Subscribe to RSS
DOI: 10.1055/s-0030-1267289
Treatment of Chronic Immune-mediated Neuropathies: Chronic Inflammatory Demyelinating Polyradiculoneuropathy, Multifocal Motor Neuropathy, and the Lewis-Sumner Syndrome
Publication History
Publication Date:
12 October 2010 (online)
![](https://www.thieme-connect.de/media/sin/201004/lookinside/thumbnails/10.1055-s-0030-1267289-1.jpg)
ABSTRACT
Current treatment approaches for the management of chronic immune-mediated peripheral neuropathies are reviewed, including chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), multifocal motor neuropathy (MMN), and the Lewis-Sumner syndrome (LSS). A summary of existing evidence for commonly used treatment modalities, such as corticosteroids, intravenous immune globulin (IVIG), and plasma exchange is provided. Evidence for the use of additional immunosuppressant and immunomodulatory agents is also reviewed.
KEYWORDS
Chronic inflammatory demyelinating polyradiculoneuropathy - multifocal motor neuropathy - Lewis-Sumner syndrome - intravenous immune globulin - plasma exchange - corticosteroids
REFERENCES
- 1 Eftimov F, Winer J B, Vermeulen M, de Haan R, van Schaik I N. Intravenous immunoglobulin for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database Syst Rev. 2009; (1) CD001797
- 2 Mehndiratta M M, Hughes R A. Corticosteroids for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database Syst Rev. 2002; (1) CD002062
- 3 Mehndiratta M M, Hughes R A, Agarwal P. Plasma exchange for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database Syst Rev. 2004; (3) CD003906
- 4 Gorson K C, Allam G, Ropper A H. Chronic inflammatory demyelinating polyneuropathy: clinical features and response to treatment in 67 consecutive patients with and without a monoclonal gammopathy. Neurology. 1997; 48(2) 321-328
- 5 Dyck P J, Litchy W J, Kratz K M et al.. A plasma exchange versus immune globulin infusion trial in chronic inflammatory demyelinating polyradiculoneuropathy. Ann Neurol. 1994; 36(6) 838-845
- 6 Hughes R, Bensa S, Willison H Inflammatory Neuropathy Cause and Treatment (INCAT) Group et al. Randomized controlled trial of intravenous immunoglobulin versus oral prednisolone in chronic inflammatory demyelinating polyradiculoneuropathy. Ann Neurol. 2001; 50(2) 195-201
- 7 Hughes R A, Bouche P, Cornblath D R 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. Eur J Neurol. 2006; 13(4) 326-332
- 8 Choudhary P P, Hughes R A. Long-term treatment of chronic inflammatory demyelinating polyradiculoneuropathy with plasma exchange or intravenous immunoglobulin. QJM. 1995; 88(7) 493-502
- 9 Kuwabara S, Misawa S, Mori M, Tamura N, Kubota M, Hattori T. Long term prognosis of chronic inflammatory demyelinating polyneuropathy: a five year follow up of 38 cases. J Neurol Neurosurg Psychiatry. 2006; 77(1) 66-70
- 10 McCombe P A, Pollard J D, McLeod J G. Chronic inflammatory demyelinating polyradiculoneuropathy. A clinical and electrophysiological study of 92 cases. Brain. 1987; 110(Pt 6) 1617-1630
- 11 Hughes R A, Swan A V, van Doorn P A. Cytotoxic drugs and interferons for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database Syst Rev. 2004; (4) CD003280
- 12 Barohn R J, Kissel J T, Warmolts J R, Mendell J R. Chronic inflammatory demyelinating polyradiculoneuropathy. Clinical characteristics, course, and recommendations for diagnostic criteria. Arch Neurol. 1989; 46(8) 878-884
- 13 Dyck P J, O'Brien P C, Oviatt K F et al.. Prednisone improves chronic inflammatory demyelinating polyradiculoneuropathy more than no treatment. Ann Neurol. 1982; 11(2) 136-141
- 14 Bromberg M B, Carter O. Corticosteroid use in the treatment of neuromuscular disorders: empirical and evidence-based data. Muscle Nerve. 2004; 30(1) 20-37
- 15 Lopate G, Pestronk A, Al-Lozi M. Treatment of chronic inflammatory demyelinating polyneuropathy with high-dose intermittent intravenous methylprednisolone. Arch Neurol. 2005; 62(2) 249-254
- 16 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. 1997; 62(4) 388-390
- 17 Dalakas M C, Engel W K. Chronic relapsing (dysimmune) polyneuropathy: pathogenesis and treatment. Ann Neurol. 1981; 9(Suppl) 134-145
- 18 Muley S A, Kelkar P, Parry G J. Treatment of chronic inflammatory demyelinating polyneuropathy with pulsed oral steroids. Arch Neurol. 2008; 65(11) 1460-1464
- 19 van Schaik I N, Eftimov F, van Doorn P A 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(3) 245-253
- 20 Hughes R A, Donofrio P, Bril V ICE Study Group 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(2) 136-144
- 21 Vucic S, Black K, Baldassari L E, Tick Chong P S, Dawson K T, Cros D. Long-term effects of intravenous immunoglobulin in CIDP. Clin Neurophysiol. 2007; 118(9) 1980-1984
- 22 Abstracts of the 2003 meeting of the Peripheral Nerve Society. Banff, Canada, July 26–30, 2003. J Peripher Nerv Syst. 2003; (8, Suppl 1) 1-78
- 23 Lee D H, Linker R A, Paulus W, Schneider-Gold C, Chan A, Gold R. Subcutaneous immunoglobulin infusion: a new therapeutic option in chronic inflammatory demyelinating polyneuropathy. Muscle Nerve. 2008; 37(3) 406-409
- 24 Elovaara I, Apostolski S, van Doorn P EFNS et al. EFNS guidelines for the use of intravenous immunoglobulin in treatment of neurological diseases: EFNS task force on the use of intravenous immunoglobulin in treatment of neurological diseases. Eur J Neurol. 2008; 15(9) 893-908
- 25 van Schaik I N, Winer J B, de Haan R, Vermeulen M. Intravenous immunoglobulin for chronic inflammatory demyelinating polyradiculoneuropathy: a systematic review. Lancet Neurol. 2002; 1(8) 491-498
- 26 Bouchard C, Lacroix C, Planté V et al.. Clinicopathologic findings and prognosis of chronic inflammatory demyelinating polyneuropathy. Neurology. 1999; 52(3) 498-503
- 27 Dyck P J, Daube J, O'Brien P et al.. Plasma exchange in chronic inflammatory demyelinating polyradiculoneuropathy. N Engl J Med. 1986; 314(8) 461-465
- 28 Hahn A F, Bolton C F, Pillay N et al.. Plasma-exchange therapy in chronic inflammatory demyelinating polyneuropathy. A double-blind, sham-controlled, cross-over study. Brain. 1996; 119(Pt 4) 1055-1066
- 29 Briellmann R S, Nydegger U E, Sturzenegger M, Fierz L, Hess C W, Hauser S P. Long-term treatment of chronic relapsing inflammatory demyelinating polyradiculoneuropathy: combination of corticosteroids, plasma exchange, and intravenous immunoglobulins. Eur Neurol. 1998; 39(3) 190-191
- 30 Walk D, Li L Y, Parry G J, Day J W. Rapid resolution of quadriplegic CIDP by combined plasmapheresis and IVIg. Neurology. 2004; 62(1) 155-156
- 31 Berger A R, Herskovitz S, Scelsa S. The restoration of IVIg efficacy by plasma exchange in CIDP. Neurology. 1995; 45(8) 1628-1629
- 32 Dyck P J, O'Brien P, Swanson C, Low P, Daube J. Combined azathioprine and prednisone in chronic inflammatory-demyelinating polyneuropathy. Neurology. 1985; 35(8) 1173-1176
- 33 Pentland B. Azathioprine in chronic relapsing idiopathic polyneuropathy. Postgrad Med J. 1980; 56(660) 734-735
- 34 Pentland B, Adams G G, Mawdsley C. Chronic idiopathic polyneuropathy treated with azathioprine. J Neurol Neurosurg Psychiatry. 1982; 45(10) 866-869
- 35 RMC Trial Group . Randomised controlled trial of methotrexate for chronic inflammatory demyelinating polyradiculoneuropathy (RMC trial): a pilot, multicentre study. Lancet Neurol. 2009; 8(2) 158-164
- 36 Fialho D, Chan Y C, Allen D C, Reilly M M, Hughes R A. Treatment of chronic inflammatory demyelinating polyradiculoneuropathy with methotrexate. J Neurol Neurosurg Psychiatry. 2006; 77(4) 544-547
- 37 Gorson K C, Amato A A, Ropper A H. Efficacy of mycophenolate mofetil in patients with chronic immune demyelinating polyneuropathy. Neurology. 2004; 63(4) 715-717
- 38 Benedetti L, Grandis M, Nobbio L et al.. Mycophenolate mofetil in dysimmune neuropathies: a preliminary study. Muscle Nerve. 2004; 29(5) 748-749
- 39 Barnett M H, Pollard J D, Davies L, McLeod J G. Cyclosporin A in resistant chronic inflammatory demyelinating polyradiculoneuropathy. Muscle Nerve. 1998; 21(4) 454-460
- 40 Matsuda M, Hoshi K, Gono T, Morita H, Ikeda S. Cyclosporin A in treatment of refractory patients with chronic inflammatory demyelinating polyradiculoneuropathy. J Neurol Sci. 2004; 224(1-2) 29-35
- 41 Hodgkinson S J, Pollard J D, McLeod J G. Cyclosporin A in the treatment of chronic demyelinating polyradiculoneuropathy. J Neurol Neurosurg Psychiatry. 1990; 53(4) 327-330
- 42 Mahattanakul W, Crawford T O, Griffin J W, Goldstein J M, Cornblath D R. Treatment of chronic inflammatory demyelinating polyneuropathy with cyclosporin-A. J Neurol Neurosurg Psychiatry. 1996; 60(2) 185-187
- 43 Ahlmén J, Andersen O, Hallgren G, Peilot B. Positive effects of tacrolimus in a case of CIDP. Transplant Proc. 1998; 30(8) 4194
- 44 Bilodeau M, Hassoun Z, Brunet D. Demyelinating sensorimotor polyneuropathy associated with the use of sirolimus: a case report. Transplant Proc. 2008; 40(5) 1545-1547
- 45 Good J L, Chehrenama M, Mayer R F, Koski C L. Pulse cyclophosphamide therapy in chronic inflammatory demyelinating polyneuropathy. Neurology. 1998; 51(6) 1735-1738
- 46 Vallat J M, Hahn A F, Léger J M et al.. Interferon beta-1a as an investigational treatment for CIDP. Neurology. 2003; 60(8, Suppl 3) S23-S28
- 47 Hadden R D, Sharrack B, Bensa S, Soudain S E, Hughes R A. Randomized trial of interferon beta-1a in chronic inflammatory demyelinating polyradiculoneuropathy. Neurology. 1999; 53(1) 57-61
- 48 Gorson K C, Ropper A H, Clark B D, Dew III R B, Simovic D, Allam G. Treatment of chronic inflammatory demyelinating polyneuropathy with interferon-alpha 2a. Neurology. 1998; 50(1) 84-87
- 49 Matsuse D, Ochi H, Tashiro K et al.. Exacerbation of chronic inflammatory demyelinating polyradiculoneuropathy during interferonbeta-1b therapy in a patient with childhood-onset multiple sclerosis. Intern Med. 2005; 44(1) 68-72
- 50 Pirko I, Kuntz N L, Patterson M, Keegan B M, Weinshenker B G, Rodriguez M. Contrasting effects of IFNbeta and IVIG in children with central and peripheral demyelination. Neurology. 2003; 60(10) 1697-1699
- 51 Meriggioli M N, Rowin J. Chronic inflammatory demyelinating polyneuropathy after treatment with interferon-alpha. Muscle Nerve. 2000; 23(3) 433-435
- 52 Gono T, Matsuda M, Shimojima Y et al.. Rituximab therapy in chronic inflammatory demyelinating polyradiculoneuropathy with anti-SGPG IgM antibody. J Clin Neurosci. 2006; 13(6) 683-687
- 53 Münch C, Anagnostou P, Meyer R, Haas J. Rituximab in chronic inflammatory demyelinating polyneuropathy associated with diabetes mellitus. J Neurol Sci. 2007; 256(1-2) 100-102
- 54 Gorson K C, Natarajan N, Ropper A H, Weinstein R. Rituximab treatment in patients with IVIg-dependent immune polyneuropathy: a prospective pilot trial. Muscle Nerve. 2007; 35(1) 66-69
- 55 Chin R L, Sherman W H, Sander H W, Hays A P, Latov N. Etanercept (Enbrel) therapy for chronic inflammatory demyelinating polyneuropathy. J Neurol Sci. 2003; 210(1-2) 19-21
- 56 Marsh E A, Hirst C L, Llewelyn J G et al.. Alemtuzumab in the treatment of IVIG-dependent chronic inflammatory demyelinating polyneuropathy. J Neurol. 2010; 257(6) 913-919
- 57 van Schaik I N, Bouche P, Illa I European Federation of Neurological Societies et al. European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of multifocal motor neuropathy. Eur J Neurol. 2006; 13(8) 802-808
- 58 van Schaik I N, van den Berg L H, de Haan R, Vermeulen M. Intravenous immunoglobulin for multifocal motor neuropathy. Cochrane Database Syst Rev. 2005; (2) CD004429
- 59 Léger J M, Chassande B, Musset L, Meininger V, Bouche P, Baumann N. Intravenous immunoglobulin therapy in multifocal motor neuropathy: a double-blind, placebo-controlled study. Brain. 2001; 124(Pt 1) 145-153
- 60 Léger J M, Viala K, Cancalon F et al.. Intravenous immunoglobulin as short- and long-term therapy of multifocal motor neuropathy: a retrospective study of response to IVIg and of its predictive criteria in 40 patients. J Neurol Neurosurg Psychiatry. 2008; 79(1) 93-96
- 61 Terenghi F, Cappellari A, Bersano A, Carpo M, Barbieri S, Nobile-Orazio E. How long is IVIg effective in multifocal motor neuropathy?. Neurology. 2004; 62(4) 666-668
- 62 Vucic S, Black K R, Chong P S, Cros D. Multifocal motor neuropathy: decrease in conduction blocks and reinnervation with long-term IVIg. Neurology. 2004; 63(7) 1264-1269
- 63 Van den Berg L H, Franssen H, Wokke J H. Improvement of multifocal motor neuropathy during long-term weekly treatment with human immunoglobulin. Neurology. 1995; 45(5) 987-988
- 64 Baumann A, Hess C W, Sturzenegger M. IVIg dose increase in multifocal motor neuropathy: a prospective six month follow-up. J Neurol. 2009; 256(4) 608-614
- 65 Van den Berg-Vos R M, Franssen H, Wokke J H, Van Es H W, Van den Berg L H. Multifocal motor neuropathy: diagnostic criteria that predict the response to immunoglobulin treatment. Ann Neurol. 2000; 48(6) 919-926
- 66 Slee M, Selvan A, Donaghy M. Multifocal motor neuropathy: the diagnostic spectrum and response to treatment. Neurology. 2007; 69(17) 1680-1687
- 67 Eftimov F, Vermeulen M, de Haan R J, van den Berg L H, van Schaik I N. Subcutaneous immunoglobulin therapy for multifocal motor neuropathy. J Peripher Nerv Syst. 2009; 14(2) 93-100
- 68 Harbo T, Andersen H, Hess A, Hansen K, Sindrup S H, Jakobsen J. Subcutaneous versus intravenous immunoglobulin in multifocal motor neuropathy: a randomized, single-blinded cross-over trial. Eur J Neurol. 2009; 16(5) 631-638
- 69 Umapathi T, Hughes R A, Nobile-Orazio E, Léger J M. Immunosuppressant and immunomodulatory treatments for multifocal motor neuropathy. Cochrane Database Syst Rev. 2009; (1) CD003217
- 70 Feldman E L, Bromberg M B, Albers J W, Pestronk A. Immunosuppressive treatment in multifocal motor neuropathy. Ann Neurol. 1991; 30(3) 397-401
- 71 Meucci N, Cappellari A, Barbieri S, Scarlato G, Nobile-Orazio E. Long term effect of intravenous immunoglobulins and oral cyclophosphamide in multifocal motor neuropathy. J Neurol Neurosurg Psychiatry. 1997; 63(6) 765-769
- 72 Piepers S, Van den Berg-Vos R, Van der Pol W L, Franssen H, Wokke J, Van den Berg L. Mycophenolate mofetil as adjunctive therapy for MMN patients: a randomized, controlled trial. Brain. 2007; 130(Pt 8) 2004-2010
- 73 Lehmann H C, Hartung H P, Meyer Zu Hörste G, Kieseier B C. Plasma exchange in immune-mediated neuropathies. Curr Opin Neurol. 2008; 21(5) 547-554
- 74 Nobile-Orazio E, Terenghi F, Cocito D, Gallia F, Casellato C. Oral methotrexate as adjunctive therapy in patients with multifocal motor neuropathy on chronic IVIg therapy. J Peripher Nerv Syst. 2009; 14(3) 203-205
- 75 Radziwill A J, Botez S A, Novy J, Kuntzer T. Interferon beta-1a as adjunctive treatment for multifocal motor neuropathy: an open label trial. J Peripher Nerv Syst. 2009; 14(3) 201-202
- 76 Liguori R, Rizzi R, Vetrugno R et al.. Steroid-responsive multifocal demyelinating neuropathy with central involvement. Muscle Nerve. 1999; 22(2) 262-265
- 77 Verschueren A, Azulay J P, Attarian S, Boucraut J, Pellissier J F, Pouget J. Lewis-Sumner syndrome and multifocal motor neuropathy. Muscle Nerve. 2005; 31(1) 88-94
- 78 Saperstein D S, Amato A A, Wolfe G I et al.. Multifocal acquired demyelinating sensory and motor neuropathy: the Lewis-Sumner syndrome. Muscle Nerve. 1999; 22(5) 560-566
- 79 Viala K, Renié L, Maisonobe T et al.. Follow-up study and response to treatment in 23 patients with Lewis-Sumner syndrome. Brain. 2004; 127(Pt 9) 2010-2017
- 80 Rajabally Y A, Chavada G. Lewis-Sumner syndrome of pure upper-limb onset: diagnostic, prognostic, and therapeutic features. Muscle Nerve. 2009; 39(2) 206-220
Benson H SederholmPharmD BCPS
Department of Pharmacy Services, Department of Pharmacotherapy University of Utah College of Pharmacy, University of Utah Hospital
50 N. Medical Drive, Room A-050, Salt Lake City, UT 84132
Email: Benson.Sederholm@hsc.utah.edu