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    Abstract:
    Prior studies have suggested that subclinical retinal abnormalities may be present in aquaporin-4 immunoglobulin G (AQP4-IgG) seropositive neuromyelitis optica spectrum disorder (NMOSD), in the absence of a clinical history of optic neuritis (ON).Our aim was to compare retinal layer thicknesses at the fovea and surrounding macula between AQP4-IgG+ NMOSD eyes without a history of ON (AQP4-nonON) and healthy controls (HC).In this single-center cross-sectional study, 83 AQP4-nonON and 154 HC eyes were studied with spectral-domain optical coherence tomography (OCT).Total foveal thickness did not differ between AQP4-nonON and HC eyes. AQP4-nonON eyes exhibited lower outer nuclear layer (ONL) and inner photoreceptor segment (IS) thickness at the fovea (ONL: -4.01 ± 2.03 μm, p = 0.049; IS: -0.32 ± 0.14 μm, p = 0.029) and surrounding macula (ONL: -1.98 ± 0.95 μm, p = 0.037; IS: -0.16 ± 0.07 μm, p = 0.023), compared to HC. Macular retinal nerve fiber layer (RNFL: -1.34 ± 0.51 μm, p = 0.009) and ganglion cell + inner plexiform layer (GCIPL: -2.44 ± 0.93 μm, p = 0.009) thicknesses were also lower in AQP4-nonON compared to HC eyes. Results were similar in sensitivity analyses restricted to AQP4-IgG+ patients who had never experienced ON in either eye.AQP4-nonON eyes exhibit evidence of subclinical retinal ganglion cell neuronal and axonal loss, as well as structural evidence of photoreceptor layer involvement. These findings support that subclinical anterior visual pathway involvement may occur in AQP4-IgG+ NMOSD.
    Keywords:
    Subclinical infection
    Neuromyelitis Optica
    Abstract For a long time, the most important inflammatory demyelinating diseases of the central nervous system ( CNS ), for example, multiple sclerosis ( MS ) and neuromyelitis optica ( NMO ), were extremely hard to differentiate, often with severe consequences for affected patients. This changed with the discovery of NMO ‐immunoglobulin G ( IgG ), a specific autoantibody which was detected in the vast majority of NMO patients, and with the demonstration that this autoantibody targets aquaporin 4 ( AQP 4), a water channel found on astrocytes in the CNS . These findings paved the way for the generation of experimental models of NMO . This chapter will discuss the contribution of experimental models to NMO research and what key questions remain to be addressed.
    Neuromyelitis Optica
    Aquaporin 4
    Demyelinating Disorder
    Citations (50)
    The specificity of the aquaporin-4 antibody to predict recurrent inflammatory central nervous system disease has led to the design of the 2015 neuromyelitis optica spectrum disorder criteria which capture all aquaporin-4 antibody seropositive patients.The purpose of this study was to compare treatment outcomes in aquaporin-4 antibody seropositive patients who met the previous 2006 clinical criteria for neuromyelitis optica with patients who meet the 2015 neuromyelitis optica spectrum disorder criteria.The study involved a three-center retrospective chart review of clinical outcomes among aquaporin-4 patients diagnosed with neuromyelitis optica and neuromyelitis optica spectrum disorder.Hazard ratios of relapse during immunosuppressive therapy, relative to pre-therapy, were not significantly different for patients who met the 2006 criteria of neuromyelitis optica versus the 2015 neuromyelitis optica spectrum disorder criteria among those treated with azathioprine ( p = 0.24), mycophenolate mofetil ( p = 0.63), or rituximab ( p = 0.97).Reductions in the hazard of relapse during treatment with immunosuppressive therapies, relative to average pre-treatment, were not different for aquaporin-4 antibody seropositive patients categorized using the 2006 criteria of neuromyelitis optica and the 2015 neuromyelitis optica spectrum disorder criteria. These therapeutic findings support the design of the 2015 neuromyelitis optica spectrum disorder criteria which capture all aquaporin-4 antibody seropositive patients.
    Neuromyelitis Optica
    Spectrum disorder
    Aquaporin 4
    Citations (16)
    Aquaporin-4 (AQP4) autoantibodies are specific for neuromyelitis optica (NMO) and related spectrum disorders; the specificity exceeds 90%. NMO and multiple sclerosis (MS) share transverse myelitis and optic neuritis as typical symptom complexes, but they tend to be more severe in NMO and often are associated with longitudinally extensive lesions in the corresponding affected central nervous system structures, which are rarely found in MS. While interferon beta and natalizumab benefit MS patients, they appear to exacerbate NMO.
    Neuromyelitis Optica
    Optic neuritis
    Natalizumab
    Aquaporin 4
    Spectrum disorder
    Demyelinating Disorder
    Citations (0)