Guillain-Barré syndrome in northern China. The spectrum of neuropathological changes in clinically defined cases.

J. W. Griffin, C. Y. Li, T. W. Ho, P. Xue, C. Macko, C. Y. Gao, C. Yang, M. Tian, B. Mishu, D. R. Cornblath

Research output: Contribution to journalArticle

Abstract

The pathology of the Guillain-Barré syndrome remains controversial, and autopsied cases available for study by contemporary techniques are uncommon. Large numbers of cases clinically diagnosed as Guillain-Barré syndrome occur in northern China. In this study we examined the neuropathological changes in 12 autopsied cases from Hebei Province, China. Eleven died early in the course of their disease. In all cases tissue was specially handled and fixed for electron microscopy and for immunocytochemistry. Three of these 12 cases had typical acute inflammatory demyelinating polyneuropathy (AIDP) with lymphocytic infiltration and macrophage-mediated demyelination, reproducing the pathological picture most often reported in Guillain-Barré syndrome in North America, Europe, and Australia. Six cases had predominantly axonal involvement, characterized by Wallerian-like degeneration of nerve fibres, with only minimal demyelination and with minimal inflammation in five. Three cases, even though paralysed at the time of death, had only very mild changes in the spinal roots and sciatic nerves. Within the group of six predominantly axonal cases, there were important differences both in the severity of the abnormalities and in the classes of fibres involved. Three cases had extensive Wallerian-like degeneration of sensory as well as motor fibres [acute motor-sensory axonal neuropathy (AMSAN)], while in the other three cases the fibre degeneration affected the motor nerve fibres almost exclusively. These latter cases establish a structural basis for the clinical and electrophysiological picture termed the acute motor axonal neuropathy (AMAN) pattern. In both the AMAN and the AMSAN patterns, a prominent feature was the presence of macrophages within the periaxonal space, surrounding or displacing the axon, and surrounded by an intact myelin sheath. These studies show that the early pathological changes in cases clinically diagnosed as the Guillain-Barré syndrome are diverse and not restricted to the well-known pattern of AIDP, and that the predominant pathological patterns may differ in different parts of the world. The differences in pathological findings between acute inflammatory demyelinating polyneuropathy and the axonal patterns are likely to reflect differences in the pathogenetic mechanisms. The periaxonal macrophages in the axonal patterns suggest that an important epitope may be localized to the axolemma or periaxonal space. The mild cases indicate that severe paralysis can occur early in Guillain-Barré syndrome without prominent structural changes along the nerve, suggesting that physiological block or nerve terminal changes may be implicated.

Original languageEnglish (US)
JournalBrain
Volume118
StatePublished - Jun 1995

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China
Guillain-Barre Syndrome
Wallerian Degeneration
Macrophages
Demyelinating Diseases
Nerve Fibers
Spinal Nerves
Nerve Block
Spinal Nerve Roots
Sciatic Nerve
Myelin Sheath
North America
Paralysis
Axons
Epitopes
Electron Microscopy
Immunohistochemistry
Pathology
Inflammation

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Griffin, J. W., Li, C. Y., Ho, T. W., Xue, P., Macko, C., Gao, C. Y., ... Cornblath, D. R. (1995). Guillain-Barré syndrome in northern China. The spectrum of neuropathological changes in clinically defined cases. Brain, 118.

Guillain-Barré syndrome in northern China. The spectrum of neuropathological changes in clinically defined cases. / Griffin, J. W.; Li, C. Y.; Ho, T. W.; Xue, P.; Macko, C.; Gao, C. Y.; Yang, C.; Tian, M.; Mishu, B.; Cornblath, D. R.

In: Brain, Vol. 118, 06.1995.

Research output: Contribution to journalArticle

Griffin, JW, Li, CY, Ho, TW, Xue, P, Macko, C, Gao, CY, Yang, C, Tian, M, Mishu, B & Cornblath, DR 1995, 'Guillain-Barré syndrome in northern China. The spectrum of neuropathological changes in clinically defined cases.', Brain, vol. 118.
Griffin, J. W. ; Li, C. Y. ; Ho, T. W. ; Xue, P. ; Macko, C. ; Gao, C. Y. ; Yang, C. ; Tian, M. ; Mishu, B. ; Cornblath, D. R. / Guillain-Barré syndrome in northern China. The spectrum of neuropathological changes in clinically defined cases. In: Brain. 1995 ; Vol. 118.
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abstract = "The pathology of the Guillain-Barr{\'e} syndrome remains controversial, and autopsied cases available for study by contemporary techniques are uncommon. Large numbers of cases clinically diagnosed as Guillain-Barr{\'e} syndrome occur in northern China. In this study we examined the neuropathological changes in 12 autopsied cases from Hebei Province, China. Eleven died early in the course of their disease. In all cases tissue was specially handled and fixed for electron microscopy and for immunocytochemistry. Three of these 12 cases had typical acute inflammatory demyelinating polyneuropathy (AIDP) with lymphocytic infiltration and macrophage-mediated demyelination, reproducing the pathological picture most often reported in Guillain-Barr{\'e} syndrome in North America, Europe, and Australia. Six cases had predominantly axonal involvement, characterized by Wallerian-like degeneration of nerve fibres, with only minimal demyelination and with minimal inflammation in five. Three cases, even though paralysed at the time of death, had only very mild changes in the spinal roots and sciatic nerves. Within the group of six predominantly axonal cases, there were important differences both in the severity of the abnormalities and in the classes of fibres involved. Three cases had extensive Wallerian-like degeneration of sensory as well as motor fibres [acute motor-sensory axonal neuropathy (AMSAN)], while in the other three cases the fibre degeneration affected the motor nerve fibres almost exclusively. These latter cases establish a structural basis for the clinical and electrophysiological picture termed the acute motor axonal neuropathy (AMAN) pattern. In both the AMAN and the AMSAN patterns, a prominent feature was the presence of macrophages within the periaxonal space, surrounding or displacing the axon, and surrounded by an intact myelin sheath. These studies show that the early pathological changes in cases clinically diagnosed as the Guillain-Barr{\'e} syndrome are diverse and not restricted to the well-known pattern of AIDP, and that the predominant pathological patterns may differ in different parts of the world. The differences in pathological findings between acute inflammatory demyelinating polyneuropathy and the axonal patterns are likely to reflect differences in the pathogenetic mechanisms. The periaxonal macrophages in the axonal patterns suggest that an important epitope may be localized to the axolemma or periaxonal space. The mild cases indicate that severe paralysis can occur early in Guillain-Barr{\'e} syndrome without prominent structural changes along the nerve, suggesting that physiological block or nerve terminal changes may be implicated.",
author = "Griffin, {J. W.} and Li, {C. Y.} and Ho, {T. W.} and P. Xue and C. Macko and Gao, {C. Y.} and C. Yang and M. Tian and B. Mishu and Cornblath, {D. R.}",
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AU - Li, C. Y.

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AU - Xue, P.

AU - Macko, C.

AU - Gao, C. Y.

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AU - Tian, M.

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N2 - The pathology of the Guillain-Barré syndrome remains controversial, and autopsied cases available for study by contemporary techniques are uncommon. Large numbers of cases clinically diagnosed as Guillain-Barré syndrome occur in northern China. In this study we examined the neuropathological changes in 12 autopsied cases from Hebei Province, China. Eleven died early in the course of their disease. In all cases tissue was specially handled and fixed for electron microscopy and for immunocytochemistry. Three of these 12 cases had typical acute inflammatory demyelinating polyneuropathy (AIDP) with lymphocytic infiltration and macrophage-mediated demyelination, reproducing the pathological picture most often reported in Guillain-Barré syndrome in North America, Europe, and Australia. Six cases had predominantly axonal involvement, characterized by Wallerian-like degeneration of nerve fibres, with only minimal demyelination and with minimal inflammation in five. Three cases, even though paralysed at the time of death, had only very mild changes in the spinal roots and sciatic nerves. Within the group of six predominantly axonal cases, there were important differences both in the severity of the abnormalities and in the classes of fibres involved. Three cases had extensive Wallerian-like degeneration of sensory as well as motor fibres [acute motor-sensory axonal neuropathy (AMSAN)], while in the other three cases the fibre degeneration affected the motor nerve fibres almost exclusively. These latter cases establish a structural basis for the clinical and electrophysiological picture termed the acute motor axonal neuropathy (AMAN) pattern. In both the AMAN and the AMSAN patterns, a prominent feature was the presence of macrophages within the periaxonal space, surrounding or displacing the axon, and surrounded by an intact myelin sheath. These studies show that the early pathological changes in cases clinically diagnosed as the Guillain-Barré syndrome are diverse and not restricted to the well-known pattern of AIDP, and that the predominant pathological patterns may differ in different parts of the world. The differences in pathological findings between acute inflammatory demyelinating polyneuropathy and the axonal patterns are likely to reflect differences in the pathogenetic mechanisms. The periaxonal macrophages in the axonal patterns suggest that an important epitope may be localized to the axolemma or periaxonal space. The mild cases indicate that severe paralysis can occur early in Guillain-Barré syndrome without prominent structural changes along the nerve, suggesting that physiological block or nerve terminal changes may be implicated.

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