[Diagnosis and treatment of multifocal motor neuropathy (Lewis-Sumner)].
We made a retrospective long-term follow-up study of 25 patients with multifocal motor neuropathy (Lewis-Sumner). The diagnosis was based upon criteria modified from those of AAEM (Sumner 1997). The electrophysiological findings indicating conduction block or focal demyelinative lesions were more diagnostic than anti-GM 1 antibody titers, which were elevated in only 40% of these patients. Demonstration of definite conduction block was not always possible in those patients who responded favorably to intravenous immunoglobulins (IVIg), whereas indirect pieces of evidence such as F-wave abnormalities or focal conduction delay or dispersion were equally helpful. IVIg had superior outcome to cyclophosphamide, which sometimes caused serious adverse effects. Three patients with severe axonal involvement showed elevated monospecific antibodies to GalNAc-GD1a.
Kaji
R
R
Department of Neurology, Kyoto University Hospital.
D000293:Adolescent; D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000032:analysis; D015415:Biomarkers / Q000032:analysis; D003711:Demyelinating Diseases / Q000175:diagnosis* / Q000628:therapy; D004594:Electrophysiology; D005260:Female; D005500:Follow-Up Studies; D005677:G(M1) Ganglioside / Q000276:immunology; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage; D008297:Male; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000175:diagnosis* / Q000628:therapy; D009435:Synaptic Transmission; D013577:Syndrome
D001323:Autoantibodies; D015415:Biomarkers; D016756:Immunoglobulins, Intravenous; D005677:G(M1) Ganglioside