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

作者: JW Griffin , CY Li , TW Ho , P Xue , C Macko

DOI: 10.1093/BRAIN/118.3.577

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

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