Skip to main content
PMC home page
Journal of Neurology, Neurosurgery, and Psychiatry logoLink to Journal of Neurology, Neurosurgery, and Psychiatry
. 1999 Jul;67(1):15–19. doi: 10.1136/jnnp.67.1.15

Use of human intravenous immunoglobulin in lower motor neuron syndromes

C Ellis 1, S Leary 1, J Payan 1, C Shaw 1, M Hu 1, M O'Brien 1, P Leigh 1
PMCID: PMC1736457  PMID: 10369816

Abstract

OBJECTIVE—To determine whether patients with the clinical phenotype of multifocal motor neuropathy but without the electrophysiological criteria for conduction block would respond to intravenous immunoglobulin (IVIg).
METHODS—Ten patients were selected with a slowly progressive, asymmetric, lower motor neuron disorder, and were treated prospectively with IVIg at a dose of 2g/kg over 5 days. All subjects had neurophysiological testing to look for evidence of conduction block before treatment. Muscle strength was assessed by MRC grades and hand held myometry, measuring pinch and grip strength. A 20% increase in both pinch and grip myometry was considered a positive response.
RESULTS—In no patient was conduction block detected. Four of the 10 patients showed a positive response to IVIg, with the best response occurring in two patients who presented with weakness but without severe muscle wasting. Three of the four responders have continued to receive IVIg for a mean period of 17 months (range 15-24 months), with continued effect. The response to IVIg was not related to the presence of anti-GM1 antiganglioside antibodies, but responders had a selective pattern of muscle weakness and normal (>90% predicted) vital capacity.
CONCLUSION—The findings suggest that a course of IVIg should be considered in patients with the clinical phenotype of multifocal motor neuropathy but without neurophysiological evidence of conduction block.



Full Text

The Full Text of this article is available as a PDF (105.5 KB).

Selected References

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

  1. Azulay J. P., Rihet P., Pouget J., Cador F., Blin O., Boucraut J., Serratrice G. Long term follow up of multifocal motor neuropathy with conduction block under treatment. J Neurol Neurosurg Psychiatry. 1997 Apr;62(4):391–394. doi: 10.1136/jnnp.62.4.391. [DOI] [PMC free article] [PubMed] [Google Scholar]
  2. Bohannon R. W. Test-retest reliability of hand-held dynamometry during a single session of strength assessment. Phys Ther. 1986 Feb;66(2):206–209. doi: 10.1093/ptj/66.2.206. [DOI] [PubMed] [Google Scholar]
  3. 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]
  4. Dailey A. T., Tsuruda J. S., Goodkin R., Haynor D. R., Filler A. G., Hayes C. E., Maravilla K. R., Kliot M. Magnetic resonance neurography for cervical radiculopathy: a preliminary report. Neurosurgery. 1996 Mar;38(3):488–492. doi: 10.1097/00006123-199603000-00013. [DOI] [PubMed] [Google Scholar]
  5. Katz J. S., Wolfe G. I., Bryan W. W., Jackson C. E., Amato A. A., Barohn R. J. Electrophysiologic findings in multifocal motor neuropathy. Neurology. 1997 Mar;48(3):700–707. doi: 10.1212/wnl.48.3.700. [DOI] [PubMed] [Google Scholar]
  6. Lange D. J., Trojaborg W., Latov N., Hays A. P., Younger D. S., Uncini A., Blake D. M., Hirano M., Burns S. M., Lovelace R. E. Multifocal motor neuropathy with conduction block: is it a distinct clinical entity? Neurology. 1992 Mar;42(3 Pt 1):497–505. doi: 10.1212/wnl.42.3.497. [DOI] [PubMed] [Google Scholar]
  7. Parry G. J. Antiganglioside antibodies do not necessarily play a role in multifocal motor neuropathy. Muscle Nerve. 1994 Jan;17(1):97–99. doi: 10.1002/mus.880170114. [DOI] [PubMed] [Google Scholar]
  8. 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 Nov;49(5):1289–1292. doi: 10.1212/wnl.49.5.1289. [DOI] [PubMed] [Google Scholar]
  9. Schady W., Goulding P. J., Lecky B. R., King R. H., Smith C. M. Massive nerve root enlargement in chronic inflammatory demyelinating polyneuropathy. J Neurol Neurosurg Psychiatry. 1996 Dec;61(6):636–640. doi: 10.1136/jnnp.61.6.636. [DOI] [PMC free article] [PubMed] [Google Scholar]
  10. Sewell W. A., Brennan V. M., Donaghy M., Chapel H. M. The use of self infused intravenous immunoglobulin home therapy in the treatment of acquired chronic demyelinating neuropathies. J Neurol Neurosurg Psychiatry. 1997 Jul;63(1):106–109. doi: 10.1136/jnnp.63.1.106. [DOI] [PMC free article] [PubMed] [Google Scholar]
  11. Thompson N., Choudhary P., Hughes R. A., Quinlivan R. M. A novel trial design to study the effect of intravenous immunoglobulin in chronic inflammatory demyelinating polyradiculoneuropathy. J Neurol. 1996 Mar;243(3):280–285. doi: 10.1007/BF00868527. [DOI] [PubMed] [Google Scholar]
  12. Van den Berg L. H., Franssen H., Wokke J. H. Improvement of multifocal motor neuropathy during long-term weekly treatment with human immunoglobulin. Neurology. 1995 May;45(5):987–988. doi: 10.1212/wnl.45.5.987. [DOI] [PubMed] [Google Scholar]
  13. Van den Berg L. H., Kerkhoff H., Oey P. L., Franssen H., Mollee I., Vermeulen M., Jennekens F. G., Wokke J. H. Treatment of multifocal motor neuropathy with high dose intravenous immunoglobulins: a double blind, placebo controlled study. J Neurol Neurosurg Psychiatry. 1995 Sep;59(3):248–252. doi: 10.1136/jnnp.59.3.248. [DOI] [PMC free article] [PubMed] [Google Scholar]
  14. van Schaik I. N., Bossuyt P. M., Brand A., Vermeulen M. Diagnostic value of GM1 antibodies in motor neuron disorders and neuropathies: a meta-analysis. Neurology. 1995 Aug;45(8):1570–1577. doi: 10.1212/wnl.45.8.1570. [DOI] [PubMed] [Google Scholar]
  15. van den Berg L. H., Franssen H., Van Doorn P. A., Wokke J. H. Intravenous immunoglobulin treatment in lower motor neuron disease associated with highly raised anti-GM1 antibodies. J Neurol Neurosurg Psychiatry. 1997 Nov;63(5):674–677. doi: 10.1136/jnnp.63.5.674. [DOI] [PMC free article] [PubMed] [Google Scholar]

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

RESOURCES