Journal of Neurology

, Volume 243, Issue 10, pp 684–692 | Cite as

Multifocal motor neuropathy: Clinical and electrophysiological findings

  • Andrea Jaspert
  • Detlef Claus
  • Holger Grehl
  • Bernhard Neundörfer
Original Communicati0n


Multifocal motor neuropathy (MMN) can be differentiated from motor neuron disease by electrophysiological evidence of conduction block. To increase the probability of recording conduction block, we studied the whole nerve length including proximal segments in 84 patients with pure motor syndromes, using a special stimulation technique. In 8 patients, the diagnosis of MMN was confirmed by electrophysiological evidence of conduction block or temporal dispersion. The typical clinical picture of MMN with chronic progressive, asymmetrical, marked distal weakness was observed in our patients. Electrophysiological routine tests of distal nerves were usually normal except in nerve segments with conduction block. In 4 patients, conduction block could be recorded only in proximal nerve segments or spinal roots. All patients showed rapid improvement of clinical features and parallel reduction of conduction block during or after highdose intravenous immunoglobulin (ivIG) therapy, supporting the diagnosis of an immune-mediated neuropathy. Three of them are now in remission without any therapy, whereas 5 still receive a regular ivIG course every 2-12 weeks as long-term treatment. In all patients with pure or predominantly motor syndromes and normal findings in electrophysiological routine tests of distal nerve segments, there should be proximal conduction block studies to avoid overlooking a treatable disorder such as MMN.

Key words

Multifocal motor neuropathy Proximal conduction block Focal demyelination Immunoglobulin therapy Motor neuron disease 


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Bouche P, Moulonguet A, Ben Younes-Chennoufi A, Adams D, Baumann N, Meininger V, Léger J-M, Said G (1995) Multifocal motor neuropathy with conduction block: a study of 24 patients. J Neurol Neurosurg Psychiatry 59: 38–44PubMedGoogle Scholar
  2. 2.
    Britton TC, Meyer B-U, Herdmann J, Benecke R (1990) Clinical use of the magnetic stimulator in the investigation of peripheral conduction time. Muscle Nerve 13: 396–406PubMedGoogle Scholar
  3. 3.
    Chaudhry V, Corse AM, Comblath DR, Kuncl RW, Drachman DB, Freimer ML, Miller RG, Griffin JW (1993) Multifocal motor neuropathy: response to human immune globulin. Ann Neurol 33: 237–242PubMedGoogle Scholar
  4. 4.
    Chaudhry V, Corse A, Cornblath D, Kuncl R, Freimer M, Griffin J (1994) Multifocal motor neuropathy: electrodiagnostic features. Muscle Nerve 17: 198–205PubMedGoogle Scholar
  5. 5.
    Claus D (1990) Central motor conduction: method and normal results. Muscle Nerve 13: 1125–1132PubMedGoogle Scholar
  6. 6.
    Claus D (1991) Conduction studies in proximal nerves. Schweiz Arch Neurol Psychiatr 142: 531Google Scholar
  7. 7.
    Claus D, Brunhölzl C, Kerling FP, Henschel S (1995) Transcranial magnetic stimulation as a diagnostic and prognostic test in amyotrophic lateral sclerosis. J Neurol Sci 129 [Suppl]:30–34PubMedGoogle Scholar
  8. 8.
    Comi G, Amadio S, Galardi G, Fazio R, Nemni R (1994) Clinical and neurophysiological assessment of immunoglobulin therapy in five patients with multifocal motor neuropathy. J Neurol Neurosurg Psychiatry 57 [Suppl]:35–37Google Scholar
  9. 9.
    Comblath DR, Sumner AJ, Daube J, Gilliat RW, Brown WF, Parry GJ, Albets JW, Miller RG, Petajan J (1991) Conduction block in clinical practice. Muscle Nerve 14: 869–871PubMedGoogle Scholar
  10. 10.
    Donaghy M, Mills KR, Boniface SJ, Simmons J, Wright I, Gregson N, Jacobs J (1994) Pure motor demyelinating neuropathy: deterioration after steroid treatment and improvement with intravenous immunoglobulin. J Neurol Neurosurg Psychiatry 57: 778–783PubMedGoogle Scholar
  11. 11.
    Donofrio PD (1994) AAEM Case Report 28: monomelic amyotrophy. Muscle Nerve 17: 1129–1134PubMedGoogle Scholar
  12. 12.
    Harding AE, Bradbury PG, Murray NMF (1983) Chronic asymmetrical spinal muscular atrophy. J Neurol Sci 59: 69–83PubMedGoogle Scholar
  13. 13.
    Jaspert A, Claus D, Grehl H, Kerling F, Neundörfer B (1995) Wertigkeit der proximalen Leitungsblockuntersuchung in der Diagnostik entzundlicher Neuropathien. Nervenarzt 66: 445–454PubMedGoogle Scholar
  14. 14.
    Kaji R, Hirota N, Oka N, Kohara N, Watanabe T, Nishio T, Kimura J (1994) Anti-GMI antibodies and impaired blood-nerve barrier may interfere with remyelination in multifocal motor neuropathy. Muscle Nerve 17: 108–110PubMedGoogle Scholar
  15. 15.
    Kornberg AJ, Pestronk A (1994) The clinical and diagnostic role of antiGMI antibody testing. Muscle Nerve 17: 100–104PubMedGoogle Scholar
  16. 16.
    Krarup C, Stewart JD, Sumner AJ, Pestronk A, Lipton SA (1990) A syndrome of asymmetric limb weakness with motor conduction block. Neurology 40: 118–127PubMedGoogle Scholar
  17. 17.
    Lamb NL, Patten BM (1991) Clinical correlations of anti-GM1 antibodies in amyotrophic lateral sclerosis and neuropathies. Muscle Nerve 14: 1021–1027PubMedGoogle Scholar
  18. 18.
    Lange D, Trojaborg W, McDonald T, Blake D (1993) Persistent and transient “conduction block” in motor neuron diseases. Muscle Nerve 16: 896–903PubMedGoogle Scholar
  19. 19.
    Lange DJ, Trojaborg W (1994) Do GM1 antibodies induce demyelination? Muscle Nerve 17: 105–107PubMedGoogle Scholar
  20. 20.
    Lange DJ, Trojaborg W, Latov N, Hays AP, Younger DS, Uncini A, Blake DM, Hirano M, Burns SM, Lovelace RE, Rowland LP (1992) Multifocal motor neuropathy with con duction block: Is it a distinct clinical entity? Neurology 42: 497–505PubMedGoogle Scholar
  21. 21.
    Lewis RA, Sumner AJ, Brown MJ, Asbury AK (1982) Multifocal demyelinating neuropathy with persistent conduction block. Neurology 32: 958–964PubMedGoogle Scholar
  22. 22.
    Medical Research Council (1976) Aids to the investigation of peripheral nervous system. Memorandum no. 45. Her Majesty's Stationery Office, LondonGoogle Scholar
  23. 23.
    Mills KR, Murray NMF (1985) Proximal conduction block in early Guillain Barré syndrome. Lancet 11: 659Google Scholar
  24. 24.
    Nobile-Orazio E, Meucci N, Barbieri S, Carpo M, Scarlato G (1993) High dose intravenous immunoglobulin therapy in multifocal motor neuropathy. Neurology 43: 537–544PubMedGoogle Scholar
  25. 25.
    Parry GJ (1993) Motor neuropathy with multifocal conduction block. In: Dyck PJ (ed) Peripheral neuropathy. Saunders, Philadelphia, pp 1518–1524Google Scholar
  26. 26.
    Parry GJG (1994) Antiganglioside antibodies do not necessarily play a role in multifocal motor neuropathy. Muscle Nerve 17: 97–99PubMedGoogle Scholar
  27. 27.
    Pestronk A (1991) Motor neuropathies, motor neuron disorders, and antiglycolipid antibodies (invited review). Muscle Nerve 14: 927–936PubMedGoogle Scholar
  28. 28.
    Pestronk A, Chaudhry V, Feldman EL (1990) Lower motor neuron syndromes defined by patterns of weakness, nerve conduction abnormalities, and high titers of antiglycolipid antibodies. Ann Neurol 27: 316–326PubMedGoogle Scholar
  29. 29.
    Report from an Ad Hoc Subcommittee of the American Academy of Neurology AIDS Task Force (1991) Research criteria for diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP). Neurology 41: 617–618Google Scholar
  30. 30.
    Rhee EK, England JD, Sumner AJ (1990) A computer simulation of conduction block: effects produced by actual block versus interphase cancellation. Ann Neurol 28: 146–156PubMedGoogle Scholar
  31. 31.
    Santoro M, Thomas FP, Fink ME, Lange DJ, Uncini A, Wadia NH, Latov N, Hays AP (1990) IgM deposits at nodes of ranvier in a patient with amyotrophic lateral sclerosis, anti-GM1 antibodies, and multifocal motor conduction block. Ann Neurol 28: 373–377PubMedGoogle Scholar
  32. 32.
    Schlegel U, Jerusalem F, Tackmann W, Cordt A, Tsuda Y (1987) Benign juvenile focal muscular atrophy of upper extremities — a familial case. JNeurol Sci 80: 351–353Google Scholar
  33. 33.
    Sumner A (1994) Multifocal motor neuropathy. Muscle Nerve 17 (Suppl 1): 520Google Scholar
  34. 34.
    Tandan R (1994) Clinical features and differential diagnosis of classical motor neuron disease. In: Williams AC (ed) Motor neuron disease. Chapman & Hall, London, pp 3–27Google Scholar
  35. 35.
    Thomas PK (1994) Neuromuscular diseases: nerve (commentary). Curr Opin Neurol 7: 367–371PubMedGoogle Scholar
  36. 36.
    Van den Bergh P, Logigian EL, Kelly JJ (1989) Motor neuropathy with multifocal conduction blocks. Muscle Nerve 12: 26–31PubMedGoogle Scholar

Copyright information

© Springer-Verlag 1996

Authors and Affiliations

  • Andrea Jaspert
    • 1
  • Detlef Claus
    • 1
  • Holger Grehl
    • 1
  • Bernhard Neundörfer
    • 1
  1. 1.Department of NeurologyUniversity of Erlangen-NurembergErlangenGermany

Personalised recommendations