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Natural history of 46 patients with multifocal motor neuropathy with conduction block

Citation

Taylor, BV and Wright, RA and Harper, CM and Dyck, PJ, Natural history of 46 patients with multifocal motor neuropathy with conduction block, Muscle and Nerve, 23, (6) pp. 900-908. ISSN 0148-639X (2000) [Refereed Article]

DOI: doi:10.1002/(SICI)1097-4598(200006)23:6<900::AID-MUS9>3.0.CO;2-Y

Abstract

We studied 46 consecutive patients with multifocal motor neuropathy with conduction block (MMN-CB). Typically, asymmetric weakness and atrophy of the hands or arms developed insidiously, but spontaneous improvement (without treatment) or death from this disease did not occur and 94% remained employed. For 18 patients examined on multiple occasions using the weakness subscore of the neuropathy impairment score [NIS(W)] for a median time of 2.3 years, worsening of 1.3 points per year was observed; many patients, however, had received intensive immunomodulating therapy. Median worsening to our first evaluation (generally without treatment) was estimated at 4.2 points per year, perhaps suggesting that treatment had influenced course. Three criteria for conduction block (CB) were compared, but the least stringent was sensitive for the diagnosis. Conduction block accompanied by weakness and atrophy typically affected only motor fibers, especially of midforearm nerves, and these sites of dysfunction persisted for months or years. Neurological signs and electrodiagnostic features were consistent with CB, axonal degeneration, a variable degree of reinnervation, and segmental demyelination. Although this study did not focus on therapy, intravenous gammaglobulin and cyclophosphamide appeared to be associated with neurological improvement, which was seldom complete or sustained. Axonal degeneration and faulty regeneration may in part explain this muted response. Possibly, treatment must be earlier, more intense, or different.

Item Details

Item Type:Refereed Article
Research Division:Medical and Health Sciences
Research Group:Neurosciences
Research Field:Central Nervous System
Objective Division:Health
Objective Group:Clinical Health (Organs, Diseases and Abnormal Conditions)
Objective Field:Nervous System and Disorders
Author:Taylor, BV (Professor Bruce Taylor)
ID Code:71716
Year Published:2000
Web of Science® Times Cited:114
Deposited By:Menzies Institute for Medical Research
Deposited On:2011-08-03
Last Modified:2011-08-03
Downloads:0

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