Inflammatory demyelinating neuropathies.
The primary goal of therapy in patients with the Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is improved strength and functional ability. Improvement in pain, sensory loss, gait disorder, and autonomic instability are other goals of therapy. Patients with very mild symptoms that do not interfere with activities of daily living can be observed for deterioration without treatment. For GBS, standard care includes plasma exchange (PE) or human immune globulin (HIG), both of which have similar efficacy. Supportive care in the intensive care unit may be needed for those patients with severe bulbar or respiratory weakness. We treat most patients with PE, usually performing an exchange every other day for a total of five exchanges. We use HIG in children, if there are antiglycolipid antibodies (eg, anti-GM1 or anti-GQ1b) or if there is a contraindication to PE, such as hemodynamic instability; severe renal, hepatic, or cardiac disease; or poor venous access. For CIDP, there are no guidelines concerning the initial choice of therapy. Corticosteroids, HIG, and PE have all been shown to be effective in prospective, randomized controlled trials, and comparison trials have shown equal efficacy among these three immunomodulating therapies. The choice of therapy depends on several factors including disease severity, concomitant illnesses, side-effect profile, potential drug interactions, venous access, age-related risks, and cost of treatment. In patients with moderate to severe symptoms, treatment with corticosteroids or HIG should be used. We usually use high-dose, intermittent methylprednisolone as the initial drug of choice. We believe intermittent corticosteroids are better than HIG because of their good safety profile, low cost, ease of administration (can be given intravenously or by mouth), and proven efficacy. If there is a major contraindication to corticosteroids, then HIG is offered. PE is less well tolerated and is primarily used as a third choice and only for a few weeks to months to induce initial improvement. Once symptoms are improving, the dose of corticosteroids or HIG should be tapered with the goal of eventual discontinuation depending on patient response. Patients who do not respond to initial therapy, experience adverse effects from the initial immunomodulating agent, or require chronic treatment can be treated with another first-line agent or one of several second-line agents.
Department of Neurology, Washington University School of Medicine, 660 South Euclid Avenue, Campus Box 8111, St. Louis, MO, 63110, USA, lopateg@neuro.wustl.edu.
Glenn
G
Lopate
Alan
A
Pestronk
J Neurol Neurosurg Psychiatry. 2010 Jul;81(7):726-30
20581137
J Clin Apher. 1999;14(3):107-13
10540364
Neurology. 1996 Jul;47(1):98-102
8710133
J Neurol. 1996 Mar;243(3):280-5
8936360
Brain. 1996 Aug;119 ( Pt 4):1067-77
8813271
N Engl J Med. 1991 Nov 21;325(21):1482-6
1658648
J Peripher Nerv Syst. 2005 Mar;10(1):11-6
15703014
Pediatr Neurol. 2001 Mar;24(3):177-82
11301217
Lancet Neurol. 2010 Mar;9(3):245-53
20133204
Arch Neurol. 2008 Nov;65(11):1460-4
19001164
Neurology. 2007 Oct 30;69(18):1802-3
17967996
Neurology. 2001 Feb 27;56(4):445-9
11222785
J Neurol Neurosurg Psychiatry. 2010 Jun;81(6):634-6
20176598
Ann Neurol. 1997 Mar;41(3):298-306
9066350
Neurology. 1985 Aug;35(8):1096-104
4022342
Ann Neurol. 2001 Aug;50(2):195-201
11506402
Brain. 1995 Apr;118 ( Pt 2):359-68
7735878
Ann Neurol. 1994 Dec;36(6):838-45
7998769
Neurology. 1998 Dec;51(6):1735-8
9855536
Ann Neurol. 1981;9 Suppl:134-45
7224612
J Neurol Neurosurg Psychiatry. 1996 Feb;60(2):185-7
8708650
Lancet. 1997 Jan 25;349(9047):225-30
9014908
Lancet. 1984 Dec 8;2(8415):1296-9
6150321
Scand J Infect Dis. 1995;27(3):241-3
8539548
Lancet Neurol. 2008 Feb;7(2):136-44
18178525
N Engl J Med. 1992 Apr 23;326(17):1123-9
1552913
Neurology. 2004 Aug 24;63(4):715-7
15326250
Neurology. 1997 Feb;48(2):321-8
9040714
J Peripher Nerv Syst. 2009 Jun;14(2):118-24
19691534
N Engl J Med. 1986 Feb 20;314(8):461-5
3511382
Eur J Neurol. 2007 Aug;14(8):e1-2
17661987
J Neurol Neurosurg Psychiatry. 1997 Apr;62(4):388-90
9120456
Muscle Nerve. 1998 Apr;21(4):454-60
9533779
Lancet. 1984 Apr 21;1(8382):877-9
6143188
Muscle Nerve. 2001 Jan;24(1):54-8
11150966
J Neurol Neurosurg Psychiatry. 1993 Jan;56(1):36-9
8429321
Neurology. 1996 Jan;46(1):100-3
8559353
Muscle Nerve. 2008 Mar;37(3):406-9
17918749
Pediatrics. 2005 Jul;116(1):8-14
15995024
Ann Neurol. 1987 Dec;22(6):753-61
2893583
J Neurol. 2010 Jun;257(6):913-9
20049473
J Neurol Neurosurg Psychiatry. 2011 Mar;82(3):306-8
20639381
J Neurol Sci. 1998 Jan 21;154(1):66-71
9543324
Arch Neurol. 2005 Feb;62(2):249-54
15710853
Neuromuscul Disord. 2000 Aug;10(6):398-406
10899445
Brain. 1996 Aug;119 ( Pt 4):1055-66
8813270
Ann Neurol. 1982 Feb;11(2):136-41
7041788
Neurology. 1985 Aug;35(8):1173-6
4022350
Ann Neurol. 2000 Mar;47(3):314-21
10716250
Pharmacol Ther. 2004 Jun;102(3):177-93
15246245