Skip to main content
NCBI home page
Journal of Neurology, Neurosurgery, and Psychiatry logoLink to Journal of Neurology, Neurosurgery, and Psychiatry
. 1998 Sep;65(3):357–361. doi: 10.1136/jnnp.65.3.357

Single fibre electromyography in multifocal motor neuropathy with persistent conduction blocks

A Lagueny 1, G Le Masson 1, P Burbeaud 1, P Deliac 1
PMCID: PMC2170261  PMID: 9728949

Abstract

OBJECTIVE—To study the process of denervation-reinnervation in multifocal motor neuropathy with persistent conduction blocks in clinically affected and unaffected muscles.
METHOD—Volitional single fibre electromyography (SFEMG) was performed in the extensor digitorum communis (EDC) of seven patients. The jitter, the fibre density, and the mean interpotential interval were determined. The results before and after treatment with intravenous immunoglobulin (IVIg) between the unaffected EDC and affected EDC examined during the same SFEMG session were also compared. In addition the values of jitter, fibre density, and mean interpotential interval were analysed for correlation with the strength score on the MRC scale, the duration of the neuropathy, the number of IVIg treatment periods, and the radial nerve conduction block values.
RESULTS—Mean jitter, percentage of jitters>60 µs, and impulse blocking percentage, were higher than normal in both the affected EDCs and to a lesser degree in unaffected EDCs. Jitter decreased significantly after IVIg and correlated only with the MRC score. Fibre density and mean interpotential interval were higher than normal equally in the affected EDC and unaffected EDCs, but no correlation was found with strength, duration of the neuropathy, number of treatment periods, and conduction block values.
CONCLUSION—The major finding is the presence of SFEMG abnormalities in clinically unaffected EDCs. This shows a process of denervation-reinnervation even in the absence of clinical symptoms, probably more frequent than commonly supposed in this neuropathy. The rapid clinical improvement after IVIg infusions could be due to remyelination after demyelination and to an interference of IVIg with the blocking effect of antibodies on the Na+ channels at the motor nerve endings.



Full Text

The Full Text of this article is available as a PDF (146.8 KB).

Selected References

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

  1. Adams D., Kuntzer T., Steck A. J., Lobrinus A., Janzer R. C., Regli F. Motor conduction block and high titres of anti-GM1 ganglioside antibodies: pathological evidence of a motor neuropathy in a patient with lower motor neuron syndrome. J Neurol Neurosurg Psychiatry. 1993 Sep;56(9):982–987. doi: 10.1136/jnnp.56.9.982. [DOI] [PMC free article] [PubMed] [Google Scholar]
  2. Arasaki K., Kusunoki S., Kudo N., Kanazawa I. Acute conduction block in vitro following exposure to antiganglioside sera. Muscle Nerve. 1993 Jun;16(6):587–593. doi: 10.1002/mus.880160603. [DOI] [PubMed] [Google Scholar]
  3. Azulay J. P., Blin O., Pouget J., Boucraut J., Billé-Turc F., Carles G., Serratrice G. Intravenous immunoglobulin treatment in patients with motor neuron syndromes associated with anti-GM1 antibodies: a double-blind, placebo-controlled study. Neurology. 1994 Mar;44(3 Pt 1):429–432. doi: 10.1212/wnl.44.3_part_1.429. [DOI] [PubMed] [Google Scholar]
  4. 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]
  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. 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 Feb;17(2):198–205. doi: 10.1002/mus.880170211. [DOI] [PubMed] [Google Scholar]
  7. Cornblath D. R., Sumner A. J., Daube J., Gilliat R. W., Brown W. F., Parry G. J., Albers J. W., Miller R. G., Petajan J. Conduction block in clinical practice. Muscle Nerve. 1991 Sep;14(9):869–868. doi: 10.1002/mus.880140913. [DOI] [PubMed] [Google Scholar]
  8. Cruz Martínez A., Arpa J., Lara M. Electrophysiological improvement after intravenous immunoglobulin in motor neuropathy with multifocal conduction block. J Neurol Neurosurg Psychiatry. 1993 Nov;56(11):1236–1237. doi: 10.1136/jnnp.56.11.1236. [DOI] [PMC free article] [PubMed] [Google Scholar]
  9. Elliott J. L., Pestronk A. Progression of multifocal motor neuropathy during apparently successful treatment with human immunoglobulin. Neurology. 1994 May;44(5):967–968. doi: 10.1212/wnl.44.5.967. [DOI] [PubMed] [Google Scholar]
  10. Gutmann L., Gutmann L. Axonal channelopathies: an evolving concept in the pathogenesis of peripheral nerve disorders. Neurology. 1996 Jul;47(1):18–21. doi: 10.1212/wnl.47.1.18. [DOI] [PubMed] [Google Scholar]
  11. Kaji R., Hirota N., Oka N., Kohara N., Watanabe T., Nishio T., Kimura J. Anti-GM1 antibodies and impaired blood-nerve barrier may interfere with remyelination in multifocal motor neuropathy. Muscle Nerve. 1994 Jan;17(1):108–110. doi: 10.1002/mus.880170117. [DOI] [PubMed] [Google Scholar]
  12. Kaji R., Oka N., Tsuji T., Mezaki T., Nishio T., Akiguchi I., Kimura J. Pathological findings at the site of conduction block in multifocal motor neuropathy. Ann Neurol. 1993 Feb;33(2):152–158. doi: 10.1002/ana.410330204. [DOI] [PubMed] [Google Scholar]
  13. 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]
  14. Krarup C., Stewart J. D., Sumner A. J., Pestronk A., Lipton S. A. A syndrome of asymmetric limb weakness with motor conduction block. Neurology. 1990 Jan;40(1):118–127. doi: 10.1212/wnl.40.1.118. [DOI] [PubMed] [Google Scholar]
  15. Lange D. J., Trojaborg W. Do GM1 antibodies induce demyelination? Muscle Nerve. 1994 Jan;17(1):105–107. doi: 10.1002/mus.880170116. [DOI] [PubMed] [Google Scholar]
  16. 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]
  17. Nobile-Orazio E. Multifocal motor neuropathy. J Neurol Neurosurg Psychiatry. 1996 Jun;60(6):599–603. doi: 10.1136/jnnp.60.6.599. [DOI] [PMC free article] [PubMed] [Google Scholar]
  18. Parry G. J. AAEM case report #30: multifocal motor neuropathy. Muscle Nerve. 1996 Mar;19(3):269–276. doi: 10.1002/(SICI)1097-4598(199603)19:3<269::AID-MUS1>3.0.CO;2-B. [DOI] [PubMed] [Google Scholar]
  19. 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]
  20. 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]
  21. Roberts M., Willison H. J., Vincent A., Newsom-Davis J. Multifocal motor neuropathy human sera block distal motor nerve conduction in mice. Ann Neurol. 1995 Jul;38(1):111–118. doi: 10.1002/ana.410380118. [DOI] [PubMed] [Google Scholar]
  22. Saida K., Sumner A. J., Saida T., Brown M. J., Silberberg D. H. Antiserum-mediated demyelination: relationship between remyelination and functional recovery. Ann Neurol. 1980 Jul;8(1):12–24. doi: 10.1002/ana.410080103. [DOI] [PubMed] [Google Scholar]
  23. Sanders D. B., Stålberg E. V. AAEM minimonograph #25: single-fiber electromyography. Muscle Nerve. 1996 Sep;19(9):1069–1083. doi: 10.1002/(SICI)1097-4598(199609)19:9<1069::AID-MUS1>3.0.CO;2-Y. [DOI] [PubMed] [Google Scholar]
  24. Santoro M., Uncini A., Corbo M., Staugaitis S. M., Thomas F. P., Hays A. P., Latov N. Experimental conduction block induced by serum from a patient with anti-GM1 antibodies. Ann Neurol. 1992 Apr;31(4):385–390. doi: 10.1002/ana.410310407. [DOI] [PubMed] [Google Scholar]
  25. Takigawa T., Yasuda H., Kikkawa R., Shigeta Y., Saida T., Kitasato H. Antibodies against GM1 ganglioside affect K+ and Na+ currents in isolated rat myelinated nerve fibers. Ann Neurol. 1995 Apr;37(4):436–442. doi: 10.1002/ana.410370405. [DOI] [PubMed] [Google Scholar]
  26. Uncini A., Santoro M., Corbo M., Lugaresi A., Latov N. Conduction abnormalities induced by sera of patients with multifocal motor neuropathy and anti-GM1 antibodies. Muscle Nerve. 1993 Jun;16(6):610–615. doi: 10.1002/mus.880160606. [DOI] [PubMed] [Google Scholar]
  27. 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]
  28. Waxman S. G. Sodium channel blockade by antibodies: a new mechanism of neurological disease? Ann Neurol. 1995 Apr;37(4):421–423. doi: 10.1002/ana.410370403. [DOI] [PubMed] [Google Scholar]

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

RESOURCES