Download PDF
Semin Neurol 2010; 30(4): 356-364
DOI: 10.1055/s-0030-1267279
© Thieme Medical Publishers

Inflammatory Neuropathies

Jackie Whitesell1
  • 1Department of Neurology, University of Utah Clinical Neurosciences Center, Salt Lake City, Utah
Further Information

Publication History

Publication Date:
12 October 2010 (online)

    Permissions and Reprints

ABSTRACT

Inflammatory neuropathies are acquired disorders of peripheral nerves and occasionally of the central nervous system that can affect individuals at any age. The course can be monophasic, relapsing, or progressive. Inflammatory neuropathies are classified as acute or chronic. The acute form reaches a nadir by 4 weeks and the chronic form over 8 weeks or greater. The most common example of an acute inflammatory neuropathy is acute inflammatory demyelinating polyradiculoneuropathy (AIDP), which is part of the Guillain-Barré syndrome (GBS). The most common chronic inflammatory neuropathy is chronic inflammatory demyelinating polyradiculopathy (CIDP). Other chronic inflammatory neuropathies are multifocal motor neuropathy (MMN) and the Lewis-Sumner syndrome. The Fisher syndrome and Bickerstaff brainstem encephalitis occur acutely and have clinical overlap with AIDP.

KEYWORDS

Guillain-Barré syndrome - acute inflammatory demyelinating polyradiculoneuropathy - chronic inflammatory demyelinating polyradiculopathy - multifocal motor neuropathy - Lewis-Sumner syndrome

REFERENCES

  • 1 Hughes R A, Rees J H. Clinical and epidemiologic features of Guillain-Barré syndrome.  J Infect Dis. 1997;  176(Suppl 2) S92-S98
    CrossrefPubMedGoogle Scholar
  • 2 G. Guillain JBaAS . Sur un syndrome de radiculonevrite avec hyperalbuminose du liquide cephalo-rachidien sans reaction cellilaire. Remarques sur les caracteres cliniques et graphiques des reflexes tendineux.  Bull Soc Med Hop Paris. 1916;  1462-1470
    PubMedGoogle Scholar
  • 3 Ropper AHWE, Truax B Guillain-Barre Syndrome. Philadelphia, PA; FA Davis Company 1991
    Google Scholar
  • 4 Winer J B, Hughes R A, Anderson M J, Jones D M, Kangro H, Watkins R P. A prospective study of acute idiopathic neuropathy. II. Antecedent events.  J Neurol Neurosurg Psychiatry. 1988;  51(5) 613-618
    CrossrefPubMedGoogle Scholar
  • 5 Moulin D E, Hagen N, Feasby T E, Amireh R, Hahn A. Pain in Guillain-Barré syndrome.  Neurology. 1997;  48(2) 328-331
    CrossrefPubMedGoogle Scholar
  • 6 Hadden R D, Karch H, Hartung H P Plasma Exchange/Sandoglobulin Guillain-Barré Syndrome Trial Group et al. Preceding infections, immune factors, and outcome in Guillain-Barré syndrome.  Neurology. 2001;  56(6) 758-765
    CrossrefPubMedGoogle Scholar
  • 7 Haber P, Sejvar J, Mikaeloff Y, DeStefano F. Vaccines and Guillain-Barré syndrome.  Drug Saf. 2009;  32(4) 309-323
    CrossrefPubMedGoogle Scholar
  • 8 Hafer-Macko C E, Sheikh K A, Li C Y et al.. Immune attack on the Schwann cell surface in acute inflammatory demyelinating polyneuropathy.  Ann Neurol. 1996;  39(5) 625-635
    CrossrefPubMedGoogle Scholar
  • 9 Albers J W, Kelly Jr J J. Acquired inflammatory demyelinating polyneuropathies: clinical and electrodiagnostic features.  Muscle Nerve. 1989;  12(6) 435-451
    CrossrefPubMedGoogle Scholar
  • 10 Van den Bergh P Y, Piéret F. Electrodiagnostic criteria for acute and chronic inflammatory demyelinating polyradiculoneuropathy.  Muscle Nerve. 2004;  29(4) 565-574
    CrossrefPubMedGoogle Scholar
  • 11 Gordon P H, Wilbourn A J. Early electrodiagnostic findings in Guillain-Barré syndrome.  Arch Neurol. 2001;  58(6) 913-917
    CrossrefPubMedGoogle Scholar
  • 12 Albers J W, Donofrio P D, McGonagle T K. Sequential electrodiagnostic abnormalities in acute inflammatory demyelinating polyradiculoneuropathy.  Muscle Nerve. 1985;  8(6) 528-539
    CrossrefPubMedGoogle Scholar
  • 13 Miller R G, Peterson G W, Daube J R, Albers J W. Prognostic value of electrodiagnosis in Guillain-Barré syndrome.  Muscle Nerve. 1988;  11(7) 769-774
    CrossrefPubMedGoogle Scholar
  • 14 Gorson K C, Ropper A H, Muriello M A, Blair R. Prospective evaluation of MRI lumbosacral nerve root enhancement in acute Guillain-Barré syndrome.  Neurology. 1996;  47(3) 813-817
    PubMedGoogle Scholar
  • 15 McKhann G M, Griffin J W, Cornblath D R, Mellits E D, Fisher R S, Quaskey S A. Plasmapheresis and Guillain-Barré syndrome: analysis of prognostic factors and the effect of plasmapheresis.  Ann Neurol. 1988;  23(4) 347-353
    CrossrefPubMedGoogle Scholar
  • 16 Cornblath D R, Mellits E D, Griffin J W et al.. Motor conduction studies in Guillain-Barré syndrome: description and prognostic value.  Ann Neurol. 1988;  23(4) 354-359
    CrossrefPubMedGoogle Scholar
  • 17 Bernsen R A, de Jager A E, Schmitz P I, van der Meché F G. Long-term impact on work and private life after Guillain-Barré syndrome.  J Neurol Sci. 2002;  201(1-2) 13-17
    CrossrefPubMedGoogle Scholar
  • 18 Feasby T E, Gilbert J J, Brown W F et al.. An acute axonal form of Guillain-Barré polyneuropathy.  Brain. 1986;  109(Pt 6) 1115-1126
    CrossrefPubMedGoogle Scholar
  • 19 Griffin J W, Li C Y, Ho T W et al.. Pathology of the motor-sensory axonal Guillain-Barré syndrome.  Ann Neurol. 1996;  39(1) 17-28
    CrossrefPubMedGoogle Scholar
  • 20 Hughes R A, Cornblath D R. Guillain-Barré syndrome.  Lancet. 2005;  366(9497) 1653-1666
    CrossrefPubMedGoogle Scholar
  • 21 Chaudhry V, Corse A M, Cornblath D R, Kuncl R W, Freimer M L, Griffin J W. Multifocal motor neuropathy: electrodiagnostic features.  Muscle Nerve. 1994;  17(2) 198-205
    CrossrefPubMedGoogle Scholar
  • 22 Ang C W, Jacobs B C, Laman J D. The Guillain-Barré syndrome: a true case of molecular mimicry.  Trends Immunol. 2004;  25(2) 61-66
    CrossrefPubMedGoogle Scholar
  • 23 Yuki N, Taki T, Takahashi M et al.. Penner's serotype 4 of Campylobacter jejuni has a lipopolysaccharide that bears a GM1 ganglioside epitope as well as one that bears a GD1 a epitope.  Infect Immun. 1994;  62(5) 2101-2103
    PubMedGoogle Scholar
  • 24 Fisher M. An unusual variant of acute idiopathic polyneuritis (syndrome of ophthalmoplegia, ataxia and areflexia).  N Engl J Med. 1956;  255(2) 57-65
    CrossrefPubMedGoogle Scholar
  • 25 Mori M, Kuwabara S, Fukutake T, Yuki N, Hattori T. Clinical features and prognosis of Miller-Fisher syndrome.  Neurology. 2001;  56(8) 1104-1106
    CrossrefPubMedGoogle Scholar
  • 26 Ilyas A A, Cook S D, Mithen F A et al.. Antibodies to GT1a ganglioside in patients with Guillain-Barré syndrome.  J Neuroimmunol. 1998;  82(2) 160-167
    CrossrefPubMedGoogle Scholar
  • 27 Chiba A, Kusunoki S, Obata H, Machinami R, Kanazawa I. Serum anti-GQ1b IgG antibody is associated with ophthalmoplegia in Miller-Fisher syndrome and Guillain-Barré syndrome: clinical and immunohistochemical studies.  Neurology. 1993;  43(10) 1911-1917
    CrossrefPubMedGoogle Scholar
  • 28 Liu J X, Willison H J, Pedrosa-Domellöf F. Immunolocalization of GQ1b and related gangliosides in human extraocular neuromuscular junctions and muscle spindles.  Invest Ophthalmol Vis Sci. 2009;  50(7) 3226-3232
    CrossrefPubMedGoogle Scholar
  • 29 Kornberg A J, Pestronk A, Blume G M, Lopate G, Yue J, Hahn A. Selective staining of the cerebellar molecular layer by serum IgG in Miller-Fisher and related syndromes.  Neurology. 1996;  47(5) 1317-1320
    CrossrefPubMedGoogle Scholar
  • 30 Jamal G A, MacLeod W N. Electrophysiologic studies in Miller-Fisher syndrome.  Neurology. 1984;  34(5) 685-688
    PubMedGoogle Scholar
  • 31 Katsuno M, Ando T, Hakusui S, Yanagi T, Sobue G. Motor conduction studies in Miller-Fisher syndrome with severe tetraparesis.  Muscle Nerve. 2002;  25(3) 378-382
    CrossrefPubMedGoogle Scholar
  • 32 Bickerstaff E R. Brain-stem encephalitis; further observations on a grave syndrome with benign prognosis.  BMJ. 1957;  1(5032) 1384-1387
    CrossrefPubMedGoogle Scholar
  • 33 Bickerstaff E R, Cloake P C. Mesencephalitis and rhombencephalitis.  BMJ. 1951;  2(4723) 77-81
    CrossrefPubMedGoogle Scholar
  • 34 Mondéjar R R, Santos J M, Villalba E F. MRI findings in a remitting-relapsing case of Bickerstaff encephalitis.  Neuroradiology. 2002;  44(5) 411-414
    CrossrefPubMedGoogle Scholar
  • 35 Yuki N. Fisher syndrome and Bickerstaff brainstem encephalitis (Fisher-Bickerstaff syndrome).  J Neuroimmunol. 2009;  215(1-2) 1-9
    CrossrefPubMedGoogle Scholar
  • 36 Austin J H. Recurrent polyneuropathies and their corticosteroid treatment; with five-year observations of a placebo-controlled case treated with corticotrophin, cortisone, and prednisone.  Brain. 1958;  81(2) 157-192
    CrossrefPubMedGoogle Scholar
  • 37 Dyck P J, Lais A C, Ohta M, Bastron J A, Okazaki H, Groover R V. Chronic inflammatory polyradiculoneuropathy.  Mayo Clin Proc. 1975;  50(11) 621-637
    PubMedGoogle Scholar
  • 38 Gorson K. Chronic inflammatory demyelinating polyradiculoneuropathy. In: Daube JR, Dillingham TR, Bromberg MB, Werner RA Proceedings of the 56th Annual AANEM. San Diego, CA; American Association of Neuromuscular & Electrodiagnostic Medicine 2009: 1-13
    Google Scholar
  • 39 Koski C L, Baumgarten M, Magder L S et al.. Derivation and validation of diagnostic criteria for chronic inflammatory demyelinating polyneuropathy.  J Neurol Sci. 2009;  277(1-2) 1-8
    CrossrefPubMedGoogle Scholar
  • 40 Dionne A, Nicolle M W, Hahn A F. Clinical and electrophysiological parameters distinguishing acute-onset chronic inflammatory demyelinating polyneuropathy from acute inflammatory demyelinating polyneuropathy.  Muscle Nerve. 2010;  41(2) 202-217
    PubMedGoogle Scholar
  • 41 Benatar M Neuromuscular Disease: Evidence and Analysis in Clinical Neurology. Totowa, NJ; Humana Press 2006
    Google Scholar
  • 42 Research criteria for diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP). Report from an Ad Hoc Subcommittee of the American Academy of Neurology AIDS Task Force.  Neurology. 1991;  41 617-618
    PubMedGoogle Scholar
  • 43 Bladé J. Clinical practice. Monoclonal gammopathy of undetermined significance.  N Engl J Med. 2006;  355(26) 2765-2770
    CrossrefPubMedGoogle Scholar
  • 44 Yan W X, Taylor J, Andrias-Kauba S, Pollard J D. Passive transfer of demyelination by serum or IgG from chronic inflammatory demyelinating polyneuropathy patients.  Ann Neurol. 2000;  47(6) 765-775
    CrossrefPubMedGoogle Scholar
  • 45 Meléndez-Vásquez C, Redford J, Choudhary P P et al.. Immunological investigation of chronic inflammatory demyelinating polyradiculoneuropathy.  J Neuroimmunol. 1997;  73(1-2) 124-134
    CrossrefPubMedGoogle Scholar
  • 46 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
    CrossrefPubMedGoogle Scholar
  • 47 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
    CrossrefPubMedGoogle Scholar
  • 48 Parry G J, Clarke S. Multifocal acquired demyelinating neuropathy masquerading as motor neuron disease.  Muscle Nerve. 1988;  11(2) 103-107
    CrossrefPubMedGoogle Scholar
  • 49 Pestronk A, Cornblath D R, Ilyas A A et al.. A treatable multifocal motor neuropathy with antibodies to GM1 ganglioside.  Ann Neurol. 1988;  24(1) 73-78
    CrossrefPubMedGoogle Scholar
  • 50 Slee M, Selvan A, Donaghy M. Multifocal motor neuropathy: the diagnostic spectrum and response to treatment.  Neurology. 2007;  69(17) 1680-1687
    CrossrefPubMedGoogle Scholar
  • 51 Bouche P, Moulonguet A, Younes-Chennoufi A B et al.. Multifocal motor neuropathy with conduction block: a study of 24 patients.  J Neurol Neurosurg Psychiatry. 1995;  59(1) 38-44
    CrossrefPubMedGoogle Scholar
  • 52 Nobile-Orazio E, Cappellari A, Priori A. Multifocal motor neuropathy: current concepts and controversies.  Muscle Nerve. 2005;  31(6) 663-680
    CrossrefPubMedGoogle Scholar
  • 53 Van Asseldonk J T, Franssen H, Van den Berg-Vos R M, Wokke J H, Van den Berg L H. Multifocal motor neuropathy.  Lancet Neurol. 2005;  4(5) 309-319
    CrossrefPubMedGoogle Scholar
  • 54 Lambrecq V, Krim E, Rouanet-Larrivière M, Lagueny A. Sensory loss in multifocal motor neuropathy: a clinical and electrophysiological study.  Muscle Nerve. 2009;  39(2) 131-136
    CrossrefPubMedGoogle Scholar
  • 55 Pestronk A, Choksi R. Multifocal motor neuropathy. Serum IgM anti-GM1 ganglioside antibodies in most patients detected using covalent linkage of GM1 to ELISA plates.  Neurology. 1997;  49(5) 1289-1292
    PubMedGoogle Scholar
  • 56 Beekman R, van den Berg L H, Franssen H, Visser L H, van Asseldonk J T, Wokke J H. Ultrasonography shows extensive nerve enlargements in multifocal motor neuropathy.  Neurology. 2005;  65(2) 305-307
    CrossrefPubMedGoogle Scholar
  • 57 Nobile-Orazio E. Multifocal motor neuropathy.  J Neuroimmunol. 2001;  115(1-2) 4-18
    CrossrefPubMedGoogle Scholar
  • 58 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
    CrossrefPubMedGoogle Scholar
  • 59 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
    PubMedGoogle Scholar
  • 60 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
    CrossrefPubMedGoogle Scholar
  • 61 Lewis R A, Sumner A J, Brown M J, Asbury A K. Multifocal demyelinating neuropathy with persistent conduction block.  Neurology. 1982;  32(9) 958-964
    CrossrefPubMedGoogle Scholar
  • 62 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
    CrossrefPubMedGoogle Scholar
  • 63 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
    CrossrefPubMedGoogle Scholar
  • 64 Lewis R A. Multifocal dysimmune demyelination neuropathies. In: AANEM 2009 Courses A&B, ed. San Diego, CA; American Association of Neuromuscular & Electrodiagnostic Medicine 2009: 35-43
    Google Scholar

Jackie WhitesellM.D. 

Department of Neurology, University of Utah Clinical Neurosciences Center

175 North Medical Drive, Salt Lake City, UT 84132

Email: Jackie.Whitesell@hsc.utah.edu

      >