Prevalence of neuromyelitis optica spectrum disorder and phenotype distribution

摘要: Neuromyelitis optica spectrum disorder (NMOsd) is a group of demyelinating disorders recently redefined and associated with NMO-IgG/anti-aquaporin 4 antibodies. Because NMOsd unknown prevalence worldwide, we conducted retrospective, cross-sectional study 850 patients hospitalized in North East Tuscany from 1998 to 2006 examine the NMO related among unselected consecutive neurological inflammatory CNS diseases evaluate clinical phenotype identified cases. Clinical data were updated after at least 2 years follow-up. An immunofluorescence technique was used detect NMO-IgG on rat brain tissue. Sera other 828 patients, 65 non-neurological 50 healthy donors served as controls. The 1.5%, MS:NMOsd ratio 42.7. Among 13 77% had long spinal cord lesions, 38% severe optic neuritis 23% or brainstem lesions. Only 56% clinically definite final EDSS score ranged 1 10, mainly depending involvement occurrence. Our findings confirm low Caucasian population. Moreover, this demonstrates an unexpectedly high limited atypical variants disease, not previously documented.