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Andreas Weishaupt - One of the best experts on this subject based on the ideXlab platform.
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contactin 1 and neurofascin 155 186 are not targets of auto antibodies in multifocal Motor Neuropathy
PLOS ONE, 2015Co-Authors: Kathrin Doppler, Judy King Man Ng, Luise Appeltshauser, Heidrun H Kramer, Edgar Meinl, Sulayman D Dibhajj, Stephen G Waxman, Peter J. Brophy, Carmen Villmann, Andreas WeishauptAbstract:Multifocal Motor Neuropathy is an immune mediated disease presenting with multifocal muscle weakness and conduction block. IgM auto-antibodies against the ganglioside GM1 are detectable in about 50% of the patients. Auto-antibodies against the paranodal proteins contactin-1 and neurofascin-155 and the nodal protein neurofascin-186 have been detected in subgroups of patients with chronic inflammatory demyelinating polyNeuropathy. Recently, auto-antibodies against neurofascin-186 and gliomedin were described in more than 60% of patients with multifocal Motor Neuropathy. In the current study, we aimed to validate this finding, using a combination of different assays for auto-antibody detection. In addition we intended to detect further auto-antibodies against paranodal proteins, specifically contactin-1 and neurofascin-155 in multifocal Motor Neuropathy patients’ sera. We analyzed sera of 33 patients with well-characterized multifocal Motor Neuropathy for IgM or IgG anti-contactin-1, anti-neurofascin-155 or -186 antibodies using enzyme-linked immunosorbent assay, binding assays with transfected human embryonic kidney 293 cells and murine teased fibers. We did not detect any IgM or IgG auto-antibodies against contactin-1, neurofascin-155 or -186 in any of our multifocal Motor Neuropathy patients. We conclude that auto-antibodies against contactin-1, neurofascin-155 and -186 do not play a relevant role in the pathogenesis in this cohort with multifocal Motor Neuropathy.
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Contactin-1 and Neurofascin-155/-186 Are Not Targets of Auto-Antibodies in Multifocal Motor Neuropathy.
PloS one, 2015Co-Authors: Kathrin Doppler, Judy King Man Ng, Luise Appeltshauser, Heidrun H Kramer, Edgar Meinl, Sulayman D Dib-hajj, Stephen G Waxman, Peter J. Brophy, Carmen Villmann, Andreas WeishauptAbstract:Multifocal Motor Neuropathy is an immune mediated disease presenting with multifocal muscle weakness and conduction block. IgM auto-antibodies against the ganglioside GM1 are detectable in about 50% of the patients. Auto-antibodies against the paranodal proteins contactin-1 and neurofascin-155 and the nodal protein neurofascin-186 have been detected in subgroups of patients with chronic inflammatory demyelinating polyNeuropathy. Recently, auto-antibodies against neurofascin-186 and gliomedin were described in more than 60% of patients with multifocal Motor Neuropathy. In the current study, we aimed to validate this finding, using a combination of different assays for auto-antibody detection. In addition we intended to detect further auto-antibodies against paranodal proteins, specifically contactin-1 and neurofascin-155 in multifocal Motor Neuropathy patients' sera. We analyzed sera of 33 patients with well-characterized multifocal Motor Neuropathy for IgM or IgG anti-contactin-1, anti-neurofascin-155 or -186 antibodies using enzyme-linked immunosorbent assay, binding assays with transfected human embryonic kidney 293 cells and murine teased fibers. We did not detect any IgM or IgG auto-antibodies against contactin-1, neurofascin-155 or -186 in any of our multifocal Motor Neuropathy patients. We conclude that auto-antibodies against contactin-1, neurofascin-155 and -186 do not play a relevant role in the pathogenesis in this cohort with multifocal Motor Neuropathy.
Miriam Hwang - One of the best experts on this subject based on the ideXlab platform.
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Pure trigeminal Motor Neuropathy: a case report.
Archives of physical medicine and rehabilitation, 2000Co-Authors: Yoon-kyoo Kang, Eun-ha Lee, Miriam HwangAbstract:Pure trigeminal Motor Neuropathy is a trigeminal Motor paralysis unaccompanied by signs of trigeminal sensory or other cranial nerve involvement. We present a case of a 38-year-old man complaining of progressive weakness and wasting of the right masticatory muscles after a bout of mild cold symptoms. No sensory symptoms were present. History, neurologic examination, electrophysiologic studies, and magnetic resonance imaging of the brain led to the diagnosis of pure trigeminal Motor Neuropathy. The cause may have been an autoimmune reaction to a viral infection.
Leonard H. Van Den Berg - One of the best experts on this subject based on the ideXlab platform.
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multifocal Motor Neuropathy controversies and priorities
Journal of Neurology Neurosurgery and Psychiatry, 2020Co-Authors: James P B Dyck, Bruce V. Taylor, Matthew C Kiernan, Leonard H. Van Den BergAbstract:Despite 30 years of research there are still significant unknowns and controversies associated with multifocal Motor Neuropathy (MMN) including disease pathophysiology, diagnostic criteria and treatment. Foremost relates to the underlying pathophysiology, specifically whether MMN represents an axonal or demyelinating Neuropathy and whether the underlying pathophysiology is focused at the node of Ranvier. In turn, this discussion promotes consideration of therapeutic approaches, an issue that becomes more directed in this evolving era of precision medicine. It is generally accepted that MMN represents a chronic progressive immune-mediated Motor Neuropathy clinically characterised by progressive asymmetric weakness and electrophysiologically by partial Motor conduction block. Anti-GM1 IgM antibodies are identified in at least 40% of patients. There have been recent developments in the use of neuromuscular ultrasound and MRI to aid in diagnosing MMN and in further elucidation of its pathophysiological mechanisms. The present Review will critically analyse the knowledge accumulated about MMN over the past 30 years, culminating in a state-of-the-art approach to therapy.
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multifocal Motor Neuropathy diagnosis pathogenesis and treatment strategies
Nature Reviews Neurology, 2012Co-Authors: Lotte Vlam, Elisabeth A Cats, Dirk C G Straver, Sanneke Piepers, Hessel Franssen, Leonard H. Van Den BergAbstract:Multifocal Motor Neuropathy (MMN) is a rare inflammatory disorder that features slowly progressive, asymmetric distal limb weakness. Leonard van den Berg and colleagues describe the diagnostic criteria for MMN, which are important for distinguishing this disease from mimic disorders such as amyotrophic lateral sclerosis. The authors also discuss the pathophysiology of MMN, emphasizing the role of GM1-specific autoantibodies, and outline current treatment options and possible new therapeutic strategies.
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intravenous immunoglobulin treatment in multifocal Motor Neuropathy
Journal of Clinical Immunology, 2010Co-Authors: Elisabeth A Cats, Leonard H. Van Den BergAbstract:Introduction Multifocal Motor Neuropathy (MMN) is characterized by asymmetric weakness of limbs and the electrophysiological finding of conduction block in Motor nerves. Conduction block is the inability of nerves to propagate action potentials and is probably caused by immune-mediated dysfunction of the axon at the nodes of Ranvier or the myelin sheath. MMN immune pathogenesis has not been elucidated.
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multifocal Motor Neuropathy
Lancet Neurology, 2005Co-Authors: Janthies H Van Asseldonk, Renske M Van Den Bergvos, John H. J. Wokke, Hessel Franssen, Leonard H. Van Den BergAbstract:Summary Multifocal Motor Neuropathy (MMN) is an immune-mediated disorder characterised by slowly progressive, asymmetrical weakness of limbs without sensory loss. The clinical presentation of MMN mimics that of lower-Motor-neuron disease, but in nerve-conduction studies of patients with MMN Motor-conduction block has been found. By contrast with chronic inflammatory demyelinating polyNeuropathy, treatment with prednisolone and plasma exchange is generally ineffective in MMN and even associated with clinical worsening in some patients. Of the immunosuppressants, cyclophosphamide has been reported as effective but only anecdotally. Various open trials and four placebo-controlled trials have shown that treatment with high-dose intravenous immunoglobulin leads to improvement of muscle strength in patients with MMN. Although clinical, pathological, imaging, immunological, and electrophysiological studies have improved our understanding of MMN over the past 15 years, further research is needed to elucidate pathogenetic disease mechanisms in the disorder.
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intravenous immunoglobulin for multifocal Motor Neuropathy
Cochrane Database of Systematic Reviews, 2005Co-Authors: Ivo N. Van Schaik, Leonard H. Van Den Berg, Rob J. De Haan, M VermeulenAbstract:Background Multifocal Motor Neuropathy is a rare, probably immune mediated disorder characterised by slowly progressive, asymmetric, distal weakness of one or more limbs with no objective loss of sensation. It may cause prolonged periods of disability. The treatment options for multifocal Motor Neuropathy are sparse. Patients with multifocal Motor Neuropathy do not usually respond to steroids or plasma exchange, and may even worsen with these treatments. Many uncontrolled studies have suggested a beneficial effect of intravenous immunoglobulin. Objectives To review systematically the evidence from randomised controlled trials concerning the efficacy and safety of intravenous immunoglobulin in multifocal Motor Neuropathy. Search methods We used the search strategy of the Cochrane Neuromuscular Disease Review Group to search the Disease Group register (searched March 2007), MEDLINE (January 1990 to March 2007), EMBASE (January 1990 to March 2007) and ISI (January 1990 to March 2007) databases for randomised controlled trials. Selection criteria Randomised controlled studies examining the effects of any dose of intravenous immunoglobulin versus placebo in patients with definite or probable multifocal Motor Neuropathy. Outcome measures had to include one of the following: disability, strength, or conduction block. Studies which reported the frequency of adverse effects were used to assess safety. Data collection and analysis Two authors reviewed literature searches to identify potentially relevant trials, scored their quality and extracted data independently. For dichotomous data, we calculated relative risks, and for continuous data, effect sizes and weighted pooled effect sizes. Statistical uncertainty was expressed with 95% confidence intervals. Main results Four randomised controlled trials including a total of 34 patients were suitable for this systematic review. Strength improved in 78% of patients treated with intravenous immunoglobulin and only 4% of placebo-treated patients. Disability improved in 39% of patients after intravenous immunoglobulin treatment and in 11% after placebo (statistically not significantly different). Mild, transient side effects were reported in 71% of intravenous immunoglobulin treated patients. Serious side effects were not encountered. Authors' conclusions Limited evidence from randomised controlled trials shows that intravenous immunoglobulin has a beneficial effect on strength. There was a non-significant trend towards improvement in disability. More research is needed to discover whether intravenous immunoglobulin improves disability and is cost-effective.
Kathrin Doppler - One of the best experts on this subject based on the ideXlab platform.
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contactin 1 and neurofascin 155 186 are not targets of auto antibodies in multifocal Motor Neuropathy
PLOS ONE, 2015Co-Authors: Kathrin Doppler, Judy King Man Ng, Luise Appeltshauser, Heidrun H Kramer, Edgar Meinl, Sulayman D Dibhajj, Stephen G Waxman, Peter J. Brophy, Carmen Villmann, Andreas WeishauptAbstract:Multifocal Motor Neuropathy is an immune mediated disease presenting with multifocal muscle weakness and conduction block. IgM auto-antibodies against the ganglioside GM1 are detectable in about 50% of the patients. Auto-antibodies against the paranodal proteins contactin-1 and neurofascin-155 and the nodal protein neurofascin-186 have been detected in subgroups of patients with chronic inflammatory demyelinating polyNeuropathy. Recently, auto-antibodies against neurofascin-186 and gliomedin were described in more than 60% of patients with multifocal Motor Neuropathy. In the current study, we aimed to validate this finding, using a combination of different assays for auto-antibody detection. In addition we intended to detect further auto-antibodies against paranodal proteins, specifically contactin-1 and neurofascin-155 in multifocal Motor Neuropathy patients’ sera. We analyzed sera of 33 patients with well-characterized multifocal Motor Neuropathy for IgM or IgG anti-contactin-1, anti-neurofascin-155 or -186 antibodies using enzyme-linked immunosorbent assay, binding assays with transfected human embryonic kidney 293 cells and murine teased fibers. We did not detect any IgM or IgG auto-antibodies against contactin-1, neurofascin-155 or -186 in any of our multifocal Motor Neuropathy patients. We conclude that auto-antibodies against contactin-1, neurofascin-155 and -186 do not play a relevant role in the pathogenesis in this cohort with multifocal Motor Neuropathy.
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Contactin-1 and Neurofascin-155/-186 Are Not Targets of Auto-Antibodies in Multifocal Motor Neuropathy.
PloS one, 2015Co-Authors: Kathrin Doppler, Judy King Man Ng, Luise Appeltshauser, Heidrun H Kramer, Edgar Meinl, Sulayman D Dib-hajj, Stephen G Waxman, Peter J. Brophy, Carmen Villmann, Andreas WeishauptAbstract:Multifocal Motor Neuropathy is an immune mediated disease presenting with multifocal muscle weakness and conduction block. IgM auto-antibodies against the ganglioside GM1 are detectable in about 50% of the patients. Auto-antibodies against the paranodal proteins contactin-1 and neurofascin-155 and the nodal protein neurofascin-186 have been detected in subgroups of patients with chronic inflammatory demyelinating polyNeuropathy. Recently, auto-antibodies against neurofascin-186 and gliomedin were described in more than 60% of patients with multifocal Motor Neuropathy. In the current study, we aimed to validate this finding, using a combination of different assays for auto-antibody detection. In addition we intended to detect further auto-antibodies against paranodal proteins, specifically contactin-1 and neurofascin-155 in multifocal Motor Neuropathy patients' sera. We analyzed sera of 33 patients with well-characterized multifocal Motor Neuropathy for IgM or IgG anti-contactin-1, anti-neurofascin-155 or -186 antibodies using enzyme-linked immunosorbent assay, binding assays with transfected human embryonic kidney 293 cells and murine teased fibers. We did not detect any IgM or IgG auto-antibodies against contactin-1, neurofascin-155 or -186 in any of our multifocal Motor Neuropathy patients. We conclude that auto-antibodies against contactin-1, neurofascin-155 and -186 do not play a relevant role in the pathogenesis in this cohort with multifocal Motor Neuropathy.
Arthur P Hays - One of the best experts on this subject based on the ideXlab platform.
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Motor nerve biopsy studies in Motor Neuropathy and Motor neuron disease
Muscle & Nerve, 1997Co-Authors: Massimo Corbo, M. Kamel Abouzahr, Angelo Quattrini, Nicola Canal, Sandro Iannaccone, Raffaello Nemni, Norman Latov, Arthur P HaysAbstract:The clinical presentation of Motor Neuropathy often resembles that of Motor neuron disease, sometimes leading to an erroneous diagnosis. Moreover, the underlying pathological process in Motor Neuropathy has been rarely investigated and there are no systematic studies of the affected Motor nerves. We describe a new Motor nerve biopsy procedure, performed in 15 patients: 6 with Motor Neuropathy and 9 with Motor neuron disease. The Motor branch from the anterior division of the obturator nerve to the gracilis muscle in the thigh was biopsied. In both groups of patients the Motor nerves exhibited depletion of myelinated nerve fibers. In Motor Neuropathy there was a significantly higher density of regenerative clusters of small myelinated fibers in comparison to Motor nerves from patients with Motor neuron disease. In addition, in 3 patients with Motor Neuropathy there was evidence for demyelination with thinly myelinated axons and small onion bulb formations. These pathological studies of Motor nerve biopsies can help to differentiate Motor Neuropathy from Motor neuron disease. © 1997 John Wiley & Sons, Inc.
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Motor nerve biopsy studies in Motor Neuropathy and Motor neuron disease
Muscle & nerve, 1997Co-Authors: Massimo Corbo, M. Kamel Abouzahr, Angelo Quattrini, Nicola Canal, Sandro Iannaccone, Raffaello Nemni, Norman Latov, Arthur P HaysAbstract:The clinical presentation of Motor Neuropathy often resembles that of Motor neuron disease, sometimes leading to an erroneous diagnosis. Moreover, the underlying pathological process in Motor Neuropathy has been rarely investigated and there are no systematic studies of the affected Motor nerves. We describe a new Motor nerve biopsy procedure, performed in 15 patients: 6 with Motor Neuropathy and 9 with Motor neuron disease. The Motor branch from the anterior division of the obturator nerve to the gracilis muscle in the thigh was biopsied. In both groups of patients the Motor nerves exhibited depletion of myelinated nerve fibers. In Motor Neuropathy there was a significantly higher density of regenerative clusters of small myelinated fibers in comparison to Motor nerves from patients with Motor neuron disease. In addition, in 3 patients with Motor Neuropathy there was evidence for demyelination with thinly myelinated axons and small onion bulb formations. These pathological studies of Motor nerve biopsies can help to differentiate Motor Neuropathy from Motor neuron disease.