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Title:
Prevalence of neuromyelitis optica spectrum disorder and phenotype distribution | Journal of Neurology
Description:
Neuromyelitis optica spectrum disorder (NMOsd) is a group of demyelinating disorders recently redefined and associated with NMO-IgG/anti-aquaporin 4 antibodies. Because NMOsd is of unknown prevalence worldwide, we conducted a retrospective, cross-sectional study of 850 patients with demyelinating disorders hospitalized in North East Tuscany from 1998 to 2006 to examine the prevalence of NMO and related disorders among unselected consecutive neurological patients with inflammatory CNS diseases and to evaluate the clinical phenotype spectrum of identified cases. Clinical data were updated after at least 2 years of follow-up. An immunofluorescence technique was used to detect NMO-IgG on rat brain tissue. Sera from other 828 neurological patients, 65 non-neurological patients and 50 healthy donors served as controls. The prevalence of NMOsd was 1.5%, with a MS:NMOsd ratio of 42.7. Among 13 NMOsd patients, 77% had long spinal cord lesions, 38% had severe optic neuritis and 23% had brain or brainstem lesions. Only 56% had clinically definite NMO at follow-up. The final EDSS score ranged from 1 to 10, mainly depending on brainstem involvement occurrence. Our findings confirm a low prevalence of NMO and related disorders among demyelinating inflammatory diseases in a Caucasian population. Moreover, this study demonstrates an unexpectedly high prevalence of limited and atypical variants of this disease, not previously documented.
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article, neuromyelitis, optica, google, scholar, pubmed, neurol, cas, spectrum, wingerchuk, prevalence, multiple, sclerosis, lucchinetti, patients, lennon, pittock, weinshenker, disorder, nmosd, disorders, brain, privacy, cookies, content, journal, antibodies, neurological, access, aquaporin, author, data, publish, research, search, neurology, maria, sabrina, matà, demyelinating, related, optic, disease, information, log, phenotype, bizzoco, lolli, repice, bahia,
Topics {✒️}
nmo-igg/anti-aquaporin 4 antibodies anti-aquaporin-4 antibodies month download article/chapter bahia hakiki detect nmo-igg blood–brain barrier permeability author information authors optic neuromyelitis syndrome severe optic neuritis inflammatory cns diseases demyelinating inflammatory diseases aquaporin-4 antibodies author correspondence full article pdf aquaporin-4 water channel clinically definite nmo privacy choices/manage cookies demyelinating disease neuromyelitis optica patients unknown prevalence worldwide unexpectedly high prevalence miranda-santos cm check access instant access igg marker rat brain tissue clinical phenotype spectrum sabrina matà demyelinating disorders hospitalized clinically distinct disorders european economic area maria pia amato north east tuscany acute disseminated encephalomyelitis neuromyelitis optica conditions privacy policy article bizzoco cross-sectional study brainstem involvement occurrence poser cm serum autoantibody marker anna maria repice disease multiple sclerosis accepting optional cookies article journal mario falcini annalisa vinattieri revised diagnostic criteria pattern-specific loss
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headline:Prevalence of neuromyelitis optica spectrum disorder and phenotype distribution
description:Neuromyelitis optica spectrum disorder (NMOsd) is a group of demyelinating disorders recently redefined and associated with NMO-IgG/anti-aquaporin 4 antibodies. Because NMOsd is of unknown prevalence worldwide, we conducted a retrospective, cross-sectional study of 850 patients with demyelinating disorders hospitalized in North East Tuscany from 1998 to 2006 to examine the prevalence of NMO and related disorders among unselected consecutive neurological patients with inflammatory CNS diseases and to evaluate the clinical phenotype spectrum of identified cases. Clinical data were updated after at least 2 years of follow-up. An immunofluorescence technique was used to detect NMO-IgG on rat brain tissue. Sera from other 828 neurological patients, 65 non-neurological patients and 50 healthy donors served as controls. The prevalence of NMOsd was 1.5%, with a MS:NMOsd ratio of 42.7. Among 13 NMOsd patients, 77% had long spinal cord lesions, 38% had severe optic neuritis and 23% had brain or brainstem lesions. Only 56% had clinically definite NMO at follow-up. The final EDSS score ranged from 1 to 10, mainly depending on brainstem involvement occurrence. Our findings confirm a low prevalence of NMO and related disorders among demyelinating inflammatory diseases in a Caucasian population. Moreover, this study demonstrates an unexpectedly high prevalence of limited and atypical variants of this disease, not previously documented.
datePublished:2009-05-28T00:00:00Z
dateModified:2009-05-28T00:00:00Z
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Neuromyelitis optica
NMO-IgG
Prevalence
Anti-aquaporin 4 antibodies
Neurology
Neurosciences
Neuroradiology
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headline:Prevalence of neuromyelitis optica spectrum disorder and phenotype distribution
description:Neuromyelitis optica spectrum disorder (NMOsd) is a group of demyelinating disorders recently redefined and associated with NMO-IgG/anti-aquaporin 4 antibodies. Because NMOsd is of unknown prevalence worldwide, we conducted a retrospective, cross-sectional study of 850 patients with demyelinating disorders hospitalized in North East Tuscany from 1998 to 2006 to examine the prevalence of NMO and related disorders among unselected consecutive neurological patients with inflammatory CNS diseases and to evaluate the clinical phenotype spectrum of identified cases. Clinical data were updated after at least 2 years of follow-up. An immunofluorescence technique was used to detect NMO-IgG on rat brain tissue. Sera from other 828 neurological patients, 65 non-neurological patients and 50 healthy donors served as controls. The prevalence of NMOsd was 1.5%, with a MS:NMOsd ratio of 42.7. Among 13 NMOsd patients, 77% had long spinal cord lesions, 38% had severe optic neuritis and 23% had brain or brainstem lesions. Only 56% had clinically definite NMO at follow-up. The final EDSS score ranged from 1 to 10, mainly depending on brainstem involvement occurrence. Our findings confirm a low prevalence of NMO and related disorders among demyelinating inflammatory diseases in a Caucasian population. Moreover, this study demonstrates an unexpectedly high prevalence of limited and atypical variants of this disease, not previously documented.
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Neuromyelitis optica
NMO-IgG
Prevalence
Anti-aquaporin 4 antibodies
Neurology
Neurosciences
Neuroradiology
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