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. 1994 Jul;57(7):778–783. doi: 10.1136/jnnp.57.7.778

Pure motor demyelinating neuropathy: deterioration after steroid treatment and improvement with intravenous immunoglobulin.

M Donaghy 1, K R Mills 1, S J Boniface 1, J Simmons 1, I Wright 1, N Gregson 1, J Jacobs 1
PMCID: PMC1073014  PMID: 8021660

Abstract

Within one month of starting oral prednisolone treatment weakness unexpectedly increased in four patients aged 34 to 75 years with purely motor forms of acquired chronic demyelinating neuropathy. By contrast, steroids produced the expected improvement in 11 other patients with symmetric sensorimotor chronic inflammatory demyelinating polyneuropathy. Two of the patients with purely motor demyelinating neuropathy were subsequently treated with high dose IVIg (0.4 g/kg/day for five days) with prompt improvements in strength measurements and motor nerve conduction. Thus IVIg seems to be the treatment of choice and steroids should be used with extreme caution, if at all, in patients with purely motor forms of acquired demyelinating polyneuropathy.

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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. Charles N., Benoit P., Vial C., Bierme T., Moreau T., Bady B. Intravenous immunoglobulin treatment in multifocal motor neuropathy. Lancet. 1992 Jul 18;340(8812):182–182. doi: 10.1016/0140-6736(92)93267-q. [DOI] [PubMed] [Google Scholar]
  3. 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]
  4. 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]
  5. 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]
  6. Dyck P. J., O'Brien P. C., Oviatt K. F., Dinapoli R. P., Daube J. R., Bartleson J. D., Mokri B., Swift T., Low P. A., Windebank A. J. Prednisone improves chronic inflammatory demyelinating polyradiculoneuropathy more than no treatment. Ann Neurol. 1982 Feb;11(2):136–141. doi: 10.1002/ana.410110205. [DOI] [PubMed] [Google Scholar]
  7. Feldman E. L., Bromberg M. B., Albers J. W., Pestronk A. Immunosuppressive treatment in multifocal motor neuropathy. Ann Neurol. 1991 Sep;30(3):397–401. doi: 10.1002/ana.410300312. [DOI] [PubMed] [Google Scholar]
  8. Gregson N. A., Jones D., Thomas P. K., Willison H. J. Acute motor neuropathy with antibodies to GM1 ganglioside. J Neurol. 1991 Dec;238(8):447–451. doi: 10.1007/BF00314652. [DOI] [PubMed] [Google Scholar]
  9. Jacobs J. M., Love S. Qualitative and quantitative morphology of human sural nerve at different ages. Brain. 1985 Dec;108(Pt 4):897–924. doi: 10.1093/brain/108.4.897. [DOI] [PubMed] [Google Scholar]
  10. Kaji R., Shibasaki H., Kimura J. Multifocal demyelinating motor neuropathy: cranial nerve involvement and immunoglobulin therapy. Neurology. 1992 Mar;42(3 Pt 1):506–509. doi: 10.1212/wnl.42.3.506. [DOI] [PubMed] [Google Scholar]
  11. 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]
  12. Matthews W. B., Howell D. A., Hughes R. D. Relapsing corticosteroid-dependent polyneuritis. J Neurol Neurosurg Psychiatry. 1970 Jun;33(3):330–337. doi: 10.1136/jnnp.33.3.330. [DOI] [PMC free article] [PubMed] [Google Scholar]
  13. 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]
  14. Nobile-Orazio E., Meucci N., Barbieri S., Carpo M., Scarlato G. High-dose intravenous immunoglobulin therapy in multifocal motor neuropathy. Neurology. 1993 Mar;43(3 Pt 1):537–544. doi: 10.1212/wnl.43.3_part_1.537. [DOI] [PubMed] [Google Scholar]
  15. Pestronk A., Chaudhry V., Feldman E. L., Griffin J. W., Cornblath D. R., Denys E. H., Glasberg M., Kuncl R. W., Olney R. K., Yee W. C. Lower motor neuron syndromes defined by patterns of weakness, nerve conduction abnormalities, and high titers of antiglycolipid antibodies. Ann Neurol. 1990 Mar;27(3):316–326. doi: 10.1002/ana.410270314. [DOI] [PubMed] [Google Scholar]
  16. 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]
  17. Sadiq S. A., Thomas F. P., Kilidireas K., Protopsaltis S., Hays A. P., Lee K. W., Romas S. N., Kumar N., van den Berg L., Santoro M. The spectrum of neurologic disease associated with anti-GM1 antibodies. Neurology. 1990 Jul;40(7):1067–1072. doi: 10.1212/wnl.40.7.1067. [DOI] [PubMed] [Google Scholar]
  18. Thomas P. K., Lascelles R. G., Hallpike J. F., Hewer R. L. Recurrent and chronic relapsing Guillain-Barré polyneuritis. Brain. 1969;92(3):589–606. doi: 10.1093/brain/92.3.589. [DOI] [PubMed] [Google Scholar]

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