Abstract
This chapter discusses the clinical manifestations, pathogenesis, and treatment of Guillain-Barre syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP), including recent advances in immunopathogenesis as well as the treatments in pipeline. GBS and CIDP are part of the spectrum of autoimmune-mediated neuropathy. The target antigen remains elusive in CIDP and acute inflammatory demyelinating polyneuropathy, the most common variant of GBS in North America. Autoimmunity against different gangliosides is involved in the pathogenesis of GBS variants, i.e., acute motor axonal neuropathy, Miller Fisher syndrome, and acute motor and sensory axonal neuropathy, as well as their sub-variants. As GBS is a monophasic disease, immunomodulatory treatment, currently consisting of intravenous immunoglobulin infusions or plasma exchange, is required only during the acute phase, unless there are relapses. On the other hand, maintenance treatment with the aforementioned treatment modalities, as well as corticosteroids or other immunosuppressants, is frequently needed in CIDP patients. Further advances in the understanding of immunopathogenesis of these diseases will likely result in development of new treatments involving targeted immunotherapy.
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Yuki N, Hartung HP. Guillain-Barre syndrome. N Engl J Med. 2012;366:2294–304.
van den Berg B, Walgaard C, Drenthen J, Fokke C, Jacobs BC, van Doorn PA. Guillain-Barre syndrome: pathogenesis, diagnosis, treatment and prognosis. Nat Rev Neurol. 2014;10:469–82.
van den Berg B, Fokke C, Drenthen J, van Doorn PA, Jacobs BC. Paraparetic Guillain-Barre syndrome. Neurology. 2014;82:1984–9.
Kuwabara S, Yuki N. Axonal Guillain-Barre syndrome: concepts and controversies. Lancet Neurol. 2013;12:1180–8.
Hiraga A, Kuwabara S, Ogawara K, et al. Patterns and serial changes in electrodiagnostic abnormalities of axonal Guillain-Barre syndrome. Neurology. 2005;64:856–60.
Aranyi Z, Kovacs T, Sipos I, Bereczki D. Miller Fisher syndrome: brief overview and update with a focus on electrophysiological findings. Eur J Neurol. 2012;19:15–20. e11–13
Wakerley BR, Yuki N. Pharyngeal-cervical-brachial variant of Guillain-Barre syndrome. J Neurol Neurosurg Psychiatry. 2014;85:339–44.
Wakerley BR, Yuki N. Guillain-Barre syndrome. Expert Rev Neurother. 2015;15:847–9.
Uncini A, Manzoli C, Notturno F, Capasso M. Pitfalls in electrodiagnosis of Guillain-Barre syndrome subtypes. J Neurol Neurosurg Psychiatry. 2010;81:1157–63.
Asbury AK, Arnason BG, Adams RD. The inflammatory lesion in idiopathic polyneuritis. Its role in pathogenesis. Medicine. 1969;48:173–215.
Prineas JW. Pathology of the Guillain-Barre syndrome. Ann Neurol. 1981;9(Suppl):6–19.
Hafer-Macko CE, Sheikh KA, Li CY, et al. Immune attack on the Schwann cell surface in acute inflammatory demyelinating polyneuropathy. Ann Neurol. 1996;39:625–35.
Griffin JW, Li CY, Ho TW, et al. Guillain-Barre syndrome in northern China. The spectrum of neuropathological changes in clinically defined cases. Brain (A Journal of Neurology). 1995;118(Pt 3):577–95.
Phillips MS, Stewart S, Anderson JR. Neuropathological findings in Miller Fisher syndrome. J Neurol Neurosurg Psychiatry. 1984;47:492–5.
Jacobs BC, Rothbarth PH, van der Meche FG, et al. The spectrum of antecedent infections in Guillain-Barre syndrome: a case-control study. Neurology. 1998;51:1110–5.
Yuki N, Taki T, Inagaki F, et al. A bacterium lipopolysaccharide that elicits Guillain-Barre syndrome has a GM1 ganglioside-like structure. J Exp Med. 1993;178:1771–5.
Koga M, Takahashi M, Masuda M, Hirata K, Yuki N. Campylobacter gene polymorphism as a determinant of clinical features of Guillain-Barre syndrome. Neurology. 2005;65:1376–81.
Tam CC, Rodrigues LC, Petersen I, Islam A, Hayward A, O'Brien SJ. Incidence of Guillain-Barre syndrome among patients with Campylobacter infection: a general practice research database study. J Infect Dis. 2006;194:95–7.
Kuwabara S, Ogawara K, Misawa S, et al. Does Campylobacter jejuni infection elicit "demyelinating" Guillain-Barre syndrome? Neurology. 2004;63:529–33.
Illa I, Ortiz N, Gallard E, Juarez C, Grau JM, Dalakas MC. Acute axonal Guillain-Barre syndrome with IgG antibodies against motor axons following parenteral gangliosides. Ann Neurol. 1995;38:218–24.
Willison HJ, Yuki N. Peripheral neuropathies and anti-glycolipid antibodies. Brain (A Journal of Neurology). 2002;125:2591–625.
Kusunoki S, Kaida K. Antibodies against ganglioside complexes in Guillain-Barre syndrome and related disorders. J Neurochem. 2011;116:828–32.
Dalakas MC. Pathogenesis of immune-mediated neuropathies. Biochim Biophys Acta. 1852;2015:658–66.
Gong Y, Tagawa Y, Lunn MP, et al. Localization of major gangliosides in the PNS: implications for immune neuropathies. Brain (A Journal of Neurology). 2002;125:2491–506.
McGonigal R, Rowan EG, Greenshields KN, et al. Anti-GD1a antibodies activate complement and calpain to injure distal motor nodes of Ranvier in mice. Brain (A Journal of Neurology). 2010;133:1944–60.
Burkel WE. The histological fine structure of perineurium. Anat Rec. 1967;158:177–89.
Olsson Y. Microenvironment of the peripheral nervous system under normal and pathological conditions. Crit Rev Neurobiol. 1990;5:265–311.
Sawai S, Satoh M, Mori M, et al. Moesin is a possible target molecule for cytomegalovirus-related Guillain-Barre syndrome. Neurology. 2014;83:113–7.
Gatto CL, Walker BJ, Lambert S. Local ERM activation and dynamic growth cones at Schwann cell tips implicated in efficient formation of nodes of Ranvier. J Cell Biol. 2003;162:489–98.
Li S, Yu M, Li H, Zhang H, Jiang Y. IL-17 and IL-22 in cerebrospinal fluid and plasma are elevated in Guillain-Barre syndrome. Mediators Inflamm. 2012;2012:260473.
Li S, Jin T, Zhang HL, et al. Circulating Th17, Th22, and Th1 cells are elevated in the Guillain-Barre syndrome and downregulated by IVIg treatments. Mediators Inflamm. 2014;2014:740947.
Maddur MS, Rabin M, Hegde P, et al. Intravenous immunoglobulin exerts reciprocal regulation of Th1/Th17 cells and regulatory T cells in Guillain-Barre syndrome patients. Immunol Res. 2014;60:320–9.
Sakaguchi S, Yamaguchi T, Nomura T, Ono M. Regulatory T cells and immune tolerance. Cell. 2008;133:775–87.
Waksman BH, Adams RD. Allergic neuritis: an experimental disease of rabbits induced by the injection of peripheral nervous tissue and adjuvants. J Exp Med. 1955;102:213–36.
Rostami A, Gregorian SK, Brown MJ, Pleasure DE. Induction of severe experimental autoimmune neuritis with a synthetic peptide corresponding to the 53-78 amino acid sequence of the myelin P2 protein. J Neuroimmunol. 1990;30:145–51.
Soliven B. Animal models of autoimmune neuropathy. ILAR (Journal/National Research Council, Institute of Laboratory Animal Resources). 2014;54:282–90.
Astrom KE, Webster HD, Arnason BG. The initial lesion in experimental allergic neuritis. A phase and electron microscopic study. J Exp Med. 1968;128:469–95.
Taylor JM, Pollard JD. Dominance of autoreactive T cell-mediated delayed-type hypersensitivity or antibody-mediated demyelination results in distinct forms of experimental autoimmune neuritis in the Lewis rat. J Neuropathol Exp Neurol. 2001;60:637–46.
Lonigro A, Devaux JJ. Disruption of neurofascin and gliomedin at nodes of Ranvier precedes demyelination in experimental allergic neuritis. Brain (A Journal of neurology). 2009;132:260–73.
Devaux JJ. Antibodies to gliomedin cause peripheral demyelinating neuropathy and the dismantling of the nodes of Ranvier. Am J Pathol. 2012;181:1402–13.
Yuki N, Yamada M, Koga M, et al. Animal model of axonal Guillain-Barre syndrome induced by sensitization with GM1 ganglioside. Ann Neurol. 2001;49:712–20.
Susuki K, Yuki N, Schafer DP, et al. Dysfunction of nodes of Ranvier: a mechanism for anti-ganglioside antibody-mediated neuropathies. Exp Neurol. 2012;233:534–42.
van Doorn PA, Ruts L, Jacobs BC. Clinical features, pathogenesis, and treatment of Guillain-Barre syndrome. Lancet Neurol. 2008;7:939–50.
Lawn ND, Fletcher DD, Henderson RD, Wolter TD, Wijdicks EF. Anticipating mechanical ventilation in Guillain-Barre syndrome. Arch Neurol. 2001;58:893–8.
Sharshar T, Chevret S, Bourdain F, Raphael JC. French Cooperative Group on Plasma Exchange in Guillain-Barre S. Early predictors of mechanical ventilation in Guillain-Barre syndrome. Crit Care Med. 2003;31:278–83.
Ropper AH, Kehne SM. Guillain-Barre syndrome: management of respiratory failure. Neurology. 1985;35:1662–5.
Dimario Jr FJ, Edwards C. Autonomic dysfunction in childhood Guillain-Barre syndrome. J Child Neurol. 2012;27:581–6.
Ruts L, Drenthen J, Jongen JL, et al. Pain in Guillain-Barre syndrome: a long-term follow-up study. Neurology. 2010;75:1439–47.
Hiraga A, Nagumo K, Suzuki K, Sakakibara Y, Kojima S. [A patient with Guillain-Barre syndrome and recurrent episodes of ST elevation and left ventricular hypokinesis in the anterior wall]. No to shinkei =. Brain Nerve. 2003;55:517–20.
Burns TM, Lawn ND, Low PA, Camilleri M, Wijdicks EF. Adynamic ileus in severe Guillain-Barre syndrome. Muscle Nerve. 2001;24:963–5.
Sakakibara R, Hattori T, Kuwabara S, Yamanishi T, Yasuda K. Micturitional disturbance in patients with Guillain-Barre syndrome. J Neurol Neurosurg Psychiatry. 1997;63:649–53.
Sakakibara R, Uchiyama T, Kuwabara S, et al. Prevalence and mechanism of bladder dysfunction in Guillain-Barre Syndrome. NeurourolUrodyn. 2009;28:432–7.
Amatya B, Khan F, Whishaw M, Pallant JF. Guillain-Barre syndrome: prevalence and long-term factors impacting bladder function in an Australian community cohort. J Clin Neurol. 2013;9:144–50.
Winer JB, Hughes RA, Osmond C. A prospective study of acute idiopathic neuropathy. I. Clinical features and their prognostic value. J Neurol Neurosurg Psychiatry. 1988;51:605–12.
Hughes RA, van Doorn PA. Corticosteroids for Guillain-Barre syndrome. Cochrane Database Syst Rev. 2012;8:CD001446.
Efficiency of plasma exchange in Guillain-Barre syndrome: role of replacement fluids. French Cooperative Group on Plasma Exchange in Guillain-Barre syndrome. Ann Neurol. 1987;22:753–61.
Appropriate number of plasma exchanges in Guillain-Barre syndrome. The French Cooperative Group on Plasma Exchange in Guillain-Barre Syndrome. Ann Neurol. 1997;41:298–306.
Plasmapheresis and acute Guillain-Barre syndrome. The Guillain-Barre syndrome Study Group. Neurology. 1985;35:1096–1104.
Hughes RA, Swan AV, Raphael JC, Annane D, van Koningsveld R, van Doorn PA. Immunotherapy for Guillain-Barre syndrome: a systematic review. Brain (A Journal of Neurology). 2007;130:2245–57.
Greenwood RJ, Newsom-Davis J, Hughes RA, et al. Controlled trial of plasma exchange in acute inflammatory polyradiculoneuropathy. Lancet. 1984;1:877–9.
van der Meche FG, Schmitz PI. A randomized trial comparing intravenous immune globulin and plasma exchange in Guillain-Barre syndrome. Dutch Guillain-Barre Study Group. N Engl J Med. 1992;326:1123–9.
Randomised trial of plasma exchange, intravenous immunoglobulin, and combined treatments in Guillain-Barre syndrome. Plasma Exchange/Sandoglobulin Guillain-Barre Syndrome Trial Group. Lancet. 1997;349:225–30.
Korinthenberg R, Schessl J, Kirschner J, Monting JS. Intravenously administered immunoglobulin in the treatment of childhood Guillain-Barre syndrome: a randomized trial. Pediatrics. 2005;116:8–14.
Kleyweg RP, van der Meche FG. Treatment related fluctuations in Guillain-Barre syndrome after high-dose immunoglobulins or plasma-exchange. J Neurol Neurosurg Psychiatry. 1991;54:957–60.
Kuitwaard K, de Gelder J, Tio-Gillen AP, et al. Pharmacokinetics of intravenous immunoglobulin and outcome in Guillain-Barre syndrome. Ann Neurol. 2009;66:597–603.
Dada MA, Kaplan AA. Plasmapheresis treatment in Guillain-Barre syndrome: potential benefit over IVIg in patients with axonal involvement. Ther Apher Dial (Official Peer-Reviewed Journal of the International Society for Apheresis, the Japanese Society for Apheresis, the Japanese Society for Dialysis Therapy). 2004;8:409–12.
Mori M, Kuwabara S, Fukutake T, Hattori T. Intravenous immunoglobulin therapy for Miller Fisher syndrome. Neurology. 2007;68:1144–6.
Hillmen P, Hall C, Marsh JC, et al. Effect of eculizumab on hemolysis and transfusion requirements in patients with paroxysmal nocturnal hemoglobinuria. N Engl J Med. 2004;350:552–9.
Halstead SK, Zitman FM, Humphreys PD, et al. Eculizumab prevents anti-ganglioside antibody-mediated neuropathy in a murine model. Brain (A Journal of Neurology). 2008;131:1197–208.
Phongsisay V, Susuki K, Matsuno K, et al. Complement inhibitor prevents disruption of sodium channel clusters in a rabbit model of Guillain-Barre syndrome. J Neuroimmunol. 2008;205:101–4.
Van den Bergh PY, Hadden RD, Bouche P, et al. European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society – first revision. Eur J Neurol. 2010;17:356–63.
Gorson KC, Katz J. Chronic inflammatory demyelinating polyneuropathy. Neurol Clin. 2013;31:511–32.
Saperstein DS, Katz JS, Amato AA, Barohn RJ. Clinical spectrum of chronic acquired demyelinating polyneuropathies. Muscle Nerve. 2001;24:311–24.
Boukhris S, Magy L, Khalil M, Sindou P, Vallat JM. Pain as the presenting symptom of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). J Neurol Sci. 2007;254:33–8.
Henderson RD, Sandroni P, Wijdicks EF. Chronic inflammatory demyelinating polyneuropathy and respiratory failure. J Neurol. 2005;252:1235–7.
Dyck PJ, Lais AC, Ohta M, Bastron JA, Okazaki H, Groover RV. Chronic inflammatory polyradiculoneuropathy. Mayo Clin Proc. 1975;50:621–37.
Mathey EK, Park SB, Hughes RA, et al. Chronic inflammatory demyelinating polyradiculoneuropathy: from pathology to phenotype. J Neurol Neurosurg Psychiatry. 2015;86:973–85.
Viala K, Maisonobe T, Stojkovic T, et al. A current view of the diagnosis, clinical variants, response to treatment and prognosis of chronic inflammatory demyelinating polyradiculoneuropathy. J Peripher Nerv Syst. 2010;15:50–6.
Oh SJ, Joy JL, Kuruoglu R. "Chronic sensory demyelinating neuropathy": chronic inflammatory demyelinating polyneuropathy presenting as a pure sensory neuropathy. J Neurol Neurosurg Psychiatry. 1992;55:677–80.
Rajabally YA, Wong SL. Chronic inflammatory pure sensory polyradiculoneuropathy: a rare CIDP variant with unusual electrophysiology. J Clin Neuromuscul Dis. 2012;13:149–52.
van Dijk GW, Notermans NC, Franssen H, Wokke JH. Development of weakness in patients with chronic inflammatory demyelinating polyneuropathy and only sensory symptoms at presentation: a long-term follow-up study. J Neurol. 1999;246:1134–9.
Simmons Z, Tivakaran S. Acquired demyelinating polyneuropathy presenting as a pure clinical sensory syndrome. Muscle Nerve. 1996;19:1174–6.
Yato M, Ohkoshi N, Sato A, Shoji S, Kusunoki S. Ataxic form of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Eur J Neurol. 2000;7:227–30.
Sinnreich M, Klein CJ, Daube JR, Engelstad J, Spinner RJ, Dyck PJ. Chronic immune sensory polyradiculopathy: a possibly treatable sensory ataxia. Neurology. 2004;63:1662–9.
Yiannikas C, Vucic S. Utility of somatosensory evoked potentials in chronic acquired demyelinating neuropathy. Muscle Nerve. 2008;38:1447–54.
Sabatelli M, Madia F, Mignogna T, Lippi G, Quaranta L, Tonali P. Pure motor chronic inflammatory demyelinating polyneuropathy. J Neurol. 2001;248:772–7.
Hattori N, Misu K, Koike H, et al. Age of onset influences clinical features of chronic inflammatory demyelinating polyneuropathy. J Neurol Sci. 2001;184:57–63.
Pitarokoili K, Schlamann M, Kerasnoudis A, Gold R, Yoon MS. Comparison of clinical, electrophysiological, sonographic and MRI features in CIDP. J Neurol Sci. 2015;357:198–203.
Matsuda M, Ikeda S, Sakurai S, Nezu A, Yanagisawa N, Inuzuka T. Hypertrophic neuritis due to chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): a postmortem pathological study. Muscle Nerve. 1996;19:163–9.
Vital C, Vital A, Lagueny A, et al. Chronic inflammatory demyelinating polyneuropathy: immunopathological and ultrastructural study of peripheral nerve biopsy in 42 cases. Ultrastruct Pathol. 2000;24:363–9.
Barohn RJ, Kissel JT, Warmolts JR, Mendell JR. Chronic inflammatory demyelinating polyradiculoneuropathy. Clinical characteristics, course, and recommendations for diagnostic criteria. Arch Neurol. 1989;46:878–84.
Gorson KC, Allam G, Ropper AH. Chronic inflammatory demyelinating polyneuropathy: clinical features and response to treatment in 67 consecutive patients with and without a monoclonal gammopathy. Neurology. 1997;48:321–8.
Harvey GK, Pollard JD, Schindhelm K, Antony J. Chronic experimental allergic neuritis. An electrophysiological and histological study in the rabbit. J Neurol Sci. 1987;81:215–25.
Adam AM, Atkinson PF, Hall SM, Hughes RA, Taylor WA. Chronic experimental allergic neuritis in Lewis rats. Neuropathol Appl Neurobiol. 1989;15:249–64.
Crino PB, Grossman RI, Rostami A. Magnetic resonance imaging of the cauda equina in chronic inflammatory demyelinating polyneuropathy. Ann Neurol. 1993;33:311–3.
Morgan GW, Barohn RJ, Bazan 3rd C, King RB, Klucznik RP. Nerve root enhancement with MRI in inflammatory demyelinating polyradiculoneuropathy. Neurology. 1993;43:618–20.
McCombe PA, Pollard JD, McLeod JG. Chronic inflammatory demyelinating polyradiculoneuropathy. A clinical and electrophysiological study of 92 cases. Brain (A Journal of Neurology). 1987;110(Pt 6):1617–30.
Chio A, Cocito D, Bottacchi E, et al. Idiopathic chronic inflammatory demyelinating polyneuropathy: an epidemiological study in Italy. J Neurol Neurosurg Psychiatry. 2007;78:1349–53.
Gibbels E, Diederich N. Human immunodeficiency virus (HIV)-related chronic relapsing inflammatory demyelinating polyneuropathy with multifocal unusual onion bulbs in sural nerve biopsy. A clinicomorphological study with qualitative and quantitative light and electron microscopy. Acta Neuropathol. 1988;75:529–34.
Chimowitz MI, Audet AM, Hallet A, Kelly Jr JJ. HIV-associated CIDP. Muscle Nerve. 1989;12:695–6.
Bird SJ, Brown MJ, Shy ME, Scherer SS. Chronic inflammatory demyelinating polyneuropathy associated with malignant melanoma. Neurology. 1996;46:822–4.
Weiss MD, Luciano CA, Semino-Mora C, Dalakas MC, Quarles RH. Molecular mimicry in chronic inflammatory demyelinating polyneuropathy and melanoma. Neurology. 1998;51:1738–41.
Rousseau A, Salachas F, Baccard M, Delattre JY, Sanson M. Chronic inflammatory polyneuropathy revealing malignant melanoma. J Neurooncol. 2005;71:335–6.
Noronha AB, Harper JR, Ilyas AA, Reisfeld RA, Quarles RH. Myelin-associated glycoprotein shares an antigenic determinant with a glycoprotein of human melanoma cells. J Neurochem. 1986;47:1558–65.
Schmidt B, Toyka KV, Kiefer R, Full J, Hartung HP, Pollard J. Inflammatory infiltrates in sural nerve biopsies in Guillain-Barre syndrome and chronic inflammatory demyelinating neuropathy. Muscle Nerve. 1996;19:474–87.
Chi LJ, Xu WH, Zhang ZW, Huang HT, Zhang LM, Zhou J. Distribution of Th17 cells and Th1 cells in peripheral blood and cerebrospinal fluid in chronic inflammatory demyelinating polyradiculoneuropathy. J Peripher Nerv Syst. 2010;15:345–56.
Winer J, Hughes S, Cooper J, Ben-Smith A, Savage C. gamma delta T cells infiltrating sensory nerve biopsies from patients with inflammatory neuropathy. J Neurol. 2002;249:616–21.
Renaud S, Erne B, Fuhr P, et al. Matrix metalloproteinases-9 and -2 in secondary vasculitic neuropathies. Acta Neuropathol. 2003;105:37–42.
Schneider-Hohendorf T, Schwab N, Uceyler N, Gobel K, Sommer C, Wiendl H. CD8+ T-cell immunity in chronic inflammatory demyelinating polyradiculoneuropathy. Neurology. 2012;78:402–8.
Mausberg AK, Dorok M, Stettner M, et al. Recovery of the T-cell repertoire in CIDP by IV immunoglobulins. Neurology. 2013;80:296–303.
Chi LJ, Wang HB, Wang WZ. Impairment of circulating CD4+CD25+ regulatory T cells in patients with chronic inflammatory demyelinating polyradiculoneuropathy. J Peripher Nerv Syst. 2008;13:54–63.
Sanvito L, Makowska A, Gregson N, Nemni R, Hughes RA. Circulating subsets and CD4(+)CD25(+) regulatory T cell function in chronic inflammatory demyelinating polyradiculoneuropathy. Autoimmunity. 2009;42:667–77.
Ledbetter JA, Imboden JB, Schieven GL, et al. CD28 ligation in T-cell activation: evidence for two signal transduction pathways. Blood. 1990;75:1531–9.
Khalili-Shirazi A, Gregson NA, Londei M, Summers L, Hughes RA. The distribution of CD1 molecules in inflammatory neuropathy. J Neurol Sci. 1998;158:154–63.
Van Rhijn I, Van den Berg LH, Bosboom WM, Otten HG, Logtenberg T. Expression of accessory molecules for T-cell activation in peripheral nerve of patients with CIDP and vasculitic neuropathy. Brain (A Journal of Neurology). 2000;123(Pt 10):2020–9.
Sommer C, Koch S, Lammens M, Gabreels-Festen A, Stoll G, Toyka KV. Macrophage clustering as a diagnostic marker in sural nerve biopsies of patients with CIDP. Neurology. 2005;65:1924–9.
Dalakas MC, Engel WK. Immunoglobulin and complement deposits in nerves of patients with chronic relapsing polyneuropathy. Arch Neurol. 1980;37:637–40.
Hays AP, Lee SS, Latov N. Immune reactive C3d on the surface of myelin sheaths in neuropathy. J Neuroimmunol. 1988;18:231–44.
Yan WX, Taylor J, Andrias-Kauba S, Pollard JD. Passive transfer of demyelination by serum or IgG from chronic inflammatory demyelinating polyneuropathy patients. Ann Neurol. 2000;47:765–75.
Koller H, Kieseier BC, Jander S, Hartung HP. Chronic inflammatory demyelinating polyneuropathy. N Engl J Med. 2005;352:1343–56.
Dalakas MC, Medscape. Advances in the diagnosis, pathogenesis and treatment of CIDP. Nat Rev Neurol. 2011;7:507–17.
Yan WX, Archelos JJ, Hartung HP, Pollard JD. P0 protein is a target antigen in chronic inflammatory demyelinating polyradiculoneuropathy. Ann Neurol. 2001;50:286–92.
Devaux JJ, Odaka M, Yuki N. Nodal proteins are target antigens in Guillain-Barre syndrome. J Peripher Nerv Syst. 2012;17:62–71.
Querol L, Nogales-Gadea G, Rojas-Garcia R, et al. Antibodies to contactin-1 in chronic inflammatory demyelinating polyneuropathy. Ann Neurol. 2013;73:370–80.
Labasque M, Hivert B, Nogales-Gadea G, Querol L, Illa I, Faivre-Sarrailh C. Specific contactin N-glycans are implicated in neurofascin binding and autoimmune targeting in peripheral neuropathies. J Biol Chem. 2014;289:7907–18.
Miura Y, Devaux JJ, Fukami Y, et al. Contactin 1 IgG4 associates to chronic inflammatory demyelinating polyneuropathy with sensory ataxia. Brain (A Journal of Neurology). 2015;138:1484–91.
Devaux JJ, Miura Y, Fukami Y, et al. Neurofascin-155 IgG4 in chronic inflammatory demyelinating polyneuropathy. Neurology. 2016;86:800–7.
McCombe PA, van der Kreek SA, Pender MP. The effects of prophylactic cyclosporin A on experimental allergic neuritis (EAN) in the Lewis rat. Induction of relapsing EAN using low dose cyclosporin A. J Neuroimmunol. 1990;28:131–40.
Salomon B, Rhee L, Bour-Jordan H, et al. Development of spontaneous autoimmune peripheral polyneuropathy in B7-2-deficient NOD mice. J Exp Med. 2001;194:677–84.
Kim HJ, Jung CG, Jensen MA, Dukala D, Soliven B. Targeting of myelin protein zero in a spontaneous autoimmune polyneuropathy. J Immunol. 2008;181:8753–60.
Hughes RA, Mehndiratta MM. Corticosteroids for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database Syst Rev. 2012;8:CD002062.
Eftimov F, Winer JB, Vermeulen M, de Haan R, van Schaik IN. Intravenous immunoglobulin for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database Syst Rev. 2013;12:CD001797.
Mehndiratta MM, Hughes RA. Plasma exchange for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database Syst Rev. 2012;9:CD003906.
Cocito D, Paolasso I, Antonini G, et al. A nationwide retrospective analysis on the effect of immune therapies in patients with chronic inflammatory demyelinating polyradiculoneuropathy. Eur J Neurol. 2010;17:289–94.
Dyck PJ, O'Brien PC, Oviatt KF, et al. Prednisone improves chronic inflammatory demyelinating polyradiculoneuropathy more than no treatment. Ann Neurol. 1982;11:136–41.
van Schaik IN, Eftimov F, van Doorn PA, et al. Pulsed high-dose dexamethasone versus standard prednisolone treatment for chronic inflammatory demyelinating polyradiculoneuropathy (PREDICT study): a double-blind, randomised, controlled trial. Lancet Neurol. 2010;9:245–53.
Lopate G, Pestronk A, Al-Lozi M. Treatment of chronic inflammatory demyelinating polyneuropathy with high-dose intermittent intravenous methylprednisolone. Arch Neurol. 2005;62:249–54.
Iijima M, Yamamoto M, Hirayama M, et al. Clinical and electrophysiologic correlates of IVIg responsiveness in CIDP. Neurology. 2005;64:1471–5.
Buttmann M, Kaveri S, Hartung HP. Polyclonal immunoglobulin G for autoimmune demyelinating nervous system disorders. Trends Pharmacol Sci. 2013;34:445–57.
Wong AH, Fukami Y, Sudo M, Kokubun N, Hamada S, Yuki N. Sialylated IgG-Fc: a novel biomarker of chronic inflammatory demyelinating polyneuropathy. J Neurol Neurosurg Psychiatry. 2016;87:275–9.
Brannagan 3rd TH. Intravenous gamma globulin (IVIg) for treatment of CIDP and related immune-mediated neuropathies. Neurology. 2002;59:S33–40.
Hughes RA, Donofrio P, Bril V, et al. Intravenous immune globulin (10% caprylate-chromatography purified) for the treatment of chronic inflammatory demyelinating polyradiculoneuropathy (ICE study): a randomised placebo-controlled trial. Lancet Neurol. 2008;7:136–44.
Markvardsen LH, Debost JC, Harbo T, et al. Subcutaneous immunoglobulin in responders to intravenous therapy with chronic inflammatory demyelinating polyradiculoneuropathy. Eur J Neurol. 2013;20:836–42.
Markvardsen LH, Harbo T, Sindrup SH, et al. Subcutaneous immunoglobulin preserves muscle strength in chronic inflammatory demyelinating polyneuropathy. Eur J Neurol. 2014;21:1465–70.
Kuitwaard K, van den Berg LH, Vermeulen M, et al. Randomised controlled trial comparing two different intravenous immunoglobulins in chronic inflammatory demyelinating polyradiculoneuropathy. J Neurol Neurosurg Psychiatry. 2010;81:1374–9.
Nobile-Orazio E, Cocito D, Jann S, et al. Intravenous immunoglobulin versus intravenous methylprednisolone for chronic inflammatory demyelinating polyradiculoneuropathy: a randomised controlled trial. Lancet Neurol. 2012;11:493–502.
Dyck PJ, Daube J, O'Brien P, et al. Plasma exchange in chronic inflammatory demyelinating polyradiculoneuropathy. N Engl J Med. 1986;314:461–5.
Hahn AF, Bolton CF, Pillay N, et al. Plasma-exchange therapy in chronic inflammatory demyelinating polyneuropathy. A double-blind, sham-controlled, cross-over study. Brain (A Journal of Neurology). 1996;119(Pt 4):1055–66.
Hughes RA, Swan AV, van Doorn PA. Cytotoxic drugs and interferons for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database Syst Rev. 2004:CD003280.
Dyck PJ, O'Brien P, Swanson C, Low P, Daube J. Combined azathioprine and prednisone in chronic inflammatory-demyelinating polyneuropathy. Neurology. 1985;35:1173–6.
Stewart JD, McKelvey R, Durcan L, Carpenter S, Karpati G. Chronic inflammatory demyelinating polyneuropathy (CIDP) in diabetics. J Neurol Sci. 1996;142:59–64.
Haq RU, Pendlebury WW, Fries TJ, Tandan R. Chronic inflammatory demyelinating polyradiculoneuropathy in diabetic patients. Muscle Nerve. 2003;27:465–70.
Group RMCT. Randomised controlled trial of methotrexate for chronic inflammatory demyelinating polyradiculoneuropathy (RMC trial): a pilot, multicentre study. Lancet Neurol. 2009;8:158–64.
Hadden RD, Sharrack B, Bensa S, Soudain SE, Hughes RA. Randomized trial of interferon beta-1a in chronic inflammatory demyelinating polyradiculoneuropathy. Neurology. 1999;53:57–61.
Brannagan 3rd TH, Pradhan A, Heiman-Patterson T, et al. High-dose cyclophosphamide without stem-cell rescue for refractory CIDP. Neurology. 2002;58:1856–8.
Visudtibhan A, Chiemchanya S, Visudhiphan P. Cyclosporine in chronic inflammatory demyelinating polyradiculoneuropathy. Pediatr Neurol. 2005;33:368–72.
Bedi G, Brown A, Tong T, Sharma KR. Chronic inflammatory demyelinating polyneuropathy responsive to mycophenolate mofetil therapy. J Neurol Neurosurg Psychiatry. 2010;81:634–6.
Gorson KC, Amato AA, Ropper AH. Efficacy of mycophenolate mofetil in patients with chronic immune demyelinating polyneuropathy. Neurology. 2004;63:715–7.
Press R, Askmark H, Svenningsson A, et al. Autologous haematopoietic stem cell transplantation: a viable treatment option for CIDP. J Neurol Neurosurg Psychiatry. 2014;85:618–24.
Benedetti L, Briani C, Franciotta D, et al. Rituximab in patients with chronic inflammatory demyelinating polyradiculoneuropathy: a report of 13 cases and review of the literature. J Neurol Neurosurg Psychiatry. 2011;82:306–8.
Querol L, Rojas-Garcia R, Diaz-Manera J, et al. Rituximab in treatment-resistant CIDP with antibodies against paranodal proteins. Neurol Neuroimmunol Neuroinflamm. 2015;2:e149.
Marsh EA, Hirst CL, Llewelyn JG, et al. Alemtuzumab in the treatment of IVIG-dependent chronic inflammatory demyelinating polyneuropathy. J Neurol. 2010;257:913–9.
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Grebenciucova, E., Rezania, K. (2017). Immunopathogenesis and Treatment of Guillain-Barre Syndrome and Chronic Inflammatory Demyelinating Polyneuropathy. In: Minagar, A., Alexander, J. (eds) Inflammatory Disorders of the Nervous System. Current Clinical Neurology. Humana Press, Cham. https://doi.org/10.1007/978-3-319-51220-4_10
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