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. 2003 Nov;74(11):1555–1561. doi: 10.1136/jnnp.74.11.1555

A variant of multifocal motor neuropathy with acute, generalised presentation and persistent conduction blocks

J Lefaucheur 1, N Gregson 1, I Gray 1, F von Raison 1, M Bertocchi 1, A Creange 1
PMCID: PMC1738247  PMID: 14617715

Abstract

Objective:Multifocal motor neuropathy with persistent conduction blocks is classically described as a chronic neuropathy with progressive onset, and acute forms have not previously been characterised. We report four cases of severe motor impairment with acute and generalised onset and with persistent motor conduction blocks.

Patients and results:An acute tetraparesis with diffuse areflexia but little or no sensory disturbance was the clinical picture. Serial electrophysiological tests showed persistent multifocal motor conduction blocks with absent F waves in most tested motor nerves. No or minor abnormalities of the sensory nerve action potentials were observed. Cerebrospinal fluid contained normal or mildly increased protein levels (<1 g/l) without cells. Campylobacter jejuni serology was negative in three patients and consistent with past infection in one patient. Anti-ganglioside antibodies were positive in three patients. A five day course of intravenous immunoglobulins produced nearly complete symptom resolution in three patients and was ineffective in one patient.

Conclusion:Because of the persistence of multifocal motor conduction blocks for several weeks or months as the isolated electrophysiological feature, these cases could not be consistent with Guillain–Barré syndrome or chronic inflammatory demyelinating polyneuropathy. They suggest an original variant of multifocal motor neuropathy with an acute and generalised initial presentation and persistent motor conduction blocks affecting all four limbs.

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Selected References

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  1. Abbruzzese M., Reni L., Schenone A., Mancardi G. L., Primavera A. Multifocal motor neuropathy with conduction block after Campylobacter jejuni enteritis. Neurology. 1997 Feb;48(2):544–544. doi: 10.1212/wnl.48.2.544. [DOI] [PubMed] [Google Scholar]
  2. Albers J. W., Kelly J. J., Jr Acquired inflammatory demyelinating polyneuropathies: clinical and electrodiagnostic features. Muscle Nerve. 1989 Jun;12(6):435–451. doi: 10.1002/mus.880120602. [DOI] [PubMed] [Google Scholar]
  3. Asbury A. K., Cornblath D. R. Assessment of current diagnostic criteria for Guillain-Barré syndrome. Ann Neurol. 1990;27 (Suppl):S21–S24. doi: 10.1002/ana.410270707. [DOI] [PubMed] [Google Scholar]
  4. Beydoun S. R., Rison R. A., Commins D. Secondary amyloidosis as a life-ending event in multifocal motor neuropathy. Muscle Nerve. 2001 Oct;24(10):1396–1402. doi: 10.1002/mus.1162. [DOI] [PubMed] [Google Scholar]
  5. Bromberg M. B. Comparison of electrodiagnostic criteria for primary demyelination in chronic polyneuropathy. Muscle Nerve. 1991 Oct;14(10):968–976. doi: 10.1002/mus.880141007. [DOI] [PubMed] [Google Scholar]
  6. Brown W. F., Feasby T. E. Conduction block and denervation in Guillain-Barré polyneuropathy. Brain. 1984 Mar;107(Pt 1):219–239. doi: 10.1093/brain/107.1.219. [DOI] [PubMed] [Google Scholar]
  7. 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]
  8. 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]
  9. Corcia P., Beaume A., Guennoc A. M., de Toffol B., Preud'homme J. L., Autret A. Neuropathie motrice aiguë démyélinisante secondaire à une gastro-entérite: une forme atypique de syndrome de Guillain-Barré? Rev Neurol (Paris) 1999 Sep;155(8):596–597. [PubMed] [Google Scholar]
  10. Cornblath D. R., Chaudhry V., Griffin J. W. Treatment of chronic inflammatory demyelinating polyneuropathy with intravenous immunoglobulin. Ann Neurol. 1991 Jul;30(1):104–106. doi: 10.1002/ana.410300119. [DOI] [PubMed] [Google Scholar]
  11. Cornblath D. R., Mellits E. D., Griffin J. W., McKhann G. M., Albers J. W., Miller R. G., Feasby T. E., Quaskey S. A. Motor conduction studies in Guillain-Barré syndrome: description and prognostic value. Ann Neurol. 1988 Apr;23(4):354–359. doi: 10.1002/ana.410230407. [DOI] [PubMed] [Google Scholar]
  12. Donaghy M., Mills K. R., Boniface S. J., Simmons J., Wright I., Gregson N., Jacobs J. Pure motor demyelinating neuropathy: deterioration after steroid treatment and improvement with intravenous immunoglobulin. J Neurol Neurosurg Psychiatry. 1994 Jul;57(7):778–783. doi: 10.1136/jnnp.57.7.778. [DOI] [PMC free article] [PubMed] [Google Scholar]
  13. Gherardi R., Gaulard P., Prost C., Rocha D., Imbert M., Andre C., Rochant H., Farcet J. P. T-cell lymphoma revealed by a peripheral neuropathy. A report of two cases with an immunohistologic study on lymph node and nerve biopsies. Cancer. 1986 Dec 15;58(12):2710–2716. doi: 10.1002/1097-0142(19861215)58:12<2710::aid-cncr2820581226>3.0.co;2-d. [DOI] [PubMed] [Google Scholar]
  14. Gordon P. H., Wilbourn A. J. Early electrodiagnostic findings in Guillain-Barré syndrome. Arch Neurol. 2001 Jun;58(6):913–917. doi: 10.1001/archneur.58.6.913. [DOI] [PubMed] [Google Scholar]
  15. Gorson K. C., Allam G., Ropper A. H. Chronic inflammatory demyelinating polyneuropathy: clinical features and response to treatment in 67 consecutive patients with and without a monoclonal gammopathy. Neurology. 1997 Feb;48(2):321–328. doi: 10.1212/wnl.48.2.321. [DOI] [PubMed] [Google Scholar]
  16. Hafer-Macko C., Hsieh S. T., Li C. Y., Ho T. W., Sheikh K., Cornblath D. R., McKhann G. M., Asbury A. K., Griffin J. W. Acute motor axonal neuropathy: an antibody-mediated attack on axolemma. Ann Neurol. 1996 Oct;40(4):635–644. doi: 10.1002/ana.410400414. [DOI] [PubMed] [Google Scholar]
  17. Ho T. W., Mishu B., Li C. Y., Gao C. Y., Cornblath D. R., Griffin J. W., Asbury A. K., Blaser M. J., McKhann G. M. Guillain-Barré syndrome in northern China. Relationship to Campylobacter jejuni infection and anti-glycolipid antibodies. Brain. 1995 Jun;118(Pt 3):597–605. doi: 10.1093/brain/118.3.597. [DOI] [PubMed] [Google Scholar]
  18. Jaspert A., Claus D., Grehl H., Neundörfer B. Multifocal motor neuropathy: clinical and electrophysiological findings. J Neurol. 1996 Oct;243(10):684–692. doi: 10.1007/BF00873973. [DOI] [PubMed] [Google Scholar]
  19. Le Forestier N., LeGuern E., Coullin P., Birouk N., Maisonobe T., Brice A., Léger J. M., Bouche P. Recurrent polyradiculoneuropathy with the 17p11.2 deletion. Muscle Nerve. 1997 Sep;20(9):1184–1186. doi: 10.1002/(sici)1097-4598(199709)20:9<1184::aid-mus16>3.0.co;2-t. [DOI] [PubMed] [Google Scholar]
  20. 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]
  21. McKhann G. M., Cornblath D. R., Griffin J. W., Ho T. W., Li C. Y., Jiang Z., Wu H. S., Zhaori G., Liu Y., Jou L. P. Acute motor axonal neuropathy: a frequent cause of acute flaccid paralysis in China. Ann Neurol. 1993 Apr;33(4):333–342. doi: 10.1002/ana.410330402. [DOI] [PubMed] [Google Scholar]
  22. McLeod J. G. Electrophysiological studies in the Guillain-Barré syndrome. Ann Neurol. 1981;9 (Suppl):20–27. doi: 10.1002/ana.410090705. [DOI] [PubMed] [Google Scholar]
  23. 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]
  24. 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]
  25. Parry G. J., Clarke S. Multifocal acquired demyelinating neuropathy masquerading as motor neuron disease. Muscle Nerve. 1988 Feb;11(2):103–107. doi: 10.1002/mus.880110203. [DOI] [PubMed] [Google Scholar]
  26. 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]
  27. Pestronk A. Multifocal motor neuropathy: diagnosis and treatment. Neurology. 1998 Dec;51(6 Suppl 5):S22–S24. doi: 10.1212/wnl.51.6_suppl_5.s22. [DOI] [PubMed] [Google Scholar]
  28. Pfeiffer G., Wicklein E. M., Wittig K. Sensitivity and specificity of different conduction block criteria. Clin Neurophysiol. 2000 Aug;111(8):1388–1394. doi: 10.1016/s1388-2457(00)00329-1. [DOI] [PubMed] [Google Scholar]
  29. Ropert A., Metral S. Conduction block in neuropathies with necrotizing vasculitis. Muscle Nerve. 1990 Feb;13(2):102–105. doi: 10.1002/mus.880130203. [DOI] [PubMed] [Google Scholar]
  30. Ropper A. H., Wijdicks E. F., Shahani B. T. Electrodiagnostic abnormalities in 113 consecutive patients with Guillain-Barré syndrome. Arch Neurol. 1990 Aug;47(8):881–887. doi: 10.1001/archneur.1990.00530080065012. [DOI] [PubMed] [Google Scholar]
  31. Roth G., Rohr J., Magistris M. R., Ochsner F. Motor neuropathy with proximal multifocal persistent conduction block, fasciculations and myokymia. Evolution to tetraplegia. Eur Neurol. 1986;25(6):416–423. doi: 10.1159/000116045. [DOI] [PubMed] [Google Scholar]
  32. Sugie K., Murata K., Ikoma K., Suzumura A., Takayanagi T. [A case of acute multifocal motor neuropathy with conduction block after Campylobacter jejuni enteritis]. Rinsho Shinkeigaku. 1998 Jan;38(1):42–45. [PubMed] [Google Scholar]
  33. Taylor B. V., Wright R. A., Harper C. M., Dyck P. J. Natural history of 46 patients with multifocal motor neuropathy with conduction block. Muscle Nerve. 2000 Jun;23(6):900–908. doi: 10.1002/(sici)1097-4598(200006)23:6<900::aid-mus9>3.0.co;2-y. [DOI] [PubMed] [Google Scholar]
  34. Van den Bergh P., Logigian E. L., Kelly J. J., Jr Motor neuropathy with multifocal conduction blocks. Muscle Nerve. 1989 Jan;12(1):26–31. doi: 10.1002/mus.880120106. [DOI] [PubMed] [Google Scholar]
  35. White J. R., Sachs G. M., Gilchrist J. M. Multifocal motor neuropathy with conduction block and Campylobacter jejuni. Neurology. 1996 Feb;46(2):562–563. doi: 10.1212/wnl.46.2.562. [DOI] [PubMed] [Google Scholar]
  36. Wijdicks E. F., Ropper A. H. Acute relapsing Guillain-Barré syndrome after long asymptomatic intervals. Arch Neurol. 1990 Jan;47(1):82–84. doi: 10.1001/archneur.1990.00530010104027. [DOI] [PubMed] [Google Scholar]
  37. Wöhrle J. C., Amoiridis G., Heye N., Przuntek H. Acute multifocal motor neuropathy with early spontaneous recovery: a distinct syndrome from Guillain-Barré syndrome? Electromyogr Clin Neurophysiol. 1996 Jan-Feb;36(1):37–41. [PubMed] [Google Scholar]
  38. Yuki N. Anti-ganglioside antibody and neuropathy: review of our research. J Peripher Nerv Syst. 1998;3(1):3–18. [PubMed] [Google Scholar]
  39. 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]

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