Skip to main content
NCBI home page
Journal of Neurology, Neurosurgery, and Psychiatry logoLink to Journal of Neurology, Neurosurgery, and Psychiatry
. 2002 Jan;72(1):37–42. doi: 10.1136/jnnp.72.1.37

Distribution patterns of demyelination correlate with clinical profiles in chronic inflammatory demyelinating polyneuropathy

S Kuwabara 1, K Ogawara 1, S Misawa 1, M Mori 1, T Hattori 1
PMCID: PMC1737682  PMID: 11784822

Abstract

Background: Chronic inflammatory demyelinating polyneuropathy (CIDP) is a heterogeneous disorder having a wide clinical range, and is characterised by multifocal demyelination that can involve the distal nerve terminals, intermediate nerve segments, and nerve roots.

Objective: To investigate whether the distribution patterns of demyelination along the course of the nerve correlate with clinical profiles in patients with CIDP.

Methods: Motor nerve conduction studies were carried out on 42 consecutive patients. According to the physiological criteria for demyelination, the presence of a demyelinative lesion was determined in the distal nerve segments (distal pattern) or intermediate nerve segments (intermediate pattern), or in both (diffuse pattern). The serum concentration of tumour necrosis factor (TNF)-α was measured by immunoassay.

Results: Patients were classified as having a distal (n=10), intermediate (n=13), or diffuse (n=15) pattern, or were unclassified (n=4). Patients with the distal or diffuse pattern had common clinical features such as subacute onset, symmetric symptoms, and weakness involving proximal as well as distal muscles. Patients with the distal pattern had a good response to treatment and a monophasic remitting course, but the diffuse pattern was associated with a treatment dependent relapsing course, reflecting longer disease activity. The serum TNF-α concentrations increased only in the "diffuse" subgroup of patients, and this might be associated with breakdown of the blood-nerve barrier and therefore, involvement of the intermediate segments. The intermediate pattern was characterised by a chronic course, asymmetric symptoms, less severe disability, and refractoriness to treatments.

Conclusions: CIDP consists of subtypes with varying predilections for lesions along the course of the nerve. The distribution patterns of conduction abnormalities may be useful in the prediction of outcome of patients with CIDP.

Full Text

The Full Text of this article is available as a PDF (149.5 KB).

Selected References

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

  1. 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]
  2. Bouchard C., Lacroix C., Planté V., Adams D., Chedru F., Guglielmi J. M., Said G. Clinicopathologic findings and prognosis of chronic inflammatory demyelinating polyneuropathy. Neurology. 1999 Feb;52(3):498–503. doi: 10.1212/wnl.52.3.498. [DOI] [PubMed] [Google Scholar]
  3. Bromberg M. B., Albers J. W. Patterns of sensory nerve conduction abnormalities in demyelinating and axonal peripheral nerve disorders. Muscle Nerve. 1993 Mar;16(3):262–266. doi: 10.1002/mus.880160304. [DOI] [PubMed] [Google Scholar]
  4. Brown W. F., Snow R. Patterns and severity of conduction abnormalities in Guillain-Barré syndrome. J Neurol Neurosurg Psychiatry. 1991 Sep;54(9):768–774. doi: 10.1136/jnnp.54.9.768. [DOI] [PMC free article] [PubMed] [Google Scholar]
  5. Chaudhry V., Corse A. M., Cornblath D. R., Kuncl R. W., Drachman D. B., Freimer M. L., Miller R. G., Griffin J. W. Multifocal motor neuropathy: response to human immune globulin. Ann Neurol. 1993 Mar;33(3):237–242. doi: 10.1002/ana.410330303. [DOI] [PubMed] [Google Scholar]
  6. 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]
  7. Dyck P. J., Litchy W. J., Kratz K. M., Suarez G. A., Low P. A., Pineda A. A., Windebank A. J., Karnes J. L., O'Brien P. C. A plasma exchange versus immune globulin infusion trial in chronic inflammatory demyelinating polyradiculoneuropathy. Ann Neurol. 1994 Dec;36(6):838–845. doi: 10.1002/ana.410360607. [DOI] [PubMed] [Google Scholar]
  8. Hall S. M., Redford E. J., Smith K. J. Tumour necrosis factor-alpha has few morphological effects within the dorsal columns of the spinal cord, in contrast to its effects in the peripheral nervous system. J Neuroimmunol. 2000 Jul 1;106(1-2):130–136. doi: 10.1016/s0165-5728(00)00213-7. [DOI] [PubMed] [Google Scholar]
  9. 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]
  10. Katz J. S., Saperstein D. S., Gronseth G., Amato A. A., Barohn R. J. Distal acquired demyelinating symmetric neuropathy. Neurology. 2000 Feb 8;54(3):615–620. doi: 10.1212/wnl.54.3.615. [DOI] [PubMed] [Google Scholar]
  11. Kuwabara S., Mori M., Ogawara K., Mizobuchi K., Hattori T., Koga M., Yuki N. Axonal involvement at the common entrapment sites in Guillain-Barré syndrome with IgG anti-GM1 antibody. Muscle Nerve. 1999 Jul;22(7):840–845. doi: 10.1002/(sici)1097-4598(199907)22:7<840::aid-mus5>3.0.co;2-2. [DOI] [PubMed] [Google Scholar]
  12. Kuwabara S., Yuki N., Koga M., Hattori T., Matsuura D., Miyake M., Noda M. IgG anti-GM1 antibody is associated with reversible conduction failure and axonal degeneration in Guillain-Barré syndrome. Ann Neurol. 1998 Aug;44(2):202–208. doi: 10.1002/ana.410440210. [DOI] [PubMed] [Google Scholar]
  13. Lewis R. A., Sumner A. J., Brown M. J., Asbury A. K. Multifocal demyelinating neuropathy with persistent conduction block. Neurology. 1982 Sep;32(9):958–964. doi: 10.1212/wnl.32.9.958. [DOI] [PubMed] [Google Scholar]
  14. 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]
  15. Midroni G., Dyck P. J. Chronic inflammatory demyelinating polyradiculoneuropathy: unusual clinical features and therapeutic responses. Neurology. 1996 May;46(5):1206–1212. doi: 10.1212/wnl.46.5.1206. [DOI] [PubMed] [Google Scholar]
  16. Olsson Y. Topographical differences in the vascular permeability of the peripheral nervous system. Acta Neuropathol. 1968 Jan 2;10(1):26–33. doi: 10.1007/BF00690507. [DOI] [PubMed] [Google Scholar]
  17. Pestronk A., Cornblath D. R., Ilyas A. A., Baba H., Quarles R. H., Griffin J. W., Alderson K., Adams R. N. A treatable multifocal motor neuropathy with antibodies to GM1 ganglioside. Ann Neurol. 1988 Jul;24(1):73–78. doi: 10.1002/ana.410240113. [DOI] [PubMed] [Google Scholar]
  18. Rhee E. K., England J. D., Sumner A. J. A computer simulation of conduction block: effects produced by actual block versus interphase cancellation. Ann Neurol. 1990 Aug;28(2):146–156. doi: 10.1002/ana.410280206. [DOI] [PubMed] [Google Scholar]
  19. Saperstein D. S., Amato A. A., Wolfe G. I., Katz J. S., Nations S. P., Jackson C. E., Bryan W. W., Burns D. K., Barohn R. J. Multifocal acquired demyelinating sensory and motor neuropathy: the Lewis-Sumner syndrome. Muscle Nerve. 1999 May;22(5):560–566. doi: 10.1002/(sici)1097-4598(199905)22:5<560::aid-mus2>3.0.co;2-q. [DOI] [PubMed] [Google Scholar]
  20. Schmidt B., Toyka K. V., Kiefer R., Full J., Hartung H. P., Pollard J. Inflammatory infiltrates in sural nerve biopsies in Guillain-Barre syndrome and chronic inflammatory demyelinating neuropathy. Muscle Nerve. 1996 Apr;19(4):474–487. doi: 10.1002/(SICI)1097-4598(199604)19:4<474::AID-MUS8>3.0.CO;2-9. [DOI] [PubMed] [Google Scholar]
  21. Sharief M. K., Ingram D. A., Swash M. Circulating tumor necrosis factor-alpha correlates with electrodiagnostic abnormalities in Guillain-Barré syndrome. Ann Neurol. 1997 Jul;42(1):68–73. doi: 10.1002/ana.410420112. [DOI] [PubMed] [Google Scholar]
  22. Sharief M. K., McLean B., Thompson E. J. Elevated serum levels of tumor necrosis factor-alpha in Guillain-Barré syndrome. Ann Neurol. 1993 Jun;33(6):591–596. doi: 10.1002/ana.410330606. [DOI] [PubMed] [Google Scholar]
  23. 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]
  24. Thomas P. K., Claus D., Jaspert A., Workman J. M., King R. H., Larner A. J., Anderson M., Emerson J. A., Ferguson I. T. Focal upper limb demyelinating neuropathy. Brain. 1996 Jun;119(Pt 3):765–774. doi: 10.1093/brain/119.3.765. [DOI] [PubMed] [Google Scholar]
  25. Uetani M., Hayashi K., Matsunaga N., Imamura K., Ito N. Denervated skeletal muscle: MR imaging. Work in progress. Radiology. 1993 Nov;189(2):511–515. doi: 10.1148/radiology.189.2.8210383. [DOI] [PubMed] [Google Scholar]
  26. Uncini A., Di Muzio A., Di Guglielmo G., De Angelis M. V., De Luca G., Lugaresi A., Gambi D. Effect of rhTNF-alpha injection into rat sciatic nerve. J Neuroimmunol. 1999 Feb 1;94(1-2):88–94. doi: 10.1016/s0165-5728(98)00229-x. [DOI] [PubMed] [Google Scholar]
  27. van der Meché F. G., Meulstee J., Vermeulen M., Kievit A. Patterns of conduction failure in the Guillain-Barré syndrome. Brain. 1988 Apr;111(Pt 2):405–416. doi: 10.1093/brain/111.2.405. [DOI] [PubMed] [Google Scholar]

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

RESOURCES