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Acute motor axonal neuropathy: An antibody‐mediated attack on axolemma
Author(s) -
HaferMacko Charlene,
Hsieh SungTsang,
Ho Tony W.,
Sheikh Kazim,
Cornblath David R.,
Li Chun Yan,
McKhann Guy M.,
Asbury Arthur K.,
Griffin John W.
Publication year - 1996
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.410400414
Subject(s) - axolemma , axon , acute motor axonal neuropathy , antibody , neuroscience , medicine , pathology , motor nerve , immunology , biology , central nervous system , myelin
The acute motor axonal neuropathy (AMAN) form of the Guillain‐Barré 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.