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<jats:sec><jats:title>Background:</jats:title><jats:p> Few data are available for patients with a late onset (≥ 50 years) of neuromyelitis optica (LONMO) or neuromyelitis optica spectrum disease (LONMOSD), defined by an optic neuritis/longitudinally extensive transverse myelitis with aquaporin-4 antibodies (AQP4-Ab). </jats:p></jats:sec><jats:sec><jats:title>Objective:</jats:title><jats:p> To characterize LONMO and LONMOSD, and to analyze their predictive factors of disability and death. </jats:p></jats:sec><jats:sec><jats:title>Methods:</jats:title><jats:p> We identified 430 patients from four cohorts of NMO/NMOSD in France, Germany, Turkey and UK. We extracted the late onset patients and analyzed them for predictive factors of disability and death, using the Cox proportional model. </jats:p></jats:sec><jats:sec><jats:title>Results:</jats:title><jats:p> We followed up on 63 patients with LONMO and 45 with LONMOSD during a mean of 4.6 years. This LONMO/LONMOSD cohort was mainly of Caucasian origin (93%), women (80%), seropositive for AQP4-Ab (85%) and from 50 to 82.5 years of age at onset. No progressive course was noted. At last follow-up, the median Expanded Disability Status Scale (EDSS) scores were 5.5 and 6 in the LONMO and LONMOSD groups, respectively. Outcome was mainly characterized by motor disability and relatively good visual function. At last follow-up, 14 patients had died, including seven (50%) due to acute myelitis and six (43%) because of opportunistic infections. The EDSS 4 score was independently predicted by an older age at onset, as a continuous variable after 50 years of age. Death was predicted by two independent factors: an older age at onset and a high annualized relapse rate. </jats:p></jats:sec><jats:sec><jats:title>Conclusion:</jats:title><jats:p> LONMO/LONMOSD is particularly severe, with a high rate of motor impairment and death. </jats:p></jats:sec>

Original publication




Journal article


Multiple Sclerosis Journal


SAGE Publications

Publication Date





1086 - 1094