title: Chronic inflammatory demyelinating polyneuropathy.
abstract: Chronic Inflammatory polyneuropathies are an important group of neuromuscular disorders that present chronically and progress over more than 8 weeks, being referred to as chronic inflammatory demyelinating polyneuropathy (CIDP). Despite tremendous progress in elucidating disease pathogenesis, the exact triggering event remains unknown. Our knowledge regarding diagnosis and management of CIDP and its variants continues to expand, resulting in improved opportunities for identification and treatment. Most clinical neurologists will be involved in the management of patients with these disorders, and should be familiar with available therapies for CIDP. We review the distinctive clinical, laboratory, and electro-diagnostic features that aid in diagnosis. We emphasize the importance of clinical patterns that define treatment responsiveness and the most appropriate therapies in order to improve prognosis.
authors.affiliation: Department of Neurology, University of Kansas Medical Center, 3599 Rainbow Blvd., Mail Stop 2012, Kansas City, KS, 66160, USA, mdimachkie@kumc.edu.
authors.forename: Mazen M
authors.initials: MM
authors.lastname: Dimachkie
authors.forename: Richard J
authors.initials: RJ
authors.lastname: Barohn
references.citation: Neurology. 2010 Feb 23;74(8):651-7
references.pmid: 20177118
references.citation: J Peripher Nerv Syst. 2005 Jun;10(2):174-80
references.pmid: 15958128
references.citation: Proc Natl Acad Sci U S A. 2009 Mar 24;106(12):4788-92
references.pmid: 19261857
references.citation: Muscle Nerve. 2001 Mar;24(3):311-24
references.pmid: 11353415
references.citation: Neurology. 1998 Jan;50(1):84-7
references.pmid: 9443462
references.citation: Eur J Neurol. 2010 Mar;17(3):356-63
references.pmid: 20456730
references.citation: J Neurol Neurosurg Psychiatry. 1990 Apr;53(4):327-30
references.pmid: 2341846
references.citation: J Neurol Neurosurg Psychiatry. 2009 Jan;80(1):70-3
references.pmid: 18768571
references.citation: Neurology. 2003 Apr 1;60(8 Suppl 3):S23-8
references.pmid: 12707419
references.citation: Neurology. 2001 Jan 9;56(1):94-6
references.pmid: 11148242
references.citation: Muscle Nerve. 2010 Oct;42(4):492-7
references.pmid: 20665514
references.citation: J Neurol Sci. 1994 Dec 20;127(2):170-8
references.pmid: 7707076
references.citation: J Neurol Neurosurg Psychiatry. 1995 May;58(5):638-9
references.pmid: 7745420
references.citation: Arch Neurol. 2008 Nov;65(11):1460-4
references.pmid: 19001164
references.citation: Neurology. 2002 Nov 26;59(10):1526-32
references.pmid: 12451191
references.citation: Brain. 2001 Dec;124(Pt 12):2427-38
references.pmid: 11701597
references.citation: Neurology. 2007 Oct 30;69(18):1802-3
references.pmid: 17967996
references.citation: Neurology. 2001 Feb 27;56(4):445-9
references.pmid: 11222785
references.citation: Clin Neuropharmacol. 2010 May;33(3):161-2
references.pmid: 20502136
references.citation: J Peripher Nerv Syst. 2008 Mar;13(1):54-63
references.pmid: 18346231
references.citation: Brain. 1996 Aug;119 ( Pt 4):1067-77
references.pmid: 8813271
references.citation: Muscle Nerve. 2009 Apr;39(4):439-47
references.pmid: 19229876
references.citation: Muscle Nerve. 2004 Apr;29(4):565-74
references.pmid: 15052622
references.citation: Blood. 2003 Apr 1;101(7):2496-506
references.pmid: 12456500
references.citation: Muscle Nerve. 1999 May;22(5):560-6
references.pmid: 10331353
references.citation: J Neurol Neurosurg Psychiatry. 2008 Nov;79(11):1255-7
references.pmid: 18469028
references.citation: PLoS One. 2010 Mar 19;5(3):e9782
references.pmid: 20333310
references.citation: Muscle Nerve. 2004 Oct;30(4):501-4
references.pmid: 15372442
references.citation: Eur J Neurol. 2006 Apr;13(4):326-32
references.pmid: 16643309
references.citation: Neurology. 2006 Jan 10;66(1):105-7
references.pmid: 16401856
references.citation: J Neuroimmunol. 1988 Jun;18(3):231-44
references.pmid: 2452830
references.citation: J Peripher Nerv Syst. 2010 Mar;15(1):50-6
references.pmid: 20433605
references.citation: Arch Neurol. 1978 Aug;35(8):509-16
references.pmid: 666609
references.citation: Neurology. 1999 Feb;52(3):498-503
references.pmid: 10025777
references.citation: Neurology. 1999 Oct 12;53(6):1364-5
references.pmid: 10522905
references.citation: Muscle Nerve. 1994 May;17(5):528-32
references.pmid: 7512691
references.citation: Arch Neurol. 1989 Aug;46(8):878-84
references.pmid: 2757528
references.citation: Ann Neurol. 2001 Aug;50(2):195-201
references.pmid: 11506402
references.citation: Muscle Nerve. 2008 Mar;37(3):281-92
references.pmid: 18041052
references.citation: J Clin Invest. 1987 Nov;80(5):1492-7
references.pmid: 3680509
references.citation: Int J Neurosci. 2013 Aug;123(8):563-7
references.pmid: 23461611
references.citation: Ann Neurol. 1996 May;39(5):625-35
references.pmid: 8619548
references.citation: Neurology. 1991 May;41(5):617-8
references.pmid: 2027473
references.citation: Neurology. 2006 Dec 12;67(11):2076-9
references.pmid: 17159126
references.citation: Ann Neurol. 1995 Nov;38(5):828-31
references.pmid: 7486876
references.citation: Muscle Nerve. 1991 Oct;14(10):968-76
references.pmid: 1944409
references.citation: J Neurol Sci. 2009 Feb 15;277(1-2):1-8
references.pmid: 19091330
references.citation: Muscle Nerve. 1999 Jun;22(6):758-65
references.pmid: 10366230
references.citation: Neurology. 1998 Dec;51(6):1735-8
references.pmid: 9855536
references.citation: Neurology. 2009 Jan 13;72(2):177-84
references.pmid: 19056667
references.citation: Neurology. 2000 Feb 8;54(3):615-20
references.pmid: 10680792
references.citation: Neurology. 2009 Jul 7;73(1):39-45
references.pmid: 19564582
references.citation: Arch Neurol. 1992 Jun;49(6):612-6
references.pmid: 1317700
references.citation: Neurology. 1993 Aug;43(8):1513-8
references.pmid: 8394521
references.citation: Muscle Nerve. 2005 Jan;31(1):88-94
references.pmid: 15536613
references.citation: Neurology. 1982 Sep;32(9):958-64
references.pmid: 7202168
references.citation: Neurology. 2003 Dec 9;61(11):1507-12
references.pmid: 14663033
references.citation: J Neurol. 1995 Mar;242(4):252-3
references.pmid: 7798127
references.citation: Neurology. 2010 May 25;74(21):1680-6
references.pmid: 20427754
references.citation: Eur J Neurol. 2010 Jul;17(7):939-44
references.pmid: 20158514
references.citation: Lancet Neurol. 2008 Feb;7(2):136-44
references.pmid: 18178525
references.citation: Neurology. 2004 Aug 24;63(4):715-7
references.pmid: 15326250
references.citation: Lancet Neurol. 2009 Feb;8(2):158-64
references.pmid: 19136303
references.citation: Neurology. 1997 Feb;48(2):321-8
references.pmid: 9040714
references.citation: Ann Neurol. 2000 Jun;47(6):765-75
references.pmid: 10852542
references.citation: Semin Neurol. 1998;18(1):7-18
references.pmid: 9562663
references.citation: Brain. 1958 Jun;81(2):157-92
references.pmid: 13572689
references.citation: J Peripher Nerv Syst. 2009 Jun;14(2):118-24
references.pmid: 19691534
references.citation: J Neurol Neurosurg Psychiatry. 2006 Apr;77(4):544-7
references.pmid: 16543541
references.citation: Am J Med. 1990 Oct;89(4):531-4
references.pmid: 2220888
references.citation: Neurology. 1987 Aug;37(8):1291-4
references.pmid: 3614649
references.citation: J Neurol Neurosurg Psychiatry. 1998 Jan;64(1):84-9
references.pmid: 9436733
references.citation: N Engl J Med. 1986 Feb 20;314(8):461-5
references.pmid: 3511382
references.citation: Neurology. 1989 Jan;39(1):106-10
references.pmid: 2909898
references.citation: J Neuroimmunol. 1999 Mar 1;95(1-2):190-4
references.pmid: 10229130
references.citation: Brain. 1996 Jun;119 ( Pt 3):765-74
references.pmid: 8673489
references.citation: Muscle Nerve. 1998 Nov;21(11):1390-7
references.pmid: 9771661
references.citation: Muscle Nerve. 1998 Apr;21(4):454-60
references.pmid: 9533779
references.citation: Arch Neurol. 2003 Jan;60(1):42-6
references.pmid: 12533087
references.citation: J Neurol. 2001 Sep;248(9):772-7
references.pmid: 11596782
references.citation: Brain. 2004 Jan;127(Pt 1):193-202
references.pmid: 14607795
references.citation: J Peripher Nerv Syst. 2005 Mar;10(1):11-6
references.pmid: 15703014
references.citation: Brain. 1987 Dec;110 ( Pt 6):1617-30
references.pmid: 3427403
references.citation: Lancet. 1996 Mar 9;347(9002):702
references.pmid: 8596427
references.citation: J Neurol Sci. 1996 Oct;142(1-2):59-64
references.pmid: 8902721
references.citation: Muscle Nerve. 2008 Mar;37(3):406-9
references.pmid: 17918749
references.citation: Mayo Clin Proc. 1975 Nov;50(11):621-37
references.pmid: 1186294
references.citation: J Neurol Neurosurg Psychiatry. 2011 Mar;82(3):306-8
references.pmid: 20639381
references.citation: J Neurol. 2010 Jun;257(6):913-9
references.pmid: 20049473
references.citation: J Peripher Nerv Syst. 2006 Mar;11(1):53-60
references.pmid: 16519782
references.citation: Neurology. 2005 Dec 27;65(12):1924-9
references.pmid: 16380614
references.citation: Arch Neurol. 2005 Feb;62(2):249-54
references.pmid: 15710853
references.citation: Brain. 1996 Aug;119 ( Pt 4):1055-66
references.pmid: 8813270
references.citation: Science. 2006 Aug 4;313(5787):670-3
references.pmid: 16888140
references.citation: N Engl J Med. 2005 Mar 31;352(13):1343-56
references.pmid: 15800230
references.citation: Mayo Clin Proc. 1982 Apr;57(4):239-46
references.pmid: 7070119
references.citation: J Neurol. 2002 Jun;249(6):777-9
references.pmid: 12173577
references.citation: J Neurol Sci. 2001 Apr 1;185(2):119-22
references.pmid: 11311292
references.citation: Semin Neurol. 1994 Jun;14(2):123-30
references.pmid: 7984827
references.citation: J Peripher Nerv Syst. 1997;2(4):362-9
references.pmid: 10975745
references.citation: Neurology. 2002 Jun 25;58(12):1856-8
references.pmid: 12084892
references.citation: Clin Neuropathol. 1993 Nov-Dec;12(6):343-52
references.pmid: 8287628
references.citation: J Neurol Sci. 2000 Feb 15;173(2):129-39
references.pmid: 10675657
references.citation: Neurology. 1980 Aug;30(8):864-7
references.pmid: 6251407
references.citation: Neurology. 1984 Oct;34(10):1375-7
references.pmid: 6541313
references.citation: Eur J Neurol. 2007 Jun;14(6):638-43
references.pmid: 17539941
references.citation: Ann Neurol. 1982 Feb;11(2):136-41
references.pmid: 7041788
references.citation: Brain. 2002 Apr;125(Pt 4):823-34
references.pmid: 11912115
references.citation: Neurology. 1985 Aug;35(8):1173-6
references.pmid: 4022350
references.citation: Muscle Nerve. 2010 Feb;41(2):202-7
references.pmid: 19882646
references.citation: Neurology. 1999 Jul 13;53(1):62-70
references.pmid: 10408538
references.citation: Ann Neurol. 1981 Sep;10(3):222-6
references.pmid: 7294727
references.citation: Brain. 2004 Sep;127(Pt 9):2010-7
references.pmid: 15289267
references.citation: Neurology. 1999 Jul 13;53(1):57-61
references.pmid: 10408537
references.citation: J Peripher Nerv Syst. 2011 Jun;16 Suppl 1:41-4
references.pmid: 21696497
references.citation: Neurology. 1983 Feb;33(2):202-10
references.pmid: 6296727