Skip to main content
PMC home page
Journal of Neurology, Neurosurgery, and Psychiatry logoLink to Journal of Neurology, Neurosurgery, and Psychiatry
. 1996 Jul;61(1):36–42. doi: 10.1136/jnnp.61.1.36

Chronic dysimmune demyelinating polyneuropathy: a clinical and electrophysiological study of 93 patients.

T Maisonobe 1, B Chassande 1, M Vérin 1, M Jouni 1, J M Léger 1, P Bouche 1
PMCID: PMC486454  PMID: 8676156

Abstract

OBJECTIVES--To identify clinical, electrophysiological, and immunological characteristics of chronic immune demyelinating polyneuropathy to define for each group the appropriate therapeutic strategies. METHODS--The clinical and electrophysiological data and the response to treatment of 93 patients with an acquired chronic dysimmune demyelinating polyneuropathy (CDDP) studied over a period of 10 years were reviewed. Two groups were identified: group 1, comprising 64 patients with an idiopathic CDDP, of whom 13 had serum monoclonal or polyclonal gammopathy without detectable antibodies directed against the "myelin associated glycoprotein" (MAG), and group 2, comprising 29 patients with an IgM monoclonal gammopathy of undetermined significance (MGUS) with antibodies binding to the MAG. RESULTS--Group 1 patients had either a progressive or relapsing course. The relapsing course had more pronounced distal slowing of motor conduction velocity. In group 1, there were no significant clinical or electrophysiological differences between patients with or without gammopathy. Patients with anti-MAG antibody (group 2) differed significantly from group 1 patients, especially on the basis of electrophysiological results. They had a more pronounced slowing of peroneal motor nerve conduction velocity, a lower frequency of conduction block, and a distal accentuation of conduction slowing, distinguishing them from those with idiopathic CDDP, Charcot-Marie-Tooth polyneuropathy type 1A, and control subjects. CONCLUSION--The idiopathic CDDP group is heterogeneous with probably different subgroups. Patients with IgM MGUS polyneuropathy and anti-MAG antibodies have characteristics which distinguish them significantly from other CDDP and suggest different immune mechanisms and responses to treatment.

Full text

PDF
42

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Albers J. W., Kelly J. J., Jr Acquired inflammatory demyelinating polyneuropathies: clinical and electrodiagnostic features. Muscle Nerve. 1989 Jun;12(6):435–451. doi: 10.1002/mus.880120602. [DOI] [PubMed] [Google Scholar]
  2. Azulay J. P., Pouget J., Pellissier J. F., Blin O., Serratrice G. Polyradiculonévrites chroniques: 25 cas. Rev Neurol (Paris) 1992;148(12):752–761. [PubMed] [Google Scholar]
  3. 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 Aug;46(8):878–884. doi: 10.1001/archneur.1989.00520440064022. [DOI] [PubMed] [Google Scholar]
  4. Berger A. R., Herskovitz S., Kaplan J. Late motor involvement in cases presenting as "chronic sensory demyelinating polyneuropathy". Muscle Nerve. 1995 Apr;18(4):440–444. doi: 10.1002/mus.880180411. [DOI] [PubMed] [Google Scholar]
  5. Bouche P., Moulonguet A., Younes-Chennoufi A. B., Adams D., Baumann N., Meininger V., Léger J. M., Said G. Multifocal motor neuropathy with conduction block: a study of 24 patients. J Neurol Neurosurg Psychiatry. 1995 Jul;59(1):38–44. doi: 10.1136/jnnp.59.1.38. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. Braun P. E., Frail D. E., Latov N. Myelin-associated glycoprotein is the antigen for a monoclonal IgM in polyneuropathy. J Neurochem. 1982 Nov;39(5):1261–1265. doi: 10.1111/j.1471-4159.1982.tb12563.x. [DOI] [PubMed] [Google Scholar]
  7. Bromberg M. B. Comparison of electrodiagnostic criteria for primary demyelination in chronic polyneuropathy. Muscle Nerve. 1991 Oct;14(10):968–976. doi: 10.1002/mus.880141007. [DOI] [PubMed] [Google Scholar]
  8. Bromberg M. B., Feldman E. L., Albers J. W. Chronic inflammatory demyelinating polyradiculoneuropathy: comparison of patients with and without an associated monoclonal gammopathy. Neurology. 1992 Jun;42(6):1157–1163. doi: 10.1212/wnl.42.6.1157. [DOI] [PubMed] [Google Scholar]
  9. Dalakas M. C., Engel W. K. Chronic relapsing (dysimmune) polyneuropathy: pathogenesis and treatment. Ann Neurol. 1981;9 (Suppl):134–145. doi: 10.1002/ana.410090719. [DOI] [PubMed] [Google Scholar]
  10. Donofrio P. D., Kelly J. J., Jr AAEE case report #17: Peripheral neuropathy in monoclonal gammopathy of undetermined significance. Muscle Nerve. 1989 Jan;12(1):1–8. doi: 10.1002/mus.880120102. [DOI] [PubMed] [Google Scholar]
  11. Dyck P. J., Lais A. C., Ohta M., Bastron J. A., Okazaki H., Groover R. V. Chronic inflammatory polyradiculoneuropathy. Mayo Clin Proc. 1975 Nov;50(11):621–637. [PubMed] [Google Scholar]
  12. Feasby T. E., Hahn A. F., Koopman W. J., Lee D. H. Central lesions in chronic inflammatory demyelinating polyneuropathy: an MRI study. Neurology. 1990 Mar;40(3 Pt 1):476–478. doi: 10.1212/wnl.40.3_part_1.476. [DOI] [PubMed] [Google Scholar]
  13. Feasby T. E. Inflammatory-demyelinating polyneuropathies. Neurol Clin. 1992 Aug;10(3):651–670. [PubMed] [Google Scholar]
  14. Hafler D. A., Johnson D., Kelly J. J., Panitch H., Kyle R., Weiner H. L. Monoclonal gammopathy and neuropathy: myelin-associated glycoprotein reactivity and clinical characteristics. Neurology. 1986 Jan;36(1):75–78. doi: 10.1212/wnl.36.1.75. [DOI] [PubMed] [Google Scholar]
  15. Hawke S. H., Hallinan J. M., McLeod J. G. Cranial magnetic resonance imaging in chronic demyelinating polyneuropathy. J Neurol Neurosurg Psychiatry. 1990 Sep;53(9):794–796. doi: 10.1136/jnnp.53.9.794. [DOI] [PMC free article] [PubMed] [Google Scholar]
  16. Hughes R., Sanders E., Hall S., Atkinson P., Colchester A., Payan P. Subacute idiopathic demyelinating polyradiculoneuropathy. Arch Neurol. 1992 Jun;49(6):612–616. doi: 10.1001/archneur.1992.00530300044009. [DOI] [PubMed] [Google Scholar]
  17. Kaku D. A., England J. D., Sumner A. J. Distal accentuation of conduction slowing in polyneuropathy associated with antibodies to myelin-associated glycoprotein and sulphated glucuronyl paragloboside. Brain. 1994 Oct;117(Pt 5):941–947. doi: 10.1093/brain/117.5.941. [DOI] [PubMed] [Google Scholar]
  18. Kelly J. J., Jr Peripheral neuropathies associated with monoclonal proteins: a clinical review. Muscle Nerve. 1985 Feb;8(2):138–150. doi: 10.1002/mus.880080210. [DOI] [PubMed] [Google Scholar]
  19. Kelly J. J., Jr The electrodiagnostic findings in peripheral neuropathy associated with monoclonal gammopathy. Muscle Nerve. 1983 Sep;6(7):504–509. doi: 10.1002/mus.880060706. [DOI] [PubMed] [Google Scholar]
  20. Kelly J. J., Jr The electrodiagnostic findings in polyneuropathies associated with IgM monoclonal gammopathies. Muscle Nerve. 1990 Dec;13(12):1113–1117. doi: 10.1002/mus.880131205. [DOI] [PubMed] [Google Scholar]
  21. Kornberg A. J., Pestronk A. Immune-mediated neuropathies. Curr Opin Neurol. 1993 Oct;6(5):681–687. doi: 10.1097/00019052-199310000-00002. [DOI] [PubMed] [Google Scholar]
  22. Latov N. Pathogenesis and therapy of neuropathies associated with monoclonal gammopathies. Ann Neurol. 1995 May;37 (Suppl 1):S32–S42. doi: 10.1002/ana.410370705. [DOI] [PubMed] [Google Scholar]
  23. Lewis R. A., Sumner A. J. The electrodiagnostic distinctions between chronic familial and acquired demyelinative neuropathies. Neurology. 1982 Jun;32(6):592–596. doi: 10.1212/wnl.32.6.592. [DOI] [PubMed] [Google Scholar]
  24. Lopate G., Pestronk A. Chronic immune demyelinating neuropathies. Semin Neurol. 1994 Jun;14(2):131–136. doi: 10.1055/s-2008-1041070. [DOI] [PubMed] [Google Scholar]
  25. McCombe P. A., Pollard J. D., McLeod J. G. Chronic inflammatory demyelinating polyradiculoneuropathy. A clinical and electrophysiological study of 92 cases. Brain. 1987 Dec;110(Pt 6):1617–1630. doi: 10.1093/brain/110.6.1617. [DOI] [PubMed] [Google Scholar]
  26. Mendell J. R., Kolkin S., Kissel J. T., Weiss K. L., Chakeres D. W., Rammohan K. W. Evidence for central nervous system demyelination in chronic inflammatory demyelinating polyradiculoneuropathy. Neurology. 1987 Aug;37(8):1291–1294. doi: 10.1212/wnl.37.8.1291. [DOI] [PubMed] [Google Scholar]
  27. Mendell J. R., Sahenk Z., Whitaker J. N., Trapp B. D., Yates A. J., Griggs R. C., Quarles R. H. Polyneuropathy and IgM monoclonal gammopathy: studies on the pathogenetic role of anti-myelin-associated glycoprotein antibody. Ann Neurol. 1985 Mar;17(3):243–254. doi: 10.1002/ana.410170305. [DOI] [PubMed] [Google Scholar]
  28. Nobile-Orazio E., Manfredini E., Carpo M., Meucci N., Monaco S., Ferrari S., Bonetti B., Cavaletti G., Gemignani F., Durelli L. Frequency and clinical correlates of anti-neural IgM antibodies in neuropathy associated with IgM monoclonal gammopathy. Ann Neurol. 1994 Sep;36(3):416–424. doi: 10.1002/ana.410360313. [DOI] [PubMed] [Google Scholar]
  29. Nobile-Orazio E., Manfredini E., Carpo M., Meucci N., Monaco S., Ferrari S., Bonetti B., Cavaletti G., Gemignani F., Durelli L. Frequency and clinical correlates of anti-neural IgM antibodies in neuropathy associated with IgM monoclonal gammopathy. Ann Neurol. 1994 Sep;36(3):416–424. doi: 10.1002/ana.410360313. [DOI] [PubMed] [Google Scholar]
  30. Notermans N. C., Wokke J. H., Lokhorst H. M., Franssen H., van der Graaf Y., Jennekens F. G. Polyneuropathy associated with monoclonal gammopathy of undetermined significance. A prospective study of the prognostic value of clinical and laboratory abnormalities. Brain. 1994 Dec;117(Pt 6):1385–1393. doi: 10.1093/brain/117.6.1385. [DOI] [PubMed] [Google Scholar]
  31. Oh S. J., Joy J. L., Kuruoglu R. "Chronic sensory demyelinating neuropathy": chronic inflammatory demyelinating polyneuropathy presenting as a pure sensory neuropathy. J Neurol Neurosurg Psychiatry. 1992 Aug;55(8):677–680. doi: 10.1136/jnnp.55.8.677. [DOI] [PMC free article] [PubMed] [Google Scholar]
  32. Oh S. J., Joy J. L., Sunwoo I., Kuruoglu R. A case of chronic sensory demyelinating neuropathy responding to immunotherapies. Muscle Nerve. 1992 Feb;15(2):255–256. doi: 10.1002/mus.880150219. [DOI] [PubMed] [Google Scholar]
  33. Ormerod I. E., Waddy H. M., Kermode A. G., Murray N. M., Thomas P. K. Involvement of the central nervous system in chronic inflammatory demyelinating polyneuropathy: a clinical, electrophysiological and magnetic resonance imaging study. J Neurol Neurosurg Psychiatry. 1990 Sep;53(9):789–793. doi: 10.1136/jnnp.53.9.789. [DOI] [PMC free article] [PubMed] [Google Scholar]
  34. Prineas J. W., McLeod J. G. Chronic relapsing polyneuritis. J Neurol Sci. 1976 Apr;27(4):427–458. doi: 10.1016/0022-510x(76)90213-6. [DOI] [PubMed] [Google Scholar]
  35. Rubin M., Karpati G., Carpenter S. Combined central and peripheral myelinopathy. Neurology. 1987 Aug;37(8):1287–1290. doi: 10.1212/wnl.37.8.1287. [DOI] [PubMed] [Google Scholar]
  36. Simmons Z., Albers J. W., Bromberg M. B., Feldman E. L. Long-term follow-up of patients with chronic inflammatory demyelinating polyradiculoneuropathy, without and with monoclonal gammopathy. Brain. 1995 Apr;118(Pt 2):359–368. doi: 10.1093/brain/118.2.359. [DOI] [PubMed] [Google Scholar]
  37. Simmons Z., Albers J. W., Bromberg M. B., Feldman E. L. Presentation and initial clinical course in patients with chronic inflammatory demyelinating polyradiculoneuropathy: comparison of patients without and with monoclonal gammopathy. Neurology. 1993 Nov;43(11):2202–2209. doi: 10.1212/wnl.43.11.2202. [DOI] [PubMed] [Google Scholar]
  38. Small G. A., Lovelace R. E. Chronic inflammatory demyelinating polyneuropathy. Semin Neurol. 1993 Sep;13(3):305–312. doi: 10.1055/s-2008-1041139. [DOI] [PubMed] [Google Scholar]
  39. Smith I. S. The natural history of chronic demyelinating neuropathy associated with benign IgM paraproteinaemia. A clinical and neurophysiological study. Brain. 1994 Oct;117(Pt 5):949–957. doi: 10.1093/brain/117.5.949. [DOI] [PubMed] [Google Scholar]
  40. Suarez G. A., Kelly J. J., Jr Polyneuropathy associated with monoclonal gammopathy of undetermined significance: further evidence that IgM-MGUS neuropathies are different than IgG-MGUS. Neurology. 1993 Jul;43(7):1304–1308. doi: 10.1212/wnl.43.7.1304. [DOI] [PubMed] [Google Scholar]
  41. Thomas P. K., Walker R. W., Rudge P., Morgan-Hughes J. A., King R. H., Jacobs J. M., Mills K. R., Ormerod I. E., Murray N. M., McDonald W. I. Chronic demyelinating peripheral neuropathy associated with multifocal central nervous system demyelination. Brain. 1987 Feb;110(Pt 1):53–76. doi: 10.1093/brain/110.1.53. [DOI] [PubMed] [Google Scholar]
  42. Trojaborg W., Hays A. P., van den Berg L., Younger D. S., Latov N. Motor conduction parameters in neuropathies associated with anti-MAG antibodies and other types of demyelinating and axonal neuropathies. Muscle Nerve. 1995 Jul;18(7):730–735. doi: 10.1002/mus.880180709. [DOI] [PubMed] [Google Scholar]
  43. Uncini A., Di Muzio A., Sabatelli M., Magi S., Tonali P., Gambi D. Sensitivity and specificity of diagnostic criteria for conduction block in chronic inflammatory demyelinating polyneuropathy. Electroencephalogr Clin Neurophysiol. 1993 Jun;89(3):161–169. doi: 10.1016/0168-5597(93)90129-d. [DOI] [PubMed] [Google Scholar]
  44. Yeung K. B., Thomas P. K., King R. H., Waddy H., Will R. G., Hughes R. A., Gregson N. A., Leibowitz S. The clinical spectrum of peripheral neuropathies associated with benign monoclonal IgM, IgG and IgA paraproteinaemia. Comparative clinical, immunological and nerve biopsy findings. J Neurol. 1991 Oct;238(7):383–391. doi: 10.1007/BF00319857. [DOI] [PubMed] [Google Scholar]
  45. van der Meché F. G., Vermeulen M., Busch H. F. Chronic inflammatory demyelinating polyneuropathy. Conduction failure before and during immunoglobulin or plasma therapy. Brain. 1989 Dec;112(Pt 6):1563–1571. doi: 10.1093/brain/112.6.1563. [DOI] [PubMed] [Google Scholar]
  46. van der Meché F. G., van Doorn P. A. Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy: immune mechanisms and update on current therapies. Ann Neurol. 1995 May;37 (Suppl 1):S14–S31. doi: 10.1002/ana.410370704. [DOI] [PubMed] [Google Scholar]

Articles from Journal of Neurology, Neurosurgery, and Psychiatry are provided here courtesy of BMJ Publishing Group

RESOURCES