232Neuromyelitis optica (NMO) is a severe idiopathic immunomediated inflammatory demyelinating and necrotizing disease that is characterized by transverse myelopathy and optic neuropathy. The exact etiology and mechanisms of NMO remain unknown. Serological and clinical evidence of B cell autoimmunity has been observed in a high proportion of patients, 1 suggesting a prominent role for humoral mechanisms in the pathogenesis of NMO. Neuromyelitis optica lesions show a marked deposition of immunoglobulins and complement in a characteristic perivascular rosette pattern along the outer rim of thickened vessel walls 2 . A specific IgG autoantibody, NMO-IgG, selectively targeting aquaporin 4 (AQP4), which accumulates in the central nervous system (CNS) microvessels, pia, subpia, and Virchow-Robin space, has been confirmed by indirect immunofluorescence with a composite substrate of mouse tissue 3,4 . According to this antibody, the new diagnostic criteria for NMO proposed by Wingerchuk et al facilitate its distinction from multiple sclerosis (MS) 5,6 . In subsequent studies, analysis of pathological brain lesions in NMO patients revealed a pattern of vasculocentric immune complex deposition and AQP4 loss ABSTRACT: Objective: To compare the clinical features of our sero-negative and sero-positive neuromyelitis optica (NMO) patients. Methods: Thirty-nine patients with NMO were recruited and analyzed retrospectively. Serum aquaporin 4 (AQP4) antibody status was determined by a cell-based assay. For the sero-negative patients, cerebrospinal fluid (CSF) and serum samples were re-tested using the cell-based assay and an indirect immunofluorescence assay. Results: By the cell-based assay, 30 patients (76.92%, 30/39), were positive for AQP4 antibodies in serum and 37 patients (94.9%, 37/39), had a CSF-positive antibody status. Seven NMO patients (17.9%, 7/39) were sero-negative by the cell-based assay but demonstrated positive CSF results. By indirect immunofluorescence, the remaining two patients, who had no AQP4 antibodies in serum or CSF by the cell-based assay, were positive for IgG antibodies in serum, which selectively targeted the central nervous system microvessels, pia, subpia, Virchow-Robin space, kidney, and stomach. There were no significant differences between the sero-positive and sero-negative NMO groups among their demographic and clinical data. Conclusions: Repeating the test using a different assay or CSF is helpful to clarify whether sero-negative NMO patients do in fact carry AQP4 antibodies.
ORIGINAL ARTICLEidentical to that seen in NMO lesions, suggesting that AQP4 antibodies are related to NMO pathology 7,8 .Although more than 15 different immunoassays for the detection of AQP4 antibodies in patients with NMO have been developed, only a proportion of patients (33-91% among Western NMO cases and 41.7-91% among Japanese opticospinal MS (OSMS) are positive for AQP4 antibodies in serum 3,9,10 . In other words, the number of sero-negative NMO patients is not negligible.https://www.cambridge.org/core/terms. h...