Article

The new diagnostic classification of neuromyelitis optica spectrum disorder
(NMOSD) in 2015 highlights the central role of biomarkers, such as antibodies
against aquaporin-4 (AQP4-Ab), in diagnosis. Also, in approximately 20-25% of
patients without AQP4-Ab (NMOSD(AQP4-)) the presence of an antibody directed
against myelin oligodendrocyte glycoprotein (MOG) characterizes a specific
population of NMOSD patients (NMOSD(MOG+)), according to their demographic and
clinical data and prognoses. While double-seronegative cases (NMOSD(NEG)) have
not been fully described, they may correspond to the very first patients with
opticospinal demyelination reported by Devic and Gault in 1894. The present
report reviews the current knowledge of the pathophysiology and clinical features
of NMOSD(AQP4+), NMOSD(MOG+) and NMOSD(NEG) patients, and also discusses the
relationship between the extended spectrum of MOG disease and NMOSD(MOG+).
Finally, the current treatments for acute relapses and relapse prevention are
described, with a focus on serological-based therapeutic responses and the
promising new therapeutic targets.
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Langue : ANGLAIS