Yanagawa K, Kawachi I, Toyoshima Y et al.
Brain Research Institute, Niigata University, Niigata, Japan
Neurology 2009;73:1628–37.
Editor’s note: In this study, the authors extensively investigated the limited form of neuromyelitis optica (NMO), which occurs only in myelitis. The eight patients studied were relapsing, seropositive for aquaporin 4-specific antibodies, and showed a prevalent involvement of central gray matter on magnetic resonance imaging scans. They also showed the same histological abnormalities as seen in the definite form of NMO, which included active demyelination, loss of aquaporin 4 immunoreactivity in plaques, and diffuse infiltration of macrophages with thickened hyalinized blood vessels. This study provides evidence that aquaporin 4-specific antibodies are the initiator of NMO lesions, and are central to the pathogenesis of the NMO spectrum disorders.