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European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of multifocal motor neuropathy *
Author(s) -
Van Schaik I. N.,
Bouche P.,
Illa I.,
Léger JM.,
Van den Bergh P.,
Cornblath D. R.,
Evers E. M. A.,
Hadden R. D. M.,
Hughes R. A. C.,
Koski C. L.,
NobileOrazio E.,
Pollard J.,
Sommer C.,
Van Doorn P. A.
Publication year - 2006
Publication title -
european journal of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.881
H-Index - 124
eISSN - 1468-1331
pISSN - 1351-5101
DOI - 10.1111/j.1468-1331.2006.01466.x
Subject(s) - medicine , multifocal motor neuropathy , guideline , azathioprine , peripheral neuropathy , neuromuscular disease , rituximab , physical therapy , disease , pathology , lymphoma , mismatch negativity , diabetes mellitus , electroencephalography , psychiatry , endocrinology
Several diagnostic criteria for multifocal motor neuropathy have been proposed in recent years and a beneficial effect of intravenous immunoglobulin (IVIg) and various other immunomodulatory drugs has been suggested in several trials and uncontrolled studies. The objectives were to prepare consensus guidelines on the definition, investigation and treatment of multifocal motor neuropathy. Disease experts and a patient representative considered references retrieved from MEDLINE and the Cochrane Library in July 2004 and prepared statements which were agreed in an iterative fashion. The Task Force agreed good practice points to define clinical and electrophysiological diagnostic criteria for multifocal motor neuropathy and investigations to be considered. The principal recommendations and good practice points were: (i) IVIg (2 g/kg given over 2–5 days) should be considered as the first line treatment (level A recommendation) when disability is sufficiently severe to warrant treatment. (ii) Corticosteroids are not recommended (good practice point). (iii) If initial treatment with IVIg is effective, repeated IVIg treatment should be considered (level C recommendation). The frequency of IVIg maintenance therapy should be guided by the individual response (good practice point). Typical treatment regimens are 1 g/kg every 2–4 weeks or 2 g/kg every 4–8 weeks (good practice point). (iv) If IVIg is not or not sufficiently effective then immunosuppressive treatment may be considered. Cyclophosphamide, ciclosporin, azathioprine, interferon beta1a, or rituximab are possible agents (good practice point). (v) Toxicity makes cyclophosphamide a less desirable option (good practice point).