Treatment of Chronic Immune-mediated Neuropathies: Chronic Inflammatory Demyelinating Polyradiculoneuropathy, Multifocal Motor Neuropathy, and the Lewis-Sumner Syndrome

Benson H Sederholm

doi:10.1055/s-0030-1267289

Seminars in Neurology, Inhaltsverzeichnis

Semin Neurol 2010; 30(4): 443-456
DOI: 10.1055/s-0030-1267289

Treatment of Chronic Immune-mediated Neuropathies: Chronic Inflammatory Demyelinating Polyradiculoneuropathy, Multifocal Motor Neuropathy, and the Lewis-Sumner Syndrome

Benson H. Sederholm¹

¹Department of Pharmacy Services, Department of Pharmacotherapy, University of Utah College of Pharmacy, University of Utah Hospital, Salt Lake City, Utah

Abstract

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

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Referenzen

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