Download PDF
Aktuelle Neurologie 2011; 38(06): 284-291
DOI: 10.1055/s-0031-1287810
Neues in der Neurologie
© Georg Thieme Verlag KG Stuttgart · New York

Neue Entwicklungen bei CIDP und MMN

New Developments in CIDP and MMN
M. Stangel
1   Abteilung Klinische Neuroimmunologie und Neurochemie, Klinik für Neurologie, Medizinische Hochschule Hannover,
,
B. C. Kieseier
2   Neurologische Klinik, Heinrich-Heine Universität Düsseldorf
› Author Affiliations
Further Information

Publication History

Publication Date:
14 October 2011 (online)

Also available at
    Permissions and Reprints

Zusammenfassung

Chronisch immunvermittelte Polyneuropathien, wie die CIDP [chronisch inflammatorische (entzündliche) demyelinisierende Polyneuropathie] und die multifokal motorische Neuropathie (MMN), stellen im klinischen Alltag eine diagnostische und therapeutische Herausforderung dar. Die Heterogenität in der klinischen Präsentation als auch die nur wenigen klinischen Therapiestudien stellen Limitationen im Umgang mit diesen Krankheitsbildern dar. In den letzten Jahren wurden auf diesem Gebiet jedoch Fortschritte erzielt: Einerseits gibt es neue diagnostische Kriterien und Leitfäden, die im klinischen Alltag die differenzialdiagnostische Einordnung erleichtern, andererseits gibt es für die CIDP inzwischen eine klinische Studie höchster Evidenzklasse, die sowohl die Kurz- als auch Langzeitwirksamkeit von intravenösen Immunglobulinen demons­triert. In dem folgenden Übersichtsartikel werden die rezenten diagnostischen und therapeutischen Erkenntnisse für die CIDP als auch die MMN zusammengestellt.

Abstract

Chronic immune-mediated polyneuropathies, such as CIDP (chronic inflammatory demyelinating polyneuropathy) and MMN (multifocal motor neuropathy) represent diagnostic and therapeutic challanges in daily practice. Their clinical heterogeneity as well as the fact that only few therapeutic studies have been conducted represent one of the limitations in handling this disease entity. However, advances have been achieved during recent years in this field: new diagnostic criteria and guidelines have been developed, which should aid the clinician as well as the practitioner in making the right diagnosis. Furthermore, in CIDP a class-I evidence study demonstrated the clinical efficacy of intravenous immunoglobulins for short-term as well as long-term treatment of CIDP. The present review summarises these recent diagnostic as well as therapeutic advances in CIDP and MMN.

Schlüsselwörter

CIDP - MMN - Therapie

Keywords

CIDP - MMN - therapy
 

  • Literatur

  • 1 Austin JH. Recurrent polyneuropathies and their corticosteroid treatment; with five-year observations of a placebo-controlled case treated with corticotrophin, cortisone, and prednisone. Brain 1958; 81: 157-192
    CrossrefPubMedGoogle Scholar
  • 2 Pestronk A, Cornblath DR, Ilyas AA et al. A treatable multifocal motor neuropathy with antibodies to GM1 ganglioside. Ann Neurol 1988; 24: 73-78
    CrossrefPubMedGoogle Scholar
  • 3 van Asseldonk JT, Franssen H, Van den Berg-Vos RM et al. Multifocal motor neuropathy. Lancet Neurol 2005; 4: 309-319
    CrossrefPubMedGoogle Scholar
  • 4 Rotta FT, Sussman AT, Bradley WG et al. The spectrum of chronic inflammatory demyelinating polyneuropathy. J Neurol Sci 2000; 173: 129-139
    CrossrefPubMedGoogle Scholar
  • 5 Lunn MP, Manji H, Choudhary PP et al. Chronic inflammatory demyelinating polyradiculoneuropathy: a prevalence study in south east England. J Neurol Neurosurg Psychiatry 1999; 66: 677-680
    CrossrefPubMedGoogle Scholar
  • 6 McLeod JG, Pollard JD, Macaskill P et al. Prevalence of chronic inflammatory demyelinating polyneuropathy in New South Wales, Australia. Ann Neurol 1999; 46: 910-913
    CrossrefPubMedGoogle Scholar
  • 7 Koski CL, Baumgarten M, Magder LS et al. Derivation and validation of diagnostic criteria for chronic inflammatory demyelinating polyneuropathy. J Neurol Sci 2009; 277: 1-8
    CrossrefPubMedGoogle Scholar
  • 8 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: 356-363
    CrossrefPubMedGoogle Scholar
  • 9 Saperstein DS, Katz JS, Amato AA et al. Clinical spectrum of chronic acquired demyelinating polyneuropathies. Muscle Nerve 2001; 24: 311-324
    CrossrefPubMedGoogle Scholar
  • 10 Ad Hoc Subcommittee of the American Academy of Neurology AIDS Task Force . Research criteria for diagnosis of chronic inflammatory demye­linating polyneuropathy (CIDP). Report from an Ad Hoc Subcommittee of the American Academy of Neurology AIDS Task Force. Neurology 1991; 41: 617-618
    CrossrefPubMedGoogle Scholar
  • 11 Midroni G, de Tilly LN, Gray B et al. MRI of the cauda equina in CIDP: clinical correlations. J Neurol Sci 1999; 170: 36-44
    CrossrefPubMedGoogle Scholar
  • 12 Eurelings M, Notermans NC, Franssen H et al. MRI of the brachial plexus in polyneuropathy associated with monoclonal gammopathy. Muscle Nerve 2001; 24: 1312-1318
    CrossrefPubMedGoogle Scholar
  • 13 Vallat JM, Sommer C, Magy L. Chronic inflammatory demyelinating polyradiculoneuropathy: diagnostic and therapeutic challenges for a treatable condition. Lancet Neurol 2010; 9: 402-412
    CrossrefPubMedGoogle Scholar
  • 14 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: 289-294
    CrossrefPubMedGoogle Scholar
  • 15 Dyck PJ, O’Brien PC, Oviatt KF et al. Prednisone improves chronic inflammatory demyelinating polyradiculoneuropathy more than no treatment. Ann Neurol 1982; 11: 136-141
    CrossrefPubMedGoogle Scholar
  • 16 Lopate G, Pestronk A, Al Lozi M. Treatment of chronic inflammatory demyelinating polyneuropathy with high-dose intermittent intravenous methylprednisolone. Arch Neurol 2005; 62: 249-254
    CrossrefPubMedGoogle Scholar
  • 17 Muley SA, Kelkar P, Parry GJ. Treatment of chronic inflammatory demyelinating polyneuropathy with pulsed oral steroids. Arch Neurol 2008; 65: 1460-1464
    CrossrefPubMedGoogle Scholar
  • 18 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: 245-253
    CrossrefPubMedGoogle Scholar
  • 19 Eftimov F, Winer JB, Vermeulen M et al. Intravenous immunoglobulin for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database Syst Rev 2009; CD001797
    PubMedGoogle Scholar
  • 20 Bril V, Katzberg H, Donofrio P et al. Electrophysiology in chronic inflammatory demyelinating polyneuropathy with IGIV. Muscle Nerve 2009; 39: 448-455
    CrossrefPubMedGoogle Scholar
  • 21 Merkies ISJ, Bril V, Dalakas MC et al. Health-related quality-of-life improvements in CIDP with immune globulin IV 10%. The ICE study. Neurology 2009; 72: 1337-1344
    CrossrefPubMedGoogle Scholar
  • 22 Rajabally YA, Seow H, Wilson P. Dose of intravenous immunoglobulins in chronic inflammatory demyelinating polyneuropathy. J Periph Nerv Syst 2006; 11: 325-329
    CrossrefPubMedGoogle Scholar
  • 23 Köller H, Schroeter M, Feischen H et al. Subcutaneous self-infusions of immunoglobulins as a potential therapeutic regimen in immune-mediated neuropathies. J Neurol 2006; 253: 1505-1506
    CrossrefPubMedGoogle Scholar
  • 24 Lee H-H, Linker RA, Paulus W et al. Subcutaneous immunoglobulin infusion: a new therapeutic option in chronic inflammatory demyelinating polyneuropathy. Muscle Nerve 2008; 37: 406-409
    CrossrefPubMedGoogle Scholar
  • 25 Dyck PJ, Daube J, O’Brien P et al. Plasma exchange in chronic inflammatory demyelinating polyradiculoneuropathy. N Engl J Med 1986; 314: 461-465
    CrossrefPubMedGoogle Scholar
  • 26 Hahn AF, Bolton CF, Pillay N et al. Plasma-exchange in chronic inflammatory demyelinating polyneuropathy. A double-blind, sham-controlled, cross-over study. Brain 1996; 119: 1055-1066
    CrossrefPubMedGoogle Scholar
  • 27 French cooperative group on plasma exchange in Guillain-Barré syndrome . Appropriate number of plasma exchanges in Guillain-Barré syndrome. . Ann Neurol 1997; 41: 298-306
    CrossrefPubMedGoogle Scholar
  • 28 Meyer zu Hörste G, Hartung HP, Kieseier BC. From bench to bedside – experimental rationale for immune-specific therapies in the inflamed peripheral nerve. Nat Clin Pract Neurol 2007; 3: 198-211
    PubMedGoogle Scholar
  • 29 Kieseier BC, Wiendl H, Hartung HP. The inflamed peripheral nervous system: update on immune therapies. Curr Opin Neurol 2006; 19: 433-436
    CrossrefPubMedGoogle Scholar
  • 30 Dyck PJ, O’Brien P, Swanson C et al. Combined azathioprine and prednisone in chronic inflammatory-demyelinating polyneuropathy. Neurology 1985; 35: 1173-1176
    CrossrefPubMedGoogle Scholar
  • 31 Fialho D, Chan YC, Allen DC et al. Treatment of chronic inflammatory demyelinating polyradiculoneuropathy with methotrexate. J Neurol Neurosurg Psychiatry 2006; 77: 544-547
    CrossrefPubMedGoogle Scholar
  • 32 RMC Trial Group. Randomised controlled trial of methotrexate for chronic inflammatory demyelinating polyradiculoneuropathy (RMC trial): a pilot, multicentre study. Lancet Neurol 2009; 8: 158-164
    CrossrefPubMedGoogle Scholar
  • 33 Matsuda M, Hoshi K, Gono T et al. Cyclosporin A in treatment of refractory patients with chronic inflammatory demyelinating polyradiculoneuropathy. J Neurol Sci 2004; 224: 29-35
    CrossrefPubMedGoogle Scholar
  • 34 Odaka M, Tatsumoto M, Susuki K et al. Intractable chronic inflammatory demyelinating polyneuropathy treated successfully with cyclosporin. J Neurol Neurosurg Psychiatry 2005; 76: 1115-1120
    CrossrefPubMedGoogle Scholar
  • 35 Bedi G, Brown A, Tong T et al. Chronic inflammatory demyelinating polyneuropathy responsive to mycophenolate mofetil therapy. J Neurol Neurosurg Psychiatry 2010; 81: 634-636
    CrossrefPubMedGoogle Scholar
  • 36 Radziwill AJ, Schweikert K, Kuntzer T et al. Mycophenolate mofetil for chronic inflammatory demyelinating polyradiculoneuropathy: An open-label study. Eur Neurol 2006; 56: 37-38
    CrossrefPubMedGoogle Scholar
  • 37 Good JL, Chehrenama M, Mayer RF et al. Pulse cyclophosphamide therapy in chronic inflammatory demyelinating polyneuropathy. Neurology 1998; 51: 1735-1738
    CrossrefPubMedGoogle Scholar
  • 38 Brannagan III TH, Pradhan A, Heiman-Patterson T et al. High-dose cyclophosphamide without stem-cell rescue for refractory CIDP. Neurology 2002; 58: 1856-1858
    CrossrefPubMedGoogle Scholar
  • 39 Gladstone DE, Prestrud AA, Brannagan TH. III. 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 2005; 10: 11-16
    CrossrefPubMedGoogle Scholar
  • 40 Hadden RD, Sharrack B, Bensa S et al. Randomized trial of interferon beta-1a in chronic inflammatory demyelinating polyradiculoneuropathy. Neurology 1999; 53: 57-61
    CrossrefPubMedGoogle Scholar
  • 41 Hughes RA, Gorson KC, Cros D et al. Intramuscular interferon beta-1a in chronic inflammatory demyelinating polyradiculoneuropathy. Neurology 2010; 74: 651-657
    CrossrefPubMedGoogle Scholar
  • 42 Munch C, Anagnostou P, Meyer R et al. Rituximab in chronic inflammatory demyelinating polyneuropathy associated with diabetes mellitus. J Neurol Sci 2007; 256: 100-102
    CrossrefPubMedGoogle Scholar
  • 43 Gorson KC, Natarajan N, Ropper AH et al. Rituximab treatment in patients with IVIg-dependent immune polyneuropathy: a prospective pilot trial. Muscle Nerve 2007; 35: 66-69
    CrossrefPubMedGoogle Scholar
  • 44 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: 306-308
    CrossrefPubMedGoogle Scholar
  • 45 Wolf C, Menge T, Stenner MP et al. Natalizumab treatment in a patient with chronic inflammatory demyelinating polyneuropathy. Arch Neurol 2010; 67: 881-883
    CrossrefPubMedGoogle Scholar
  • 46 Hirst C, Raasch S, Llewelyn G et al. Remission of chronic inflammatory demyelinating polyneuropathy after alemtuzumab (Campath 1H). J Neurol Neurosurg Psychiatry 2006; 77: 800-802
    PubMedGoogle Scholar
  • 47 Marsh EA, Hirst CL, Llewelyn JG et al. Alemtuzumab in the treatment of IVIG-dependent chronic inflammatory demyelinating polyneuropathy. J Neurol 2010; 257: 913-919
    CrossrefPubMedGoogle Scholar
  • 48 Vermeulen M, van Oers MH. Relapse of chronic inflammatory demyelinating polyneuropathy 5 years after autologous stem cell transplantation. J Neurol Neurosurg Psychiatry 2007; 78: 1154
    CrossrefPubMedGoogle Scholar
  • 49 Mahdi-Rogers M, Kazmi M, Ferner R et al. Autologous peripheral blood stem cell transplantation for chronic acquired demyelinating neuropathy. J Peripher Nerv Syst 2009; 14: 118-124
    CrossrefPubMedGoogle Scholar
  • 50 Oyama Y, Sufit R, Loh Y et al. Nonmyeloablative autologous hematopoietic stem celltransplantation for refractory CIDP. Neurology 2007; 69: 1802-1803
    CrossrefPubMedGoogle Scholar
  • 51 Chad DA, Hammer K, Sargent J. Slow resolution of multifocal weakness and fasciculation: a reversible motor neuron syndrome. Neurology 1986; 36: 1260-1263
    CrossrefPubMedGoogle Scholar
  • 52 Roth G, Rohr J, Magistris MR et al. Motor neuropathy with proximal multifocal persistent conduction block, fasciculations and myokymia. Evolution to tetraplegia. Eur Neurol 1986; 25: 416-423
    CrossrefPubMedGoogle Scholar
  • 53 Nobile-Orazio E. Multifocal motor neuropathy. J Neuroimmunol 2001; 115: 4-18
    CrossrefPubMedGoogle Scholar
  • 54 Cats EA, van der Pol WL, Piepers S et al. Correlates of outcome and response to IVIg in 88 patients with multifocal motor neuropathy. Neurology 2010; 75: 818-825
    CrossrefPubMedGoogle Scholar
  • 55 van Schaik IN, Bossuyt PM, Brand A et al. Diagnostic value of GM1 antibodies in motor neuron disorders and neuropathies: a meta-analysis. Neurology 1995; 45: 1570-1577
    CrossrefPubMedGoogle Scholar
  • 56 Cats EA, Jacobs BC, Yuki N et al. Multifocal motor neuropathy: association of anti-GM1 IgM antibodies with clinical features. Neurology 2010; 75: 1961-1967
    CrossrefPubMedGoogle Scholar
  • 57 Joint Task Force of the EFNS and the PNS . 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 Periph Nerv Syst 2010; 295-301
    PubMedGoogle Scholar
  • 58 Feldman EL, Bromberg MB, Albers JW et al. Immunosuppressive treatment in multifocal motor neuropathy. Ann Neurol 1991; 30: 397-401
    CrossrefPubMedGoogle Scholar
  • 59 Carpo M, Cappellari A, Mora G et al. Deterioration of multifocal motor neuropathy after plasma exchange. Neurology 1998; 50: 1480-1482
    CrossrefPubMedGoogle Scholar
  • 60 van Schaik IN, van den Berg LH, de Haan R et al. Intravenous immunoglobulin for multifocal motor neuropathy. Cochrane Database Syst Rev 2005; CD004429
    PubMedGoogle Scholar
  • 61 Terenghi F, Cappellari A, Bersano A et al. How long is IVIg effective in multifocal motor neuropathy?. Neurology 2004; 62: 666-668
    CrossrefPubMedGoogle Scholar
  • 62 Eftimov F, Vermeulen M, de Haan RJ et al. Subcutaneous immunoglobulin therapy for multifocal motor neuropathy. J Peripher Nerv Syst 2009; 14: 93-100
    CrossrefPubMedGoogle Scholar
  • 63 Harbo T, Andersen H, Hess A et al. Subcutaneous versus intravenous immunoglobulin in multifocal motor neuropathy: a randomized, single-blinded cross-over trial. Eur J Neurol 2009; 16: 631-638
    CrossrefPubMedGoogle Scholar
  • 64 Umapathi T, Hughes RA, Nobile-Orazio E et al. Immunosuppressant and immunomodulatory treatments for multifocal motor neuropathy. Cochrane Database Syst Rev 2009; CD003217
    PubMedGoogle Scholar
  • 65 Piepers S, Van den Berg-Vos R, Van der Pol W-L et al. Mycophenolate mofetil as adjunctive therapy for MMN patients: a randomized controlled trial. Brain 2007; 130: 2004-2010
    CrossrefPubMedGoogle Scholar
  • 66 Nobile-Orazio E, Terenghi F, Cocito D et al. Oral methotrexate as adjunctive therapy in patients with multifocal motor neuropathy on chronic IVIg therapy. J Peripher Nerv Syst 2009; 14: 203-205
    CrossrefPubMedGoogle Scholar
  • 67 Radziwill AJ, Botez SA, Novy J et al. Interferon beta-1a as adjunctive treatment for multifocal motor neuropathy: an open label trial. J Peripher Nerv Syst 2009; 14: 201-202
    CrossrefPubMedGoogle Scholar
  • 68 Stieglbauer K, Topakian R, Hinterberger G et al. Beneficial effect of rituximab monotherapy in multifocal motor neuropathy. Neuromusc Disord 2009; 19: 473-475
    CrossrefPubMedGoogle Scholar
  • 69 Chaudhry V, Cornblath DR. An open-label trial of rituximab (Rituxan(R)) in multifocal motor neuropathy. J Peripher Nerv Syst 2010; 15: 196-201
    CrossrefPubMedGoogle Scholar
  • 70 Hughes RAC, Bensa S, Willison H et al. Randomized controlled trial of intravenous immunoglobulin (IVIg) versus oral prednisolone in chronic inflammatory demyelinating polyradiculoneuropathy. Ann Neurol 2001; 50: 195-201
    CrossrefPubMedGoogle Scholar
  • 71 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: 136-144
    CrossrefPubMedGoogle Scholar