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Idiopathic Chronic Immune-Mediated Neuropathies: Chronic Inflammatory Demyelinating Polyradiculoneuropathy and Multifocal Motor Neuropathy

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Acquired Neuromuscular Disorders

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Abstract

Chronic immune-mediated neuropathies are a rather heterogeneous group of disorders of the peripheral nervous system deemed to be caused by an autoimmune attack against peripheral nerves. They include idiopathic disorders where the disease only involve the peripheral nerve such as chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and multifocal motor neuropathy (MMN) or may be the consequence of other disease that causes the production of antibodies against the nerve such as neuropathies associated with monoclonal gammopathy or paraneoplastic neuropathies. The spreading interest on these neuropathies derives from the fact that most affected patients can be treated successfully with immune therapies that differ however from disease to disease highlighting the need for a correct diagnosis. We will here review the clinical, pathogenetic and therapeutic aspects of the two main forms of idiopathic immune-mediated neuropathies, CIDP and MMN.

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References

  1. Vallat J-M, Sommer C, Magy L (2010) Chronic inflammatory demyelinating polyradiculoneuropathy: diagnostic and therapeutic challenges for a treatable condition. Lancet Neurol 9:402–412

    Article  PubMed  Google Scholar 

  2. Hughes RAC, Allen D, Makowska A, Gregson NA (2006) Pathogenesis of chronic inflammatory demyelinating polyradiculoneuropathy. J Peripher Nerv Syst 11:30–46

    Article  PubMed  Google Scholar 

  3. Nobile-Orazio E (2014) Chronic Inflammatory demyelinating polyradiculoneuropathy. Where we are, where we should go. J Peripher Nerv Syst 19:2–13

    Article  CAS  PubMed  Google Scholar 

  4. Kusumi M, Nakashima K, Nakayama H, Takahashi K (1995) Epidemiology of inflammatory neurological and inflammatory neuromuscular diseases in Tattori Prefecture, Japan. Psychiatry Clin Neurosci 49:169–174

    Article  CAS  PubMed  Google Scholar 

  5. Laughlin RS, Dyck PJ, Melton LJ 3rd et al (2009) Incidence and prevalence of CIDP and the associations with diabetes mellitus. Neurology 73:39–45

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  6. Mathey EK, Park SB, Hughes RA et al (2015) Chronic inflammatory demyelinating polyradiculoneuropathy: from pathology to phenotype. J Neurol Neurosurg Psychiatry 86:973–985

    Google Scholar 

  7. Ruts L, Drenthen J, Jacobs BC et al (2010) Distinguishing acute onset CIDP from fluctuating Guillain-Barré syndrome. A prospective study Neurology 74:1680–1686

    CAS  PubMed  Google Scholar 

  8. Lunn MPT, Manji H, Choudhary PP et al (1999) Chronic inflammatory demyelinating polyradiculoneuropathy: a prevalence study in south east England. J Neurol Neurosurg Psychiatry 66:677–680

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  9. Chiò A, Cocito D, Bottacchi E et al (2007) Idiopathic chronic inflammatory demyelinating polyneuropathy: an epidemiological study in Italy. J Neurol Neurosurg Psychiatry 78:1349–1353

    Article  PubMed  PubMed Central  Google Scholar 

  10. Oh SJ, Joy JL, Kuruoglu R (1992) “Chronic sensory demyelinating neuropathy:” chronic inflammatory demyelinating polyneuropathy presenting as a pure sensory neuropathy. J Neurol Neurosurg Psychiatry 55:677–680

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  11. Ayrignac X, Viala K, Morizot Koutlidis R et al (2013) Sensory chronic inflammatory demyelinating polyneuropathy: an under-recognized entity? Muscle Nerve 48:727–732

    Article  PubMed  Google Scholar 

  12. Sinnreich M, Klein CJ, Daube JR et al (2004) Chronic immune sensory polyradiculopathy. A possibly treatable sensory ataxia. Neurology 63:1662–1669

    Article  CAS  PubMed  Google Scholar 

  13. Donaghy M, Mills KR, Boniface SJ et al (1994) Pure motor demyelinating neuropathy: deterioration after steroid treatment and improvement with intravenous immunoglobulin. J Neurol Neurosurg Psychiatry 57:778–783

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  14. Thomas PK, Claus D, Jaspert A et al (1996) Focal upper limb demyelinating neuropathy. Brain 119:765–774

    Article  PubMed  Google Scholar 

  15. Lewis RA, Sumner AJ, Brown MJ, Asbury AK (1982) Multifocal demyelinating neuropathy with persistent conduction block. Neurology 32:958–964

    Article  CAS  PubMed  Google Scholar 

  16. Viala K, Renié L, Maisonobe T et al (2004) Follow-up study and response to treatment in 23 patients with Lewis-Sumner syndrome. Brain 127:2010–2017

    Article  CAS  PubMed  Google Scholar 

  17. Katz JS, Saperstein DS, Gronseth G et al (2000) Distal acquired demyelinating symmetric neuropathy. Neurology 54:615–620

    Article  CAS  PubMed  Google Scholar 

  18. Nobile-Orazio E (2013) Neuropathy and monoclonal gammopathy. In: Said G, Krarup C (eds) Handbook of clinical neurology. Vol. 115 (3rd series); Peripheral nerve disorders. Elsevier BV. Amsterdam. 115, pp 443–459

    Google Scholar 

  19. Hughes RA, Bouche P, Cornblath DR et al (2006) 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. Eur J Neurol 13:326–332

    Article  CAS  PubMed  Google Scholar 

  20. Querol L, Nogales-Gadea G, Rojas-Garcia R et al (2013) Antibody to contactin-1 in chronic inflammatory demyelinating polyneuropathy. Ann Neurol 73:370–380

    Article  CAS  PubMed  Google Scholar 

  21. Querol L, Nogales-Gadea G, Rojas-Garcia R et al (2014) Neurofascin IgG4 antibodies in CIDP associate with disabling tremor and poor response to IVIg. Neurology 82:879–886

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  22. Miura Y, Devaux JJ, Fukami Y et al (2015) Contactin 1 IgG4 associates to chronic inflammatory demyelinating polyneuropathy with sensory ataxia. Brain 138:1484–1491

    Article  PubMed  Google Scholar 

  23. Breiner A, Brannagan TH (2013) Comparison of sensitivity and specificity among 15 criteria for chronic inflammatory demyelinating polyneuropathy. Muscle Nerve 50:40–46

    Article  PubMed  Google Scholar 

  24. Joint Task Force of the EFNS and the PNS (2010) 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. J Peripher Nerv Syst 15:1–9. Erratum in: J Peripher Nerv Syst 15:373

    Google Scholar 

  25. Mehndiratta MM, Hughes RAC (2015) Corticosteroids for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database Syst Rev (1):CD002062

    Google Scholar 

  26. Mehndiratta MM, Hughes RAC, Agarwal P (2012) Plasma exchange for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database Syst Rev (9):CD003906

    Google Scholar 

  27. Eftimov F, Winer JB, Vermeulen M et al (2013) Intravenous immunoglobulin for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database Syst Rev (12):CD001797

    Google Scholar 

  28. Cocito D, Paolasso I, Antonini G et al (2010) A nationwide retrospective analysis on the effect of immune therapies in patients with chronic inflammatory demyelinating polyradiculoneuropathy. Eur J Neurol 17:289–294

    Article  CAS  PubMed  Google Scholar 

  29. Hughes RAC, Bensa S, Willison HJ et al (2001) Randomized controlled trial of intravenous immunoglobulin versus oral prednisolone in chronic inflammatory demyelinating polyradiculoneuropathy. Ann Neurol 50:195–201

    Article  CAS  PubMed  Google Scholar 

  30. Dyck PJ, Litchy WJ, Kratz KM et al (1994) A plasma exchange versus immune globulin infusion trial in chronic inflammatory demyelinating polyradiculoneuropathy. Ann Neurol 36:838–845

    Article  CAS  PubMed  Google Scholar 

  31. Nobile-Orazio E, Cocito D, Jann S et al (2012) Intravenous immunoglobulin versus intravenous methylprednisolone for chronic inflammatory demyelinating polyradiculoneuropathy: a randomised controlled trial. Lancet Neurol 11:493–502

    Article  CAS  PubMed  Google Scholar 

  32. Nobile-Orazio E, Cocito D, Jann S et al (2015) Frequency and time to relapse after discontinuing 6-month therapy with IVIg or pulsed methylprednisolone in CIDP. J Neurol Neurosurg Psychiatry 86:729–734

    Article  PubMed  Google Scholar 

  33. Markvardsen LH, Debost JC, Harbo T et al (2013) Subcutaneous immunoglobulin in responders to intravenous therapy with chronic inflammatory demyelinating polyradiculoneuropathy. Eur J Neurol 20:836–842

    Article  CAS  PubMed  Google Scholar 

  34. Mahdi-Rogers M, Swan AV, van Doorn PA, Hughes RAC (2013) Immunomodulatory treatment other than corticosteroids, immunoglobulin and plasma exchange for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database Syst Rev (6):CD003280

    Google Scholar 

  35. Cocito D, Grimaldi S, Paolasso I et al (2011) Immunosuppressive treatment in refractory chronic inflammatory demyelinating polyradiculoneuropathy. A nationwide retrospective analysis. Eur J Neurol 18:1417–1421

    Article  CAS  PubMed  Google Scholar 

  36. Pestronk A, Cornblath DR, Ilyas AA et al (1988) A treatable multifocal motor neuropathy with antibodies to GM1 ganglioside. Ann Neurol 24:73–78

    Article  CAS  PubMed  Google Scholar 

  37. Nobile-Orazio E, Cappellari A, Priori A (2005) Multifocal motor neuropathy: current concepts and controversies. Muscle Nerve 31:663–680

    Article  PubMed  Google Scholar 

  38. Van Asseldonk JT, Franssen H, Van den Berg-Vos RM et al (2005) Multifocal motor neuropathy. Lancet Neurol 4:309–319

    Article  PubMed  Google Scholar 

  39. Cats EA, van der Pol WL, Piepers S et al (2010) Correlates of outcome and response to IVIg in 88 patients with multifocal motor neuropathy. Neurology 75:818–825

    Article  CAS  PubMed  Google Scholar 

  40. Joint Task Force of the EFNS and the PNS (2010) European Federation of Neurological Societies/Peripheral Nerve Society Guideline on management of multifocal motor neuropathy. Report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society. First revision. J Peripher Nerv Syst 15:295–301

    Article  Google Scholar 

  41. Nobile-Orazio E, Giannotta C, Musset L et al (2014) Sensitivity and predictive value of anti-GM1/galactocerebroside IgM antibodies in multifocal motor neuropathy. J Neurol Neurosurg Psychiatry 85:754–758

    Article  PubMed  Google Scholar 

  42. Riva N, Iannaccone S, Corbo M et al (2011) Motor nerve biopsy: clinical usefulness and histopathological criteria. Ann Neurol 69:197–201

    Article  PubMed  Google Scholar 

  43. Roberts M, Willison HJ, Vincent A, Newsom-Davis J (1995) Multifocal motor neuropathy human sera block distal motor nerve conduction in mice. Ann Neurol 38:111–118

    Article  CAS  PubMed  Google Scholar 

  44. van Schaik IN, van den Berg LH, de Haan R, Vermeulen M (2005) Intravenous immunoglobulin for multifocal motor neuropathy. Cochrane Database Syst Rev (2):CD004429

    Google Scholar 

  45. Van den Berg-Vos RM, Franssen H, Wokke JHJ et al (2002) Multifocal motor neuropathy: long-term clinical and electrophysiological assessment of intravenous immunoglobulin maintenance treatment. Brain 125:1875–1886

    Article  PubMed  Google Scholar 

  46. Terenghi F, Cappellari A, Bersano A et al (2004) How long is IVIg effective in multifocal motor neuropathy? Neurology 62:666–668

    Article  CAS  PubMed  Google Scholar 

  47. Harbo T, Andersen H, Hess A et al (2009) Subcutaneous versus intravenous immunoglobulin in multifocal motor neuropathy: a randomized single – blinded cross-over trial. Eur J Neurol 16:631–638

    Article  CAS  PubMed  Google Scholar 

  48. Eftimov F, Vermeulen M, de Haan RJ et al (2009) Subcutaneous immunoglobulin therapy for multifocal motor neuropathy. J Peripher Nerv Syst 14:93–100

    Article  CAS  PubMed  Google Scholar 

  49. Umapathi T, Hughes R, Nobile-Orazio E, Leger J (2015) Immunosuppressant and immunomodulatory treatments for multifocal motor neuropathy. Cochrane Database Syst (3):CD003217

    Google Scholar 

  50. Piepers S, Van den Berg-Vos R, Van der Pol W-L et al (2007) Mycophenolate mofetil as adjunctive therapy for MMN patients: a randomized, controlled trial. Brain 130:2004–2010

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Medical Biotechnology and Translational Medicine (BIOMETRA), University of Milan, Rozzano, Milan, Italy

    Eduardo Nobile-Orazio MD, PhD, FAAN

  2. 2nd Neurology, Humanitas Clinical and Research Institute, Rozzano, Milan, Italy

    Eduardo Nobile-Orazio MD, PhD, FAAN & Francesca Gallia MD

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  1. Eduardo Nobile-Orazio MD, PhD, FAAN
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  2. Francesca Gallia MD
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Correspondence to Eduardo Nobile-Orazio MD, PhD, FAAN .

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Editors and Affiliations

  1. Neuromuscular Center, IRCCS Fondazione San Camillo Hospit, Venice, Italy

    Corrado Angelini

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Nobile-Orazio, E., Gallia, F. (2016). Idiopathic Chronic Immune-Mediated Neuropathies: Chronic Inflammatory Demyelinating Polyradiculoneuropathy and Multifocal Motor Neuropathy. In: Angelini, C. (eds) Acquired Neuromuscular Disorders. Springer, Cham. https://doi.org/10.1007/978-3-319-29514-5_14

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  • DOI: https://doi.org/10.1007/978-3-319-29514-5_14

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  • Online ISBN: 978-3-319-29514-5

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