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PAPER |
1 "Giorgio Spagnol" Service of Clinical Neuroimmunology, Department of Neurological Sciences, Dino Ferrari Centre, Milan University, IRCCS Ospedale Maggiore Policlinico, Milan, Italy
2 Service of Clinical Neurophysiology, Department of Neurological Sciences, Dino Ferrari Centre
3 Department of Neurological Sciences, Dino Ferrari Centre
Correspondence to:
Correspondence to:
Dr E Nobile-Orazio, Department of Neurological Sciences, University of Milan, IRCCS Ospedale Maggiore Policlinico, Via Francesco Sforza 35, 20122 Milan, Italy;
eduardo.nobile{at}unimi.it
Objective: To determine whether patients with clinically typical multifocal motor neuropathy (MMN) with or without definite or probable conduction block (CB) differ in terms of clinical presentation, immunological findings, or response to treatment with intravenous immunoglobulin (IVIg).
Methods: 23 consecutive patients were studied with the typical clinical features of MMN, consisting of a progressive multineuropathic motor impairment with minimal or no sensory loss. In 14 patients, electrophysiological studies disclosed the presence of a definite or probable CB according to the criteria proposed by the American Association of Electrodiagnostic Medicine (AAEM) in at least one motor nerve. Six patients had possible CB, defined as a degree of CB 10% less than that required by the AAEM for probable CB, while no CB was detected in three patients.
Results: Patients with possible CB did not differ from those with a definite or probable CB in terms of age at disease onset (mean 38.8 v 38.2 years, respectively), distribution and severity of limb weakness, clinical impairment (mean Rankin score 2.2 in both), and frequency of antiganglioside antibodies (33% v 29%). Patients with possible CB had a longer mean disease duration (9 v 5.9 years, p < 0.05) and a less frequent consistent response to IVIg (67% v 86%) than those with a definite or probable CB. Patients without a detectable CB had a similar frequency of antiganglioside antibodies (33%) but had a longer disease duration (20.3 years), greater impairment (Rankin score 2.7), and more frequent signs of axonal degeneration (41% of examined motor nerves) than patients with CB (13–15%, p < 0.005). Only one patient without detectable CB (33%) consistently improved with IVIg.
Conclusions: Patients with possible CB were clinically and immunologically indistinguishable from those with definite or probable CB, albeit with a slightly less frequent response to IVIg. This finding suggests that failure to fulfil AAEM criteria for CB in patients with otherwise clinically typical MMN should not preclude this diagnosis and consequently a treatment trial with IVIg. Whether the longer duration and greater severity of the disease and more frequent axonal impairment in patients without detectable CB than in those with CB explain their lower response to IVIg remains to be established.
Keywords: multifocal motor neuropathy; conduction block; intravenous immunoglobulin treatment; gangliosides
Abbreviations: AAEM, American Association of Electrodiagnostic Medicine; CB, conduction block; CMAP, compound muscle action potential; ELISA, enzyme linked immunosorbent assay; IVIg, intravenous immunoglobulin; MMN, multifocal motor neuropathy; MRC, Medical Research Council
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