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Fulminant Guillain–Barré Syndrome with universal inexcitability of peripheral nerves: A clinicopathological study
Author(s) -
Berciano José,
Figols Javier,
García Antonio,
Calle Ester,
Illa Isabel,
Lafarga Miguel,
Berciano María T.
Publication year - 1997
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/(sici)1097-4598(199707)20:7<846::aid-mus9>3.0.co;2-7
Subject(s) - pathology , wallerian degeneration , guillain barre syndrome , fulminant , medicine , sural nerve , endoneurium , nerve root , anatomy , sciatic nerve , immunology
The pathological basis of nerve inexcitability in Guillain–Barré syndrome has not been established with certainty. We report the clinicopathological findings in a 67‐year‐old patient with fulminant Guillain–Barré syndrome who died 18 days after onset. Three serial electrophysiological studies revealed nerve inexcitability. Antibodies to Campylobacter jejuni were present but there was no antiganglioside reactivity. Spinal root sections revealed extensive and almost pure macrophage‐associated demyelination with occasional presence of T lymphocytes and neutrophil leukocytes. Conversely, in femoral, median, and sural nerves the outstanding lesion was axonal degeneration, with some denuded axons remaining. Unmyelinated fibers, posterior root ganglia, and dorsal columns were preserved. Endoneurial postcapillary venules showed plump endothelial cells with loss of their tight junctions. We conclude that both primary demyelination and axonal degeneration secondary to inflammation account for nerve inexcitability. Our findings lend support to the hypothesis of increased endoneurial pressure as the cause of wallerian degeneration in nerve trunks. © 1997 John Wiley & Sons, Inc. Muscle Nerve 20: 846–857, 1997