title: Expanding the range of immunopathology in neuromyelitis optica spectrum disorder.
abstract: Neuromyelitis optica (NMO) spectrum disorder typically associates with a pathognomonic antibody response directed against aquaporin 4 and a unique immunopathological signature characterised by loss of aquaporin 4 expression. We describe a tumefactive presentation of NMO in which a biopsy specimen demonstrated active demyelination and reactive astrocytes with preserved surface aquaporin 4 immunoreactivity and dystrophic processes. While the astrocytic pathology is more typical of classical multiple sclerosis, a positive NMO-IgG (1:80) and subsequent clinical presentation with an area postrema syndrome is consistent with a diagnosis of NMO spectrum disorder. This report expands the immunopathological features that have been reported in NMO spectrum disorder.
authors.affiliation: St Vincents Hospital, Sydney, New South Wales, Australia.
authors.forename: Jennifer
authors.initials: J
authors.lastname: Massey
authors.affiliation: Brain and Mind Research Institution, University of Sydney, Sydney, New South Wales, Australia.
authors.forename: Michael E
authors.initials: ME
authors.lastname: Buckland
authors.affiliation: St Vincents Hospital, Sydney, New South Wales, Australia.
authors.forename: Yael
authors.initials: Y
authors.lastname: Barnett
authors.affiliation: St Vincents Hospital, Sydney, New South Wales, Australia.
authors.forename: Ian
authors.initials: I
authors.lastname: Sutton
mesh_terms: D000328:Adult; D051401:Aquaporin 4 / Q000276:immunology; D001253:Astrocytes / Q000276:immunology* / Q000473:pathology; D001706:Biopsy; D003711:Demyelinating Diseases / Q000276:immunology* / Q000473:pathology; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology*; D007155:Immunologic Factors / Q000627:therapeutic use; D009471:Neuromyelitis Optica / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology*; D000069283:Rituximab / Q000627:therapeutic use
chemical_list: D051401:Aquaporin 4; D007074:Immunoglobulin G; D007155:Immunologic Factors; D000069283:Rituximab