The Experts below are selected from a list of 5931 Experts worldwide ranked by ideXlab platform
Richard A. C. Hughes - One of the best experts on this subject based on the ideXlab platform.
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European Handbook of Neurological Management - Chronic Inflammatory Demyelinating Polyradiculoneuropathy
European Handbook of Neurological Management, 2010Co-Authors: Richard A. C. HughesAbstract:Background Chronic inflammatory demyelinating Polyradiculoneuropathy is a chronic progressive or relapsing and remitting disease with a prevalence of up to 8.9 per 100,000.
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Chronic Inflammatory Demyelinating Polyradiculoneuropathy.
Journal of Clinical Immunology, 2010Co-Authors: Richard A. C. HughesAbstract:Background Chronic inflammatory demyelinating Polyradiculoneuropathy is a chronic progressive or relapsing and remitting disease with a prevalence of up to 8.9 per 100,000.
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Pathogenesis of chronic inflammatory demyelinating Polyradiculoneuropathy
Journal of the peripheral nervous system : JPNS, 2006Co-Authors: Richard A. C. Hughes, David Allen, Anna Makowska, Norman A. GregsonAbstract:The acute lesions of chronic inflammatory demyelinating Polyradiculoneuropathy (CIDP) consist of endoneurial foci of chemokine and chemokine receptor expression and T cell and macrophage activation. The myelin protein antigens, P2, P0, and PMP22, each induce experimental autoimmune neuritis in rodent models and might be autoantigens in CIDP. The strongest evidence incriminates P0, to which antibodies have been found in 20% of cases. Failure of regulatory T-cell mechanism is thought to underlie persistent or recurrent disease, differentiating CIDP from the acute inflammatory demyelinating Polyradiculoneuropathy form of Guillain-Barre syndrome. Corticosteroids, intravenous immunoglobulin and plasma exchange each provide short term benefit but the possible long-term benefits of immunosuppressive drugs have yet to be confirmed in randomised, controlled trials.
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Cytotoxic drugs and interferons for chronic inflammatory demyelinating Polyradiculoneuropathy.
The Cochrane database of systematic reviews, 2004Co-Authors: Richard A. C. Hughes, A V Swan, P A Van DoornAbstract:Chronic inflammatory demyelinating Polyradiculoneuropathy is a disease causing progressive or relapsing and remitting weakness and numbness. It is probably due to an autoimmune inflammatory process. Immunosuppressive or immunomodulatory drugs would be expected to be beneficial. We aimed to review systematically the evidence from randomised trials concerning cytotoxic drugs and interferons for chronic inflammatory demyelinating Polyradiculoneuropathy. We searched the Cochrane Neuromuscular Disease Group trials register (searched May 2004), MEDLINE (searched January 1977 to May 2004), EMBASE (January 1980 to May 2004), CINAHL (searched January 1982 to December 2001) and LILACS (searched January 1982 to December 2001). We contacted the authors of the trials identified and other disease experts seeking other published and unpublished trials. We sought randomised and quasi-randomised trials of all immunosuppressive agents such as azathioprine, cyclophosphamide, methotrexate, cyclosporin A, mycophenolate mofetil, and rituximab and all immunomodulatory agents such as alpha interferon and beta interferon in participants fulfilling standard diagnostic criteria for chronic inflammatory demyelinating Polyradiculoneuropathy. Two of us independently selected the trials which met our criteria, judged their methodological quality and extracted the data onto specially designed forms. We wanted to measure the change in disability after one year as our primary outcome measure. We found one parallel group open trial of azathioprine for nine months involving 27 participants and another of interferon beta involving 10 participants in a double blind crossover trial with each treatment period lasting 12 weeks. Neither trial provided our primary outcome measure and neither showed a significant beneficial effect on any of the outcome measures selected by the authors or ourselves in the protocol for this review. The evidence is inadequate to decide whether azathioprine, interferon beta or any other immunosuppressive drug or interferon is beneficial in chronic inflammatory demyelinating Polyradiculoneuropathy.
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The Cochrane Library - Cytotoxic drugs and interferons for chronic inflammatory demyelinating Polyradiculoneuropathy
The Cochrane database of systematic reviews, 2003Co-Authors: Richard A. C. Hughes, A V Swan, P A Van DoornAbstract:BACKGROUND Chronic inflammatory demyelinating Polyradiculoneuropathy is a disease causing progressive or relapsing and remitting weakness and numbness. It is probably due to an autoimmune inflammatory process. Immunosuppressive or immunomodulatory drugs would be expected to be beneficial. OBJECTIVES We aimed to review systematically the evidence from randomised trials concerning cytotoxic drugs and interferons for chronic inflammatory demyelinating Polyradiculoneuropathy. SEARCH STRATEGY We searched the Cochrane Neuromuscular Disease Group trials register (searched December 2001), MEDLINE (searched January 1977 to December 2001), EMBASE (January 1980 to December 2001), CINAHL (searched January 1982 to December 2001) and LILACS (searched January 1982 to December 2001). We contacted the authors of the trials identified and other disease experts seeking other published and unpublished trials. SELECTION CRITERIA We sought randomised and quasi-randomised trials of all immunosuppressive agents such as azathioprine, cyclophosphamide, methotrexate, cyclosporin A, mycophenolate mofetil, and rituximab and all immunomodulatory agents such as alpha interferon and beta interferon in participants fulfilling standard diagnostic criteria for chronic inflammatory demyelinating Polyradiculoneuropathy. DATA COLLECTION AND ANALYSIS Two of us independently selected the trials which met our criteria, judged their methodological quality and extracted the data onto specially designed forms. We wanted to measure the change in disability after one year as our primary outcome measure. MAIN RESULTS We found one parallel group open trial of azathioprine for nine months involving 27 participants and another of interferon beta involving 10 participants in a double blind crossover trial with each treatment period lasting 12 weeks. Neither trial provided our primary outcome measure and neither showed a significant beneficial effect on any of the outcome measures selected by the authors or ourselves in the protocol for this review. REVIEWER'S CONCLUSIONS The evidence is inadequate to decide whether azathioprine, interferon beta or any other immunosuppressive drug or interferon is beneficial in chronic inflammatory demyelinating Polyradiculoneuropathy.
Jun Li - One of the best experts on this subject based on the ideXlab platform.
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Shortened internodal length of dermal myelinated nerve fibres in Charcot-Marie-Tooth disease type 1A.
Brain, 2009Co-Authors: Mario A. Saporta, Stacey Masse, Istvan Katona, Richard A Lewis, Jun LiAbstract:Charcot–Marie-Tooth disease type 1A is the most common inherited neuropathy and is caused by duplication of chromosome 17p11.2 containing the peripheral myelin protein-22 gene. This disease is characterized by uniform slowing of conduction velocities and secondary axonal loss, which are in contrast with non-uniform slowing of conduction velocities in acquired demyelinating disorders, such as chronic inflammatory demyelinating Polyradiculoneuropathy. Mechanisms responsible for the slowed conduction velocities and axonal loss in Charcot–Marie-Tooth disease type 1A are poorly understood, in part because of the difficulty in obtaining nerve samples from patients, due to the invasive nature of nerve biopsies. We have utilized glabrous skin biopsies, a minimally invasive procedure, to evaluate these issues systematically in patients with Charcot–Marie-Tooth disease type 1A ( n = 32), chronic inflammatory demyelinating Polyradiculoneuropathy ( n = 4) and healthy controls ( n = 12). Morphology and molecular architecture of dermal myelinated nerve fibres were examined using immunohistochemistry and electron microscopy. Internodal length was uniformly shortened in patients with Charcot–Marie-Tooth disease type 1A, compared with those in normal controls ( P < 0.0001). Segmental demyelination was absent in the Charcot–Marie-Tooth disease type 1A group, but identifiable in all patients with chronic inflammatory demyelinating Polyradiculoneuropathy. Axonal loss was measurable using the density of Meissner corpuscles and associated with an accumulation of intra-axonal mitochondria. Our study demonstrates that skin biopsy can reveal pathological and molecular architectural changes that distinguish inherited from acquired demyelinating neuropathies. Uniformly shortened internodal length in Charcot–Marie-Tooth disease type 1A suggests a potential developmental defect of internodal lengthening. Intra-axonal accumulation of mitochondria provides new insights into the pathogenesis of axonal degeneration in Charcot–Marie-Tooth disease type 1A.
Claudia Sommer - One of the best experts on this subject based on the ideXlab platform.
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auto antibodies to contactin associated protein 1 caspr in two patients with painful inflammatory neuropathy
Brain, 2016Co-Authors: Kathrin Doppler, Luise Appeltshauser, Corinna Martin, Heidrun H Kramer, Axel Haarmann, Mathias Buttmann, Carmen Villmann, Elior Peles, Claudia SommerAbstract:Auto-antibodies against the paranodal proteins neurofascin-155 and contactin-1 have recently been described in patients with chronic inflammatory demyelinating Polyradiculoneuropathy and are associated with a distinct clinical phenotype and response to treatment. Contactin-associated protein 1 (Caspr, encoded by CNTNAP1 ) is a paranodal protein that is attached to neurofascin-155 and contactin-1 (CNTN1) but has not yet been identified as a sole antigen in patients with inflammatory neuropathies. In the present study, we screened a cohort of 35 patients with chronic inflammatory demyelinating Polyradiculoneuropathy (age range 20–80, 10 female, 25 male) and 22 patients with Guillain-Barre syndrome (age range 17–86, eight female, 14 male) for autoantibodies against paranodal antigens. We identified two patients, one with chronic inflammatory demyelinating Polyradiculoneuropathy and one with Guillain-Barre syndrome, with autoantibodies against Caspr by binding assays using Caspr transfected human embryonic kidney cells and murine teased fibres. IgG3 was the predominant autoantibody subclass in the patient with Guillain-Barre syndrome, IgG4 was predominant in the patient with chronic inflammatory demyelinating Polyradiculoneuropathy. Accordingly, complement deposition after binding to HEK293 cells was detectable in the patient with IgG3 autoantibodies only, not in the patient with IgG4. Severe disruption of the paranodal and nodal architecture was detectable in teased fibres of the sural nerve biopsy and in dermal myelinated fibres, supporting the notion of the paranodes being the site of pathology. Deposition of IgG at the paranodes was detected in teased fibre preparations of the sural nerve, further supporting the pathogenicity of anti-Caspr autoantibodies. Pain was one of the predominant findings in both patients, possibly reflected by binding of patients’ IgG to TRPV1 immunoreactive dorsal root ganglia neurons. Our results demonstrate that the paranodal protein Caspr constitutes a new antigen that leads to autoantibody generation as part of the novel entity of neuropathies associated with autoantibodies against paranodal proteins. * Abbreviations : Casprs : contactin-associated protein 1 CIDP : chronic inflammatory demyelinating Polyradiculoneuropathy DRG : dorsal root ganglia GBS : Guillain-Barre syndrome HEK : human embryonic kidney
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Auto-antibodies to contactin-associated protein 1 (Caspr) in two patients with painful inflammatory neuropathy.
Brain : a journal of neurology, 2016Co-Authors: Kathrin Doppler, Luise Appeltshauser, Corinna Martin, Heidrun H Kramer, Axel Haarmann, Mathias Buttmann, Carmen Villmann, Elior Peles, Claudia SommerAbstract:Auto-antibodies against the paranodal proteins neurofascin-155 and contactin-1 have recently been described in patients with chronic inflammatory demyelinating Polyradiculoneuropathy and are associated with a distinct clinical phenotype and response to treatment. Contactin-associated protein 1 (Caspr, encoded by CNTNAP1) is a paranodal protein that is attached to neurofascin-155 and contactin-1 (CNTN1) but has not yet been identified as a sole antigen in patients with inflammatory neuropathies. In the present study, we screened a cohort of 35 patients with chronic inflammatory demyelinating Polyradiculoneuropathy (age range 20-80, 10 female, 25 male) and 22 patients with Guillain-Barré syndrome (age range 17-86, eight female, 14 male) for autoantibodies against paranodal antigens. We identified two patients, one with chronic inflammatory demyelinating Polyradiculoneuropathy and one with Guillain-Barré syndrome, with autoantibodies against Caspr by binding assays using Caspr transfected human embryonic kidney cells and murine teased fibres. IgG3 was the predominant autoantibody subclass in the patient with Guillain-Barré syndrome, IgG4 was predominant in the patient with chronic inflammatory demyelinating Polyradiculoneuropathy. Accordingly, complement deposition after binding to HEK293 cells was detectable in the patient with IgG3 autoantibodies only, not in the patient with IgG4. Severe disruption of the paranodal and nodal architecture was detectable in teased fibres of the sural nerve biopsy and in dermal myelinated fibres, supporting the notion of the paranodes being the site of pathology. Deposition of IgG at the paranodes was detected in teased fibre preparations of the sural nerve, further supporting the pathogenicity of anti-Caspr autoantibodies. Pain was one of the predominant findings in both patients, possibly reflected by binding of patients' IgG to TRPV1 immunoreactive dorsal root ganglia neurons. Our results demonstrate that the paranodal protein Caspr constitutes a new antigen that leads to autoantibody generation as part of the novel entity of neuropathies associated with autoantibodies against paranodal proteins.
F Terenghi - One of the best experts on this subject based on the ideXlab platform.
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chronic inflammatory demyelinating Polyradiculoneuropathy and multifocal motor neuropathy treatment update
Current Opinion in Neurology, 2010Co-Authors: E Nobileorazio, Francesca Gallia, Francesco Tuccillo, F TerenghiAbstract:Purpose of reviewChronic inflammatory demyelinating Polyradiculoneuropathy (CIDP) and multifocal motor neuropathy usually respond to immune therapies including steroids and plasma exchange for CIDP and high-dose intravenous immunoglobulins (IVIgs) for both diseases. Other immune therapies have been
Brendon J. Coventry - One of the best experts on this subject based on the ideXlab platform.
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Therapeutic options for chronic inflammatory demyelinating Polyradiculoneuropathy: a systematic review
BMC neurology, 2014Co-Authors: Richard Bright, Jenny M. Wilkinson, Brendon J. CoventryAbstract:Background: Chronic inflammatory demyelinating Polyradiculoneuropathy is a rare acquired immune-mediated progressive or relapsing disorder causing peripheral neuropathic disease of duration more than two months. Many individuals with chronic inflammatory demyelinating Polyradiculoneuropathy fail to make a long-term recovery with current treatment regimes. The aim of this study was to prospectively review the literature to determine the effectiveness of therapies for chronic inflammatory demyelinating Polyradiculoneuropathy. Methods: Articles published from January 1990 to December 2012 were searched for studies to treat adults with chronic inflammatory demyelinating Polyradiculoneuropathy. Peer-reviewed full-text articles published in English were included. Results: Nine placebo-controlled double-blinded randomised trials were reviewed to treat subjects with chronic inflammatory demyelinating Polyradiculoneuropathy exhibiting various degrees of effectiveness. The most effect treatments were; three randomised controlled trials using intravenous immunoglobulin, a study comparing pulsed dexamethasone and short term prednisolone and rituximab all showed promising results and were well tolerated. Conclusion: IVIg and corticosteroids remain first line treatments for CIDP. Therapies using monoclonal antibodies, such as Rituximab and Natalizumab offer the most promise for treatment of Chronic inflammatory demyelinating Polyradiculoneuropathy however they also need further research, as does the use of stem cell therapy for treating Chronic inflammatory demyelinating Polyradiculoneuropathy. Large randomised controlled trials and better patient selection are required to address responsiveness of CIDP patients to conventional treatments to elucidate mechanisms of action and future directions for therapeutic improvement.
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therapeutic options for chronic inflammatory demyelinating Polyradiculoneuropathy a systematic review
BMC Neurology, 2014Co-Authors: Richard Bright, Jenny M. Wilkinson, Brendon J. CoventryAbstract:Background Chronic inflammatory demyelinating Polyradiculoneuropathy is a rare acquired immune-mediated progressive or relapsing disorder causing peripheral neuropathic disease of duration more than two months. Many individuals with chronic inflammatory demyelinating Polyradiculoneuropathy fail to make a long-term recovery with current treatment regimes. The aim of this study was to prospectively review the literature to determine the effectiveness of therapies for chronic inflammatory demyelinating Polyradiculoneuropathy.