ABSTRACT
Treatment of specific immune-mediated neuromuscular disorders involves consideration of many factors including severity of illness, concurrent medical problems, supportive therapies, and immune-modulating therapies. Many immune-modulating therapies are available, including steroids, an increasing number of immunosuppressive drugs, plasmapheresis, and intravenous immunoglobulin (IVIG). Deciding on which immune-modulating therapy involves selecting from those with proven efficacy for a specific disorder and global considerations of which therapies are most appropriate for an individual patient's circumstances. IVIG has become a commonly used therapy as it is well tolerated, easily administered, and is often efficacious with a relatively rapid action. IVIG has become a first-line therapy for several immune-mediated demyelinating polyneuropathies and may play a role in treating exacerbations of myasthenia gravis and selected chronic treatment-refractory cases of Lambert-Eaton myasthenic syndrome, dermatomyositis, and polymyositis.
KEYWORDS
Intravenous immunoglobulin - neuromuscular disorders - Guillain-Barré syndrome - chronic inflammatory demyelinative polyneuropathy - multifocal motor neuropathy - myasthenia gravis - Lambert-Eaton myasthenic syndrome - dermatomyositis - polymyositis - inflammatory myopathy - inclusion body myositis - immunosuppressive therapy - plasmapheresis
REFERENCES
-
1
Darabi K, Abdel-Wahab O, Dzik W H.
Current usage of intravenous immune globulin and the rationale behind it: the Massachusetts General Hospital data and a review of the literature.
Transfusion.
2006;
46
741-753
-
2
Dalakas M C.
Intravenous immunoglobulin in autoimmune neuromuscular diseases.
JAMA.
2004;
291
2367-2375
-
3
Dalakas M C.
The use of intravenous immunoglobulin in the treatment of autoimmune neuromuscular diseases: evidence-based indications and safety profile.
Pharmacol Ther.
2004;
102
177-193
-
4
Gajdos P, Chevret S, Clair B, Tranchant C, Chastang C.
Clinical trial of plasma exchange and high-dose intravenous immunoglobulin in myasthenia gravis. Myasthenia Gravis Clinical Study Group.
Ann Neurol.
1997;
41
789-796
-
5
Bain P G, Motomura M, Newsom-Davis J et al..
Effects of intravenous immunoglobulin on muscle weakness and calcium-channel autoantibodies in the Lambert-Eaton myasthenic syndrome.
Neurology.
1996;
47
678-683
-
6
Dalakas M C, Illa I, Dambrosia J M et al..
A controlled trial of high-dose intravenous immune globulin infusions as treatment for dermatomyositis.
N Engl J Med.
1993;
329
1993-2000
-
7
Cherin P, Pelletier S, Teixeira A et al..
Results and long-term follow-up of intravenous immunoglobulin infusions in chronic, refractory polymyositis: an open study with thirty-five adult patients.
Arthritis Rheum.
2002;
46
467-474
-
8
Dalakas M C, Sonies B, Dambrosia J M, Sekul E, Cupler E, Sivakumar K.
Treatment of inclusion-body myositis with IVIg: a double-blind, placebo-controlled study.
Neurology.
1997;
48
712-716
-
9
Walter M C, Lochmuller H, Toepfer M et al..
High-dose immunoglobulin therapy in sporadic inclusion body myositis: a double-blind, placebo-controlled study.
J Neurol.
2000;
247
22-28
-
10
Griffin J W, Li C Y, Ho T W et al..
Guillain-Barre syndrome in northern China: the spectrum of neuropathological changes in clinically defined cases.
Brain.
1995;
118
577-595
-
11
Griffin J W, Li C Y, Ho T W et al..
Pathology of the motor-sensory axonal Guillain-Barre syndrome.
Ann Neurol.
1996;
39
17-28
-
12
McKhann G M, Cornblath D R, Griffin J W et al..
Acute motor axonal neuropathy: a frequent cause of acute flaccid paralysis in China.
Ann Neurol.
1993;
33
333-342
-
13
Hughes R A, Cornblath D R.
Guillain-Barre syndrome.
Lancet.
2005;
366
1653-1666
-
14
van der Meche F G, Schmitz P I.
A randomized trial comparing intravenous immune globulin and plasma exchange in Guillain-Barre syndrome. Dutch Guillain-Barre Study Group.
N Engl J Med.
1992;
326
1123-1129
-
15
Raphael J C, Chevret S, Harboun M, Jars-Guincestre M C.
Intravenous immune globulins in patients with Guillain-Barre syndrome and contraindications to plasma exchange: 3 days versus 6 days.
J Neurol Neurosurg Psychiatry.
2001;
71
235-238
-
16
Van Koningsveld R, Schmitz P IM, van der Meche F G et al..
Effect of methylprednisolone when added to standard treatment with intravenous immunoglobulin for Guillain-Barre syndrome: randomized trial.
Lancet.
2004;
363
192-196
-
17 Hughes R A, van der Meche F GA. Corticosteroids for GBS. The Cochrane Library, Issue 1. Oxford; Update Software 2003
-
18
Farcas P, Avnun L, Herishanu Y O, Wirguin I.
Efficacy of repeated intravenous immunoglobulin in severe unresponsive Guillain-Barre syndrome.
Lancet.
1997;
350
1747
-
19
Hughes R, Sanders E, Hall S, Atkinson P, Colchester A, Payan P.
Subacute idiopathic demyelinating polyradiculoneuropathy.
Arch Neurol.
1992;
49
612-616
-
20
Saperstein D S, Katz J S, Amato A A, Barohn R J.
Clinical spectrum of chronic acquired demyelinating polyneuropathies.
Muscle Nerve.
2001;
24
311-324
-
21
Dyck P J, O'Brien P, Swanson C, Low P, Daube J.
Combined azathioprine and prednisone in chronic inflammatory-demyelinating polyneuropathy.
Neurology.
1985;
35
1173-1176
-
22
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
136-141
-
23
Dyck P J, Low P A, Windebank A J et al..
Plasma exchange in polyneuropathy associated with monoclonal gammopathy of undetermined significance.
N Engl J Med.
1991;
325
1482-1486
-
24
Hahn A F.
Treatment of chronic inflammatory demyelinating polyneuropathy with intravenous immunoglobulin.
Neurology.
1998;
51
S16-S21
-
25
Mendell J R, Barohn R J, Freimer M L et al..
Randomized controlled trial of IVIg in untreated chronic inflammatory demyelinating polyradiculoneuropathy.
Neurology.
2001;
56
445-449
-
26
Thompson N, Choudhary P, Hughes R A, Quinlivan R M.
A novel trial design to study the effect of intravenous immunoglobulin in chronic inflammatory demyelinating polyradiculoneuropathy.
J Neurol.
1996;
243
280-285
-
27
Vermeulen M, van Doorn P A, Brand A, Strengers P F, Jennekens F G, Busch H F.
Intravenous immunoglobulin treatment in patients with chronic inflammatory demyelinating polyneuropathy: a double blind, placebo controlled study.
J Neurol Neurosurg Psychiatry.
1993;
56
36-39
-
28
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
838-845
-
29
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
491-498
-
30
Nobile-Orazio E, Cappellari A, Priori A.
Multifocal motor neuropathy: current concepts and controversies.
Muscle Nerve.
2005;
31
663-680
-
31
Katz J S, Barohn R J, Kojan S et al..
Axonal multifocal motor neuropathy without conduction block or other features of demyelination.
Neurology.
2002;
58
615-620
-
32
Chaudhry V, Corse A M, Cornblath D R et al..
Multifocal motor neuropathy: response to human immune globulin.
Ann Neurol.
1993;
33
237-242
-
33
Feldman E L, Bromberg M B, Albers J W, Pestronk A.
Immunosuppressive treatment in multifocal motor neuropathy.
Ann Neurol.
1991;
30
397-401
-
34
Pestronk A, Cornblath D R, Ilyas A A et al..
A treatable multifocal motor neuropathy with antibodies to GM1 ganglioside.
Ann Neurol.
1988;
24
73-78
-
35
Azulay J P, Blin O, Pouget J et al..
Intravenous immunoglobulin treatment in patients with motor neuron syndromes associated with anti-GM1 antibodies: a double-blind, placebo-controlled study.
Neurology.
1994;
44
429-432
-
36
Federico P, Zochodne D W, Hahn A F, Brown W F, Feasby T E.
Multifocal motor neuropathy improved by IVIg: randomized, double-blind, placebo-controlled study.
Neurology.
2000;
55
1256-1262
-
37
Leger 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
145-153
-
38
Gajdos P, Tranchant C, Clair B et al..
Treatment of myasthenia gravis exacerbation with intravenous immunoglobulin 1g/kg versus 2g/kg: a randomized double blind clinical trial.
Arch Neurol.
2005;
62
1689-1693
-
39
Illa I.
IVIg in myasthenia gravis, Lambert Eaton myasthenic syndrome and inflammatory myopathies: current status.
J Neurol.
2005;
252
I14-I18
-
40
McEvoy K M, Windebank A J, Daube J R, Low P A.
3.4-Diaminopyridine in the treatment of Lambert-Eaton myasthenic syndrome.
N Engl J Med.
1989;
321
1567-1571
-
41
Dalakas M C, Koffman B, Fujii M, Spector S, Sivakumar K, Cupler E.
A controlled study of intravenous immunoglobulin combined with prednisone in the treatment of IBM.
Neurology.
2001;
56
323-327
-
42
Buchwald B, Ahangari R, Weishaupt A, Toyka K V.
Intravenous immunoglobulins neutralize blocking antibodies in Guillain-Barre syndrome.
Ann Neurol.
2002;
51
673-680
-
43
Kazatchkine M D, Kaveri S V.
Immunomodulation of autoimmune and inflammatory diseases with intravenous immune globulin.
N Engl J Med.
2001;
345
747-755
-
44
Gold R, Dalakas M C, Toyka K V.
Immunotherapy in autoimmune neuromuscular disorders.
Lancet Neurol.
2003;
2
22-32
-
45
Duhem C, Dicato M A, Ries F.
Side-effects of intravenous immune globulins.
Clin Exp Immunol.
1994;
97(suppl 1)
79-83
-
46 Stiehm E. Immunoglobulin therapy in primary antibody deficiency and HIV infection. In: Kazatchkine M, Morell A Intravenous Immunoglobulin: Research and Therapy. Pearl River, NY; Parthenon 1996: 193-203
-
47
Sekul E A, Cupler E J, Dalakas M C.
Aseptic meningitis associated with high-dose intravenous immunoglobulin therapy: frequency and risk factors.
Ann Intern Med.
1994;
121
259-262
-
48
Dalakas M C.
High-dose intravenous immunoglobulin and serum viscosity: risk of precipitating thromboembolic events.
Neurology.
1994;
44
223-226
-
49
Dalakas M C, Clark W M.
Strokes, thromboembolic events, and IVIg: rare incidents blemish an excellent safety record.
Neurology.
2003;
60
1736-1737
Mark A RossM.D.
Department of Neurology, Mayo Clinic Scottsdale
13400 East Shea Boulevard, Scottsdale, AZ 85259
