Acute motor axonal neuropathy with high titer IgG and IgA anti-GD1a antibodies following Campylobacter enteritis.
We describe the first two European cases of acute axonal motor neuropathy with both IgG and IgA anti-GD1a antibodies following Campylobacter enteritis. Both patients acutely developed severe weakness without sensory involvement, had antibodies to Campylobacter jejuni and polyclonal IgG and IgA titers > or = 12,800 to GD1a at onset, which decreased during follow-up. Serial electrophysiologic studies showed: 1, normal or only slightly slowed motor conductions; 2, evidence of a progressive loss of excitability and conduction failure in nerve fibers undergoing axonal degeneration in intermediate nerve segments and evidence of distal axonal involvement in one nerve; 3, normal sensory conductions, sensory potential amplitudes and somatosensory evoked potentials. Although we cannot exclude that axonal degeneration followed demyelination, we think that anti-GD1a antibodies account for the axonal involvement because GD1a is present in the axolemma and exposed at the node of Ranvier and in nerve terminals. The exclusive motor involvement could be explained by the fact that GD1a has a different internal structure in motor and sensory fibers.
Lugaresi
A
A
Center for Neuromuscular Diseases, University of Chieti, Ospedale Mazzoni, Ascoli Piceno, Italy.
Ragno
M
M
Torrieri
F
F
Di Guglielmo
G
G
Fermani
P
P
Uncini
A
A
D000328:Adult; D000368:Aged; D000907:Antibodies, Bacterial / Q000097:blood; D001323:Autoantibodies / Q000097:blood; D001369:Axons / Q000473:pathology; D002169:Campylobacter Infections / Q000150:complications / Q000276:immunology*; D016123:Campylobacter jejuni / Q000276:immunology*; D005060:Europe; D019054:Evoked Potentials, Motor / Q000502:physiology; D005073:Evoked Potentials, Somatosensory / Q000502:physiology; D005260:Female; D005677:G(M1) Ganglioside / Q000276:immunology; D005678:G(M2) Ganglioside / Q000276:immunology; D005732:Gangliosides / Q000276:immunology*; D006801:Humans; D007070:Immunoglobulin A / Q000097:blood; D007074:Immunoglobulin G / Q000097:blood; D008297:Male; D008475:Median Nerve / Q000502:physiology; D016472:Motor Neuron Disease / Q000276:immunology* / Q000382:microbiology* / Q000503:physiopathology; D009431:Neural Conduction / Q000502:physiology; D011129:Polyradiculoneuropathy / Q000276:immunology / Q000382:microbiology / Q000503:physiopathology; D014459:Ulnar Nerve / Q000502:physiology
D000907:Antibodies, Bacterial; D001323:Autoantibodies; D005732:Gangliosides; D007070:Immunoglobulin A; D007074:Immunoglobulin G; C021298:ganglioside, GD1a; C033851:ganglioside, GD1b; D005678:G(M2) Ganglioside; D005677:G(M1) Ganglioside