Article

The relationship between neuromyelitis optica (NMO) and multiple sclerosis (MS)
has long been controversial. NMO was previously considered a form of MS involving
predominantly the spinal cord and optic nerve. However, since the discovery of
NMO-IgG/aquaporin-4 (AQP4) antibody, an NMO-specific autoantibody to AQP4, some
unique clinical features, and magnetic resonance imaging (MRI) and other
laboratory findings in NMO, have been further clarified. AQP4 antibody is now the
most important laboratory finding for the diagnosis of NMO. Besides typical NMO,
some patients with recurrent optic neuritis or recurrent longitudinally extensive
transverse myelitis alone are also often positive for AQP4 antibody. Moreover,
studies of AQP4 antibody-positive patients have revealed that brain and brainstem
lesions are not uncommon in NMO, and some patterns appear to be unique to NMO.
All these findings have expanded the NMO concept into 'NMO spectrum disorder'
(NMOSD), and new criteria have recently been published. A new antigenic target,
myelin oligodendrocyte glycoprotein (MOG), has also been discovered recently.
This new antibody seems to correspond to around 20% of seronegative patients, but
its specificity needs to be evaluated more precisely, especially in pediatric
populations. These recent findings may also have therapeutic impact, as it has
been demonstrated that many MS drugs can exacerbate NMO. This report provides an
overview of the clinical and neuroimaging features of NMOSD, followed by its
treatment.
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