Devic disease

Abstract

More than a century after histopathologic achievements in understanding multiple sclerosis, substantial molecular progress has permitted greater understanding of disease heterogeneity. For acute demyelinating lesions, Lassmann et al.1 proposed a classification based on molecular findings from diagnostic biopsies and autopsies. The understanding of neuromyelitis optica (NMO; Devic disease) has been further advanced by the discovery of the aquaporin-4 water channel as a target of autoantibodies in more than 80% of patients with NMO.2 Koch-Witebsky criteria have been partially fulfilled in NMO, because passive transfer of purified aquaporin antibodies to rodents creates similar clinical signs and histopathology of NMO, although these do not recapitulate human disease in all aspects.3 In one study, anti-KIR 4.1 antibodies occurred in approximately 50% of patients4; this finding is of interest because the KIR 4.1 channel is expressed close to the aquaporin channel on astrocytic endfeet.