of autoimmune central nervous system (CNS) inflammation. To address this issue, we studied the induction and effector phase of experimental autoimmune encephalomyelitis in C57BL/10 mice producing intact or functionally deficient dysferlin. We found that both types of mice ... Read more >>

Autoimmunity (Autoimmunity)
[2009, 42(3):235-241]

Cited: 1 time

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After injection into the striatum, in vitro-differentiated microglia- and bone marrow-derived dendritic cells can leave the central nervous system via the blood stream.

Sonja Hochmeister, Manuel Zeitelhofer, Jan Bauer, Eva-Maria Nicolussi, Marie-Therese Fischer, Bernhard Heinke, Edgar Selzer, Hans Lassmann, Monika Bradl,

The prototypic migratory trail of tissue-resident dendritic cells (DCs) is via lymphatic drainage. Since the central nervous system (CNS) lacks classical lymphatic vessels, and antigens and cells injected into both the CNS and cerebrospinal fluid have been found in deep cervical lymph nodes, it was thought that CNS-derived DCs exclusively ... Read more >>

Am. J. Pathol. (The American journal of pathology)
[2008, 173(6):1669-1681]

Cited: 26 times

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An X-linked myopathy with postural muscle atrophy and generalized hypertrophy, termed XMPMA, is caused by mutations in FHL1.

Christian Windpassinger, Benedikt Schoser, Volker Straub, Sonja Hochmeister, Abdul Noor, Birgit Lohberger, Natalie Farra, Erwin Petek, Thomas Schwarzbraun, Lisa Ofner, Wolfgang N Löscher, Klaus Wagner, Hanns Lochmüller, John B Vincent, Stefan Quasthoff,

We have identified a large multigenerational Austrian family displaying a novel form of X-linked recessive myopathy. Affected individuals develop an adult-onset scapulo-axio-peroneal myopathy with bent-spine syndrome characterized by specific atrophy of postural muscles along with pseudoathleticism or hypertrophy and cardiac involvement. Known X-linked myopathies were excluded by simple-tandem-repeat polymorphism (STRP) ... Read more >>

Am. J. Hum. Genet. (American journal of human genetics)
[2008, 82(1):88-99]

Cited: 88 times

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Mutation in the Scyl1 gene encoding amino-terminal kinase-like protein causes a recessive form of spinocerebellar neurodegeneration.

Wolfgang M Schmidt, Cornelia Kraus, Harald Höger, Sonja Hochmeister, Felicitas Oberndorfer, Manuela Branka, Sonja Bingemann, Hans Lassmann, Markus Müller, Lúcia Inês Macedo-Souza, Mariz Vainzof, Mayana Zatz, André Reis, Reginald E Bittner,

Here, we show that the murine neurodegenerative disease mdf (autosomal recessive mouse mutant 'muscle deficient') is caused by a loss-of-function mutation in Scyl1, disrupting the expression of N-terminal kinase-like protein, an evolutionarily conserved putative component of the nucleocytoplasmic transport machinery. Scyl1 is prominently expressed in neurons, and enriched at central ... Read more >>

EMBO Rep. (EMBO reports)
[2007, 8(7):691-697]

Cited: 33 times

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Dysferlin is a new marker for leaky brain blood vessels in multiple sclerosis.

Sonja Hochmeister, Roland Grundtner, Jan Bauer, Britta Engelhardt, Ruth Lyck, Grace Gordon, Thomas Korosec, Alexandra Kutzelnigg, Johannes J Berger, Monika Bradl, Reginald E Bittner, Hans Lassmann,

Dysferlin is a muscle protein involved in cell membrane repair and its deficiency is associated with muscular dystrophy. We describe that dysferlin is also expressed in leaky endothelial cells. In the normal central nervous system (CNS), dysferlin is only present in endothelial cells of circumventricular organs. In the inflamed CNS ... Read more >>

J. Neuropathol. Exp. Neurol. (Journal of neuropathology and experimental neurology)
[2006, 65(9):855-865]

Cited: 79 times

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