@entity1652 [neuromyelitis optica (NMO)] is an @entity17 of the CNS, characterized by attacks of @entity7995 and @entity4685 . The mechanisms that result in selective localization of inflammatory demyelinating lesions to the optic nerves and spinal cord are unknown. Serological and clinical evidence of B cell autoimmunity has been observed in a high proportion of @entity1 with NMO. The purpose of this study was to investigate the importance of humoral mechanisms, including complement activation, in producing the @entity39 @entity17 seen in the spinal cord and optic nerves. Eighty-two lesions were examined from nine autopsy cases of clinically confirmed @entity1652 . Demyelinating activity in the lesions was immunocytochemically classified as early active (21 lesions), late active (18 lesions), inactive (35 lesions) or remyelinating (eight lesions) by examining the antigenic profile of myelin degradation products within macrophages. The pathology of the lesions was analysed using a broad spectrum of immunological and neurobiological markers, and lesions were defined on the basis of myelin protein loss, the geography and extension of plaques, the patterns of oligodendrocyte destruction and the immunopathological evidence of complement activation. The pathology was identical in all nine @entity1 . Extensive @entity17 was present across multiple spinal cord levels, associated with cavitation, @entity39 and acute axonal pathology (spheroids), in both grey and white matter. There was a pronounced loss of oligodendrocytes within the lesions. The inflammatory infiltrates in active lesions were characterized by extensive macrophage infiltration associated with large numbers of perivascular granulocytes and eosinophils and rare CD3(+) and @entity405 (+) T cells. There was a pronounced perivascular deposition of immunoglobulins (mainly IgM) and complement C9neo antigen in active lesions associated with prominent vascular @entity82 and hyalinization in both active and inactive lesions. The extent of complement activation, eosinophilic infiltration and vascular @entity82 observed in the Devic NMO cases is more prominent compared with that in prototypic @entity15 , and supports a role for humoral immunity in the pathogenesis of NMO. Based on this study, future therapeutic strategies designed to limit the deleterious effects of complement activation, eosinophil degranulation and neutrophil/macrophage/microglial activation are worthy of further investigation.

A role for humoral mechanisms in the pathogenesis of XXXX .

@entity1 :: ('9606', 'Species') :: ['patients'] @entity17 :: ('MESH:D003711', 'Disease') :: ['idiopathic inflammatory demyelinating disease', 'demyelination'] @entity82 :: ('MESH:D005355', 'Disease') :: ['fibrosis'] @entity39 :: ('MESH:D009336', 'Disease') :: ['necrosis', 'necrotizing'] @entity1652 :: ('MESH:D009471', 'Disease') :: ["Devic's disease"] @entity7995 :: ('MESH:D009902', 'Disease') :: ['optic neuritis'] @entity15 :: ('MESH:D009103', 'Disease') :: ['multiple sclerosis'] @entity405 :: ('925', 'Gene') :: ['CD8'] @entity4685 :: ('MESH:D009187', 'Disease') :: ['myelitis']
@entity1652 :: (MESH:D009471,Disease) :: ['Devic's neuromyelitis optica']
