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Chronic inflammatory demyelinating polyneuropathy

Published:November 12, 2012DOI:https://doi.org/10.1016/j.jns.2012.10.020

      Abstract

      Chronic inflammatory demyelinating polyneuropathy (CIDP) is the commonest treatable neuropathy in the western world. Untreated it may result in severe disability but if diagnosed and treated early there is effective treatment for the majority of patients. Typical CIDP is readily recognised but the diagnosis of other subgroups can be more challenging. The pathology of polyradiculoneuropathies such as CIDP characteristically affects the most proximal regions of the peripheral nervous system, nerve roots and major plexuses. It is important to test these regions with electrodiagnostic studies since routine neurophysiology may not encounter regions of pathology. Although accepted as an autoimmune disorder with an underlying immunopathology involving T cell and B cell responses, there is no agreement on major target antigens; however recent studies have highlighted a role for molecules in non compact myelin which play an essential role in the formation and maintenance of the nodal structures and hence in the function of ion channels central to saltatory conduction. Controlled trials have proven the efficacy of corticosteroid, intravenous immunoglobulin and plasma exchange in the short term and intravenous immunoglobulin also in the long term. Immunosuppressive agents are widely used but their efficacy has not been proven in controlled trials. Recent trials have shown the importance of attempting treatment withdrawal in patients apparently in remission to conserve treatments that are very expensive and in short supply, since a significant proportion of patients may enter long lasting remission following short term therapy. For the relatively small group of patients who do not respond to these first line therapies new agents including monoclonal antibodies may have a role.

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      References

        • Van den Bergh P.Y.
        • Hadden R.D.
        • Bouche P.
        • Cornblath D.R.
        • Hahn A.
        • Illa I.
        • 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. Mar 2010; 17: 356-363
        • Latov N.
        Diagnosis of CIDP.
        Neurology. Dec 24, 2002; 59: S2-S6
        • Hyland H.H.
        • Russell W.R.
        Chronic progressive polyneuritis, with report of a fatal case.
        Brain. 1930; 53: 278-289
        • Dyck P.J.
        • Lais A.C.
        • Ohta M.
        • Bastron J.A.
        • Okazaki H.
        • Groover R.V.
        Chronic inflammatory polyradiculoneuropathy.
        Mayo Clin Proc. Nov 1975; 50: 621-637
        • Duggins A.J.
        • McLeod J.G.
        • Pollard J.D.
        • Davies L.
        • Yang F.
        • Thompson E.O.
        • et al.
        Spinal root and plexus hypertrophy in chronic inflammatory demyelinating polyneuropathy.
        Brain. Jul 1999; 122: 1383-1390
        • Harvey G.K.
        • Pollard J.D.
        • Schindhelm K.
        • Antony J.
        Chronic experimental allergic neuritis. An electrophysiological and histological study in the rabbit.
        J Neurol Sci. Nov 1987; 81: 215-225
        • Prineas J.W.
        • McLeod J.G.
        Chronic relapsing polyneuritis.
        J Neurol Sci. Apr 1976; 27: 427-458
        • Pollard J.D.
        • McCombe P.A.
        • Baverstock J.
        • Gatenby P.A.
        • McLeod J.G.
        Class II antigen expression and T lymphocyte subsets in chronic inflammatory demyelinating polyneuropathy.
        J Neuroimmunol. Dec 1986; 13: 123-134
        • Spies J.M.
        • Westland K.W.
        • Bonner J.G.
        • Pollard J.D.
        Intraneural activated T cells cause focal breakdown of the blood-nerve barrier.
        Brain. Aug 1995; 118: 857-868
        • Spies J.M.
        • Pollard J.D.
        • Bonner J.G.
        • Westland K.W.
        • McLeod J.G.
        Synergy between antibody and P2-reactive T cells in experimental allergic neuritis.
        J Neuroimmunol. Mar 1995; 57: 77-84
        • Schmidt B.
        • Toyka K.V.
        • Kiefer R.
        • Full J.
        • Hartung H.P.
        • Pollard J.
        Inflammatory infiltrates in sural nerve biopsies in Guillain–Barre syndrome and chronic inflammatory demyelinating neuropathy.
        Muscle Nerve. Apr 1996; 19: 474-487
        • Prineas J.W.
        Demyelination and remyelination in recurrent idiopathic polyneuropathy. An electron microscope study.
        Acta Neuropathol. 1971; 18: 34-57
        • Sommer C.
        • Koch S.
        • Lammens M.
        • Gabreels-Festen A.
        • Stoll G.
        • Toyka K.V.
        Macrophage clustering as a diagnostic marker in sural nerve biopsies of patients with CIDP.
        Neurology. Dec 27, 2005; 65: 1924-1929
        • Steinhoff U.
        • Kaufmann S.H.
        Specific lysis by CD8+ T cells of Schwann cells expressing Mycobacterium leprae antigens.
        Eur J Immunol. Jun 1988; 18: 969-972
        • Pollard J.D.
        Chronic idiopathic demyelinating polyneuropathy and Schwann cells.
        in: Armati P.J. The biology of Schwann cells. Cambridge University Press, New York2007: 171-184
        • Hahn A.F.
        • Bolton C.F.
        • Pillay N.
        • Chalk C.
        • Benstead T.
        • Bril V.
        • et al.
        Plasma-exchange therapy in chronic inflammatory demyelinating polyneuropathy. A double-blind, sham-controlled, cross-over study.
        Brain. Aug 1996; 119: 1055-1066
        • Dalakas M.C.
        • Engel W.K.
        Immunoglobulin and complement deposits in nerves of patients with chronic relapsing polyneuropathy.
        Arch Neurol. Oct 1980; 37: 637-640
        • Heininger K.
        • Liebert U.G.
        • Toyka K.V.
        • Haneveld F.T.
        • Schwendemann G.
        • Kolb-Bachofen V.
        • et al.
        Chronic inflammatory polyneuropathy. Reduction of nerve conduction velocities in monkeys by systemic passive transfer of immunoglobulin G.
        J Neurol Sci. Oct 1984; 66: 1-14
        • Khalili-Shirazi A.
        • Atkinson P.
        • Gregson N.
        • Hughes R.A.
        Antibody responses to P0 and P2 myelin proteins in Guillain–Barre syndrome and chronic idiopathic demyelinating polyradiculoneuropathy.
        J Neuroimmunol. Jul 1993; 46: 245-251
        • Melendez-Vasquez C.
        • Redford J.
        • Choudhary P.P.
        • Gray I.A.
        • Maitland P.
        • Gregson N.A.
        • et al.
        Immunological investigation of chronic inflammatory demyelinating polyradiculoneuropathy.
        J Neuroimmunol. Mar 1997; 73: 124-134
        • Gabriel C.M.
        • Gregson N.A.
        • Hughes R.A.
        Anti-PMP22 antibodies in patients with inflammatory neuropathy.
        J Neuroimmunol. May 1, 2000; 104: 139-146
        • Ritz M.F.
        • Lechner-Scott J.
        • Scott R.J.
        • Fuhr P.
        • Malik N.
        • Erne B.
        • et al.
        Characterisation of autoantibodies to peripheral myelin protein 22 in patients with hereditary and acquired neuropathies.
        J Neuroimmunol. May 1, 2000; 104: 155-163
        • Yan W.X.
        • Archelos J.J.
        • Hartung H.P.
        • Pollard J.D.
        P0 protein is a target antigen in chronic inflammatory demyelinating polyradiculoneuropathy.
        Ann Neurol. Sep 2001; 50: 286-292
        • Kwa M.S.
        • van Schaik I.N.
        • de Jonge R.R.
        • Brand A.
        • Kalaydjieva L.
        • van BN
        • et al.
        Autoimmunoreactivity to Schwann cells in patients with inflammatory neuropathies.
        Brain. Feb 2003; 126: 361-375
        • Allen D.
        • Giannopoulos K.
        • Gray I.
        • Gregson N.
        • Makowska A.
        • Pritchard J.
        • et al.
        Antibodies to peripheral nerve myelin proteins in chronic inflammatory demyelinating polyradiculoneuropathy.
        J Peripher Nerv Syst. Jun 2005; 10: 174-180
        • Su M.A.
        • Davini D.
        • Cheng P.
        • Giang K.
        • Fan U.
        • DeVoss J.J.
        • et al.
        Defective autoimmune regulator-dependent central tolerance to myelin protein zero is linked to autoimmune peripheral neuropathy.
        J Immunol. May 15, 2012; 188: 4906-4912
        • Nagavalli N.
        • Malik J.
        • Anderson M.
        • Su M.A.
        A new Aire-deficient model of spontaneous autoimmune peripheral neuropathy.
        J Peripher Nerv Syst. 2012; 17: 261-262
        • Ilyas A.A.
        • Mithen F.A.
        • Dalakas M.C.
        • Chen Z.W.
        • Cook S.D.
        Antibodies to acidic glycolipids in Guillain–Barre syndrome and chronic inflammatory demyelinating polyneuropathy.
        J Neurol Sci. Jan 1992; 107: 111-121
        • Yuki N.
        • Tagawa Y.
        • Handa S.
        Autoantibodies to peripheral nerve glycosphingolipids SPG, SLPG, and SGPG in Guillain–Barre syndrome and chronic inflammatory demyelinating polyneuropathy.
        J Neuroimmunol. Oct 1996; 70: 1-6
        • Tsuruta T.
        • Goda N.
        • Umetsu Y.
        • Iwaya N.
        • Kuwahara Y.
        • Hiroaki H.
        (1)H, (1)(3)C, and (1)(5)N resonance assignment of the SPFH domain of human stomatin.
        Biomol NMR Assign. Apr 2012; 6: 23-25
        • Kuwahara M.
        • Suzuki S.
        • Takada K.
        • Kusunoki S.
        Antibodies to LM1 and LM1-containing ganglioside complexes in Guillain–Barre syndrome and chronic inflammatory demyelinating polyneuropathy.
        J Neuroimmunol. Oct 28, 2011; 239: 87-90
        • Rinaldi S.
        • Brennan K.M.
        • Willison H.J.
        Combinatorial glycoarray.
        Methods Mol Biol. 2012; 808: 413-423
        • Rinaldi S.
        • Brennan K.M.
        • Willison H.J.
        Heteromeric glycolipid complexes as modulators of autoantibody and lectin binding.
        Prog Lipid Res. Jan 2010; 49: 87-95
        • Saida K.
        • Saida T.
        • Brown M.J.
        • Silberberg D.H.
        In vivo demyelination induced by intraneural injection of anti-galactocerebroside serum: a morphologic study.
        Am J Pathol. Apr 1979; 95: 99-116
        • Saida T.
        • Saida K.
        • Dorfman S.H.
        • Silberberg D.H.
        • Sumner A.J.
        • Manning M.C.
        • et al.
        Experimental allergic neuritis induced by sensitization with galactocerebroside.
        Science. Jun 8, 1979; 204: 1103-1106
        • Willison H.J.
        Periperal nerve antigens.
        in: Dyck P.J. Thomas P.K. Peripheral neuropathy. 4th ed. Elsevier Saunders, Philadelphia2005: 573-608
        • Susuki K.
        • Rasband M.N.
        • Tohyama K.
        • Koibuchi K.
        • Okamoto S.
        • Funakoshi K.
        • et al.
        Anti-GM1 antibodies cause complement-mediated disruption of sodium channel clusters in peripheral motor nerve fibers.
        J Neurosci. Apr 11, 2007; 27: 3956-3967
      1. David M, Spies JM, Pollard JD, Armati PJ, Zhang G. Anti-ganglioside antibodies induce axonal conduction failure. Submitted for publication.

        • Mathey E.K.
        • Derfuss T.
        • Storch M.K.
        • Williams K.R.
        • Hales K.
        • Woolley D.R.
        • et al.
        Neurofascin as a novel target for autoantibody-mediated axonal injury.
        J Exp Med. Oct 1, 2007; 204: 2363-2372
        • Cifuentes-Diaz C.
        • Dubourg O.
        • Irinopoulou T.
        • Vigny M.
        • Lachkar S.
        • Decker L.
        • et al.
        Nodes of Ranvier and paranodes in chronic acquired neuropathies.
        PLoS One. 2011; 6: e14533
        • Devaux J.J.
        • Odaka M.
        • Yuki N.
        Nodal proteins are target antigens in Guillain–Barre syndrome.
        J Peripher Nerv Syst. Mar 2012; 17: 62-71
        • Derfuss T.
        • Parikh K.
        • Velhin S.
        • Braun M.
        • Mathey E.
        • Krumbholz M.
        • et al.
        Contactin-2/TAG-1-directed autoimmunity is identified in multiple sclerosis patients and mediates gray matter pathology in animals.
        Proc Natl Acad Sci U S A. May 19, 2009; 106: 8302-8307
        • Lancaster E.
        • Huijbers M.G.
        • Bar V.
        • Boronat A.
        • Wong A.
        • Martinez-Hernandez E.
        • et al.
        Investigations of caspr2, an autoantigen of encephalitis and neuromyotonia.
        Ann Neurol. Feb 2011; 69: 303-311
        • Yan W.X.
        • Mathey E.
        • Yiannikas C.
        • Pollard J.D.
        Antineurofascin antibodies are present in patients with peripheral demyelinating neuropathies and mediate changes in nerve conductin in animals.
        J Peripher Nerv Syst. 2010; 15: 288-289
        • Pollard J.D.
        • Armati P.J.
        CIDP — the relevance of recent advances in Schwann cell/axonal neurobiology.
        J Peripher Nerv Syst. Mar 2011; 16: 15-23
        • Dhaunchak A.S.
        • Becker C.
        • Schulman H.
        • De Jr., F.O.
        • Rajasekharan S.
        • Banwell B.
        • et al.
        Implication of perturbed axoglial apparatus in early pediatric multiple sclerosis.
        Ann Neurol. May 2012; 71: 601-613
        • Lonigro A.
        • Devaux J.J.
        Disruption of neurofascin and gliomedin at nodes of Ranvier precedes demyelination in experimental allergic neuritis.
        Brain. Jan 2009; 132: 260-273
        • Hughes R.A.
        • Donofrio P.
        • Bril V.
        • Dalakas M.C.
        • Deng C.
        • Hanna K.
        • 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. Feb 2008; 7: 136-144
        • Mehndiratta M.M.
        • Hughes R.A.
        Corticosteroids for chronic inflammatory demyelinating polyradiculoneuropathy.
        Cochrane Database Syst Rev. 2002; : CD002062
        • Hughes R.A.
        Chronic inflammatory demyelinating polyradiculoneuropathy.
        Ann Neurol. Sep 2001; 50: 281-282
        • Kissel J.T.
        The treatment of chronic inflammatory demyelinating polyradiculoneuropathy.
        Semin Neurol. Jun 2003; 23: 169-180
        • Molenaar D.S.
        • van Doorn P.A.
        • Vermeulen M.
        Pulsed high dose dexamethasone treatment in chronic inflammatory demyelinating polyneuropathy: a pilot study.
        J Neurol Neurosurg Psychiatry. Apr 1997; 62: 388-390
        • Koski C.L.
        Therapy of CIDP and related immune-mediated neuropathies.
        Neurology. Dec 24, 2002; 59: S22-S27
        • Muley S.A.
        • Kelkar P.
        • Parry G.J.
        Treatment of chronic inflammatory demyelinating polyneuropathy with pulsed oral steroids.
        Arch Neurol. Nov 2008; 65: 1460-1464
        • Dyck P.J.
        • Daube J.
        • O'Brien P.
        • Pineda A.
        • Low P.A.
        • Windebank A.J.
        • et al.
        Plasma exchange in chronic inflammatory demyelinating polyradiculoneuropathy.
        N Engl J Med. Feb 20, 1986; 314: 461-465
        • Mehndiratta M.M.
        • Hughes R.A.
        • Agarwal P.
        Plasma exchange for chronic inflammatory demyelinating polyradiculoneuropathy.
        Cochrane Database Syst Rev. 2004; : CD003906
        • Yuill G.M.
        • Swinburn W.R.
        • Liversedge L.A.
        Treatment of polyneuropathy with azathioprine.
        Lancet. Oct 24, 1970; 2: 854-856
        • Dyck P.J.
        • O'Brien P.
        • Swanson C.
        • Low P.
        • Daube J.
        Combined azathioprine and prednisone in chronic inflammatory-demyelinating polyneuropathy.
        Neurology. Aug 1985; 35: 1173-1176
        • Gorson K.C.
        • Amato A.A.
        • Ropper A.H.
        Efficacy of mycophenolate mofetil in patients with chronic immune demyelinating polyneuropathy.
        Neurology. Aug 24, 2004; 63: 715-717
        • Radziwill A.J.
        • Schweikert K.
        • Kuntzer T.
        • Fuhr P.
        • Steck A.J.
        Mycophenolate mofetil for chronic inflammatory demyelinating polyradiculoneuropathy: an open-label study.
        Eur Neurol. 2006; 56: 37-38
        • Gladstone D.E.
        • Prestrud A.A.
        • Brannagan III, T.H.
        High-dose cyclophosphamide results in long-term disease remission with restoration of a normal quality of life in patients with severe refractory chronic inflammatory demyelinating polyneuropathy.
        J Peripher Nerv Syst. Mar 2005; 10: 11-16
        • Barnett M.H.
        • Pollard J.D.
        • Davies L.
        • McLeod J.G.
        Cyclosporin A in resistant chronic inflammatory demyelinating polyradiculoneuropathy.
        Muscle Nerve. Apr 1998; 21: 454-460
        • Benedetti L.
        • Briani C.
        • Franciotta D.
        • Fazio R.
        • Paolasso I.
        • Comi C.
        • et al.
        Rituximab in patients with chronic inflammatory demyelinating polyradiculoneuropathy: a report of 13 cases and review of the literature.
        J Neurol Neurosurg Psychiatry. Mar 2011; 82: 306-308
        • Hartung H.P.
        • Lehmann H.C.
        • Kieseier B.C.
        • Hughes R.A.
        Novel treatment for immune neuropathies on the horizon.
        J Peripher Nerv Syst. Jun 2011; 16: 75-83
        • Marsh E.A.
        • Hirst C.L.
        • Llewelyn J.G.
        • Cossburn M.D.
        • Reilly M.M.
        • Krishnan A.
        • et al.
        Alemtuzumab in the treatment of IVIG-dependent chronic inflammatory demyelinating polyneuropathy.
        J Neurol. Jun 2010; 257: 913-919
        • Donaghy M.
        • Mills K.R.
        • Boniface S.J.
        • Simmons J.
        • Wright I.
        • Gregson N.
        • et al.
        Pure motor demyelinating neuropathy: deterioration after steroid treatment and improvement with intravenous immunoglobulin.
        J Neurol Neurosurg Psychiatry. Jul 1994; 57: 778-783
        • Gorson K.C.
        • van Schaik I.N.
        • Merkies I.S.
        • Lewis R.A.
        • Barohn R.J.
        • Koski C.L.
        • et al.
        Chronic inflammatory demyelinating polyneuropathy disease activity status: recommendations for clinical research standards and use in clinical practice.
        J Peripher Nerv Syst. Dec 2010; 15: 326-333
        • Dalakas M.C.
        Advances in the diagnosis, pathogenesis and treatment of CIDP.
        Nat Rev Neurol. Sep 2011; 7: 507-517