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A treatable multifocal motor neuropathy with antibodies to GM1 ganglioside
Author(s) -
Pestronk A.,
Cornblath D. R.,
Ilyas A. A.,
Baba H.,
Quarles R. H.,
Griffin J. W.,
Alderson K.,
Adams R. N.
Publication year - 1988
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.410240113
Subject(s) - multifocal motor neuropathy , medicine , plasmapheresis , weakness , amyotrophic lateral sclerosis , chronic inflammatory demyelinating polyneuropathy , lower motor neuron , ganglioside , antibody , motor neuron , prednisone , gastroenterology , immunology , pathology , surgery , disease , mismatch negativity , biochemistry , electroencephalography , psychiatry , chemistry
We report 2 patients with a treatable, immune‐mediated motor polyneuropathy associated with antibodies to defined neural antigens. In these patients asymmetrical weakness developed in one arm and progressed over 2 to 3 years to involve the other arm, legs, and trunk. Both patients were initially diagnosed as having lower motor neuron forms of amyotrophic lateral sclerosis. However, repeated electrophysiological testing eventually showed multifocal conduction blocks in motor but not sensory fibers compatible with patchy selective demyelination. Serum testing by thin‐layer chromatography and enzyme‐linked immunosorbent assay revealed that both patients had high titers of antibody directed against GM1 and other gangliosides. Initial therapeutic trials of prednisone (100 mg daily for 4 to 6 months) and plasmapheresis were unsuccessful. Treatment with cyclophosphamide, however, was followed by Marchked improvement in strength in both patients.