Acute motor axonal neuropathy: an antibody-mediated attack on axolemma.
The acute motor axonal neuropathy (AMAN) form of the Guillain-Barre syndrome is a paralytic disorder of abrupt onset characterized pathologically by motor nerve fiber degeneration of variable severity and by sparing of sensory fibers. There is little demyelination or lymphocytic inflammation. Most cases have antecedent infection with Campylobacter jejuni and many have antibodies directed toward GM1 ganglioside-like epitopes, but the mechanism of nerve-fiber injury has not been defined. In 7 fatal cases of AMAN, immunocytochemistry demonstrated the presence of IgG and the complement activation product C3d bound to the axolemma of motor fibers. The most frequently involved site was the nodal axolemma, but in more severe cases IgG and C3d were found within the periaxonal space of the myelinated internodes, bound to the outer surface of the motor axon. These results suggest that AMAN is a novel disorder caused by an antibody- and complement-mediated attack on the axolemma of motor fibers.
Hafer-Macko
C
C
Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
Hsieh
S T
ST
Li
C Y
CY
Ho
T W
TW
Sheikh
K
K
Cornblath
D R
DR
McKhann
G M
GM
Asbury
A K
AK
Griffin
J W
JW
D000208:Acute Disease; D000293:Adolescent; D000328:Adult; D001369:Axons / Q000648:ultrastructure; D016123:Campylobacter jejuni / Q000302:isolation & purification; D002675:Child, Preschool; D003167:Complement Activation; D015933:Complement C3d / Q000648:ultrastructure; D003711:Demyelinating Diseases / Q000503:physiopathology; D005260:Female; D005677:G(M1) Ganglioside; D006801:Humans; D007074:Immunoglobulin G; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000382:microbiology / Q000503:physiopathology*; D009410:Nerve Degeneration; D012720:Severity of Illness Index
D007074:Immunoglobulin G; D005677:G(M1) Ganglioside; D015933:Complement C3d