The Experts below are selected from a list of 7071 Experts worldwide ranked by ideXlab platform
Hanspeter Hartung - One of the best experts on this subject based on the ideXlab platform.
-
advances in understanding and treatment of immune mediated disorders of the peripheral nervous system
2004Co-Authors: Bernd C Kieseier, Hugh J. Willison, Reinhard Kiefer, Ralf Gold, Bernhard Hemmer, Hanspeter HartungAbstract:During recent years, novel insights in basic immunology and advances in biotechnology have contributed to an increased understanding of the pathogenetic mechanisms of Immune-Mediated disorders of the peripheral nervous system. This increased knowledge has an impact on the management of patients with this class of disorders. Current advances are outlined and their implication for therapeutic approaches addressed. As a prototypic Immune-Mediated Neuropathy, special emphasis is placed on the pathogenesis and treatment of the Guillain-Barre syndrome and its variants. Moreover, neuropathies of the chronic inflammatory demyelinating, multifocal motor, and nonsystemic vasculitic types are discussed. This review summarizes recent progress with currently available therapies and--on the basis of present immunopathogenetic concepts--outlines future treatment strategies.
-
immunopathogenesis and treatment of the guillain barre syndrome part i
1995Co-Authors: Hanspeter Hartung, Klaus V Toyka, J D Pollard, Graham K HarveyAbstract:In the second part of our review the role of antecedent infections in the pathogenesis of GBS is discussed. The association with Campylobacter jejuni (C. jejuni) is highlighted and the concept of molecular mimicry, i.e., sharing of epitopes between microbes and peripheral nerve, explained. Alternative mechanisms to relate an infection with the Immune-Mediated Neuropathy are elaborated. Current therapies of the GBS include plasma exchange, high-dose intravenous immunoglobulins, and supportive treatment directed to secondary complications. Published therapeutic trials are reviewed and future approaches are outlined. Principles of general care are also summarized.© 1995 John Wiley &Sons, Inc.
Hugh J. Willison - One of the best experts on this subject based on the ideXlab platform.
-
The Effects of Age and Ganglioside Composition on the Rate of Motor Nerve Terminal Regeneration Following Antibody-Mediated Injury in Mice
2016Co-Authors: Angie Rupp, Koichi Furukawa, Madeleine E. Cunningham, Denggao Yao, Hugh J. WillisonAbstract:KEY WORDS antibody; complement; ganglioside; neuromuscular junction; Neuropathy ABSTRACT Gangliosides are glycosphingolipids highly enriched in neural plasma membranes, where they mediate a diverse range of functions and can act as targets for auto-antibodies present in human Immune-Mediated Neuropathy sera. The ensuing autoimmune injury results in axonal and motor nerve terminal (mNT) degeneration. Both aging and ganglioside-deficiency have been linked to impaired axonal regenera-tion. To assess the effects of age and ganglioside expression on mNT regeneration in an autoimmune injury paradigm, anti-ganglioside antibodies and complement were applied to young adult and aged mice wildtype (WT) mice, mice deficient in either b-and c-series (GD3sKO) or mice deficient in all complex gangliosides (GM2sKO). The extent of mNT injury and regeneration was assessed immediately or after 5 days, respectively. Depending on ganglioside expression and antibody-specificity, either a selective mNT injury or a combined injury of mNTs and neuromuscular glial cells was elicited. Immediately after induction of the injury, between 1.5 % and 11.8 % of neuro
-
advances in understanding and treatment of immune mediated disorders of the peripheral nervous system
2004Co-Authors: Bernd C Kieseier, Hugh J. Willison, Reinhard Kiefer, Ralf Gold, Bernhard Hemmer, Hanspeter HartungAbstract:During recent years, novel insights in basic immunology and advances in biotechnology have contributed to an increased understanding of the pathogenetic mechanisms of Immune-Mediated disorders of the peripheral nervous system. This increased knowledge has an impact on the management of patients with this class of disorders. Current advances are outlined and their implication for therapeutic approaches addressed. As a prototypic Immune-Mediated Neuropathy, special emphasis is placed on the pathogenesis and treatment of the Guillain-Barre syndrome and its variants. Moreover, neuropathies of the chronic inflammatory demyelinating, multifocal motor, and nonsystemic vasculitic types are discussed. This review summarizes recent progress with currently available therapies and--on the basis of present immunopathogenetic concepts--outlines future treatment strategies.
Con Yiannikas - One of the best experts on this subject based on the ideXlab platform.
-
multifocal motor Neuropathy presenting as pseudodystonia
2017Co-Authors: Nidhi Garg, Robert Heard, L Kiers, Richard P. Gerraty, Con YiannikasAbstract:: Multifocal motor Neuropathy (MMN) is an Immune-Mediated Neuropathy. Wasting and weakness typically dominate the clinical presentation. We describe four cases presenting with prominent cramping resembling a primary movement disorder. All cases had features of focal motor conduction block on neurophysiological studies. The involuntary movements resolved in all four patients following treatment with intravenous immunoglobulin. The presented cases highlight an unusual presentation of MMN and emphasize that peripheral nerve pathology can present with movement disorders mimicking central nervous system disease. Furthermore, the movement disorder appears particularly sensitive to standard therapy.
Christoph Kleinschnitz - One of the best experts on this subject based on the ideXlab platform.
-
multifocal motor Neuropathy update on clinical characteristics pathophysiological concepts and therapeutic options
2010Co-Authors: Sven G. Meuth, Christoph KleinschnitzAbstract:Multifocal motor Neuropathy (MMN) is an acquired Immune-Mediated Neuropathy characterized by chronic or stepwise progressive asymmetrical limb weakness without sensory deficits. The upper extremities
-
multifocal motor Neuropathy update on clinical characteristics pathophysiological concepts and therapeutic options
2010Co-Authors: Sven G. Meuth, Christoph KleinschnitzAbstract:Multifocal motor Neuropathy (MMN) is an acquired Immune-Mediated Neuropathy characterized by chronic or stepwise progressive asymmetrical limb weakness without sensory deficits. The upper extremities are more often affected than the lower extremities with distal paresis dominating over proximal paresis. Important diagnostic features are persistent multifocal partial conduction blocks (CBs) and the presence of high-titer anti-GM1 serum antibodies. Motor neuron disease, other chronic dysimmune neuropathies, such as chronic inflammatory demyelinating polyNeuropathy and the Lewis-Sumner syndrome (MADSAM Neuropathy), are important differential diagnoses. While corticosteroids and plasma exchange are largely ineffective, high-dose intravenous immunoglobulins are regarded as first-line treatment. In spite of significant success in elucidating the underlying disease mechanisms in MMN during the past few years, important pathophysiological issues and the optimum long-term therapy remain to be clarified. The present review summarizes the clinical picture and current pathophysiological concepts of MMN with a special focus on the molecular and electrophysiological basis of CBs and highlights established therapies as well as possible novel treatment options.
Pieter A Van Doorn - One of the best experts on this subject based on the ideXlab platform.
-
progress in diagnosis and treatment of chronic inflammatory demyelinating polyradiculoNeuropathy
2019Co-Authors: Carina Bunschoten, Bart C Jacobs, Peter Van Den Bergh, David R Cornblath, Pieter A Van DoornAbstract:Chronic inflammatory demyelinating polyradiculoNeuropathy (CIDP) is a rare and heterogeneous but treatable Immune-Mediated Neuropathy. Nerve conduction studies are considered essential for a definite diagnosis, but poor performance and misinterpretation of the results frequently leads to misdiagnosis. Nerve ultrasound and MRI could be helpful in diagnosis. Whereas typical CIDP is relatively easy to diagnose, atypical variants with distinct phenotypes can be a diagnostic challenge. Intravenous or subcutaneous immunoglobulin, corticosteroids, and plasma exchange are effective treatments, but maintenance treatments are often required for years, and treatment regimens require careful and regular adjustments to avoid undertreatment or overtreatment. Patients who do not improve, or insufficiently improve after treatment, might have specific characteristics related to a distinct disease mechanism caused by immunoglobulin G4 antibodies to nodal or paranodal proteins, and could require alternative treatments. Future studies should focus on curative and individualised treatment regimens to improve the patient's condition and to prevent further nerve damage.
