Characterization of neuromyelitis optica and neuromyelitis optica spectrum disorder patients with a late onset: $hN. Collongues, R. Marignier, A. Jacob, M.I. Leite, A. Siva, F. Paul, H. Zephir, G. Akman-Demir, L. Elsone, S. Jarius, C. Papeix, K. Mutch, S. Saip, B. Wildemann, J. Kitley, R. Karabudak, O. Aktas, D. Kuscu, A. Altintas, J. Palace, C. Confavreux and J. De Seze
Background: 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). Objective: To characteri...
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| Main Authors: | , , |
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| Format: | Article (Journal) |
| Language: | English |
| Published: |
2014
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| In: |
Multiple sclerosis journal
Year: 2013, Volume: 20, Issue: 8, Pages: 1086-1094 |
| ISSN: | 1477-0970 |
| DOI: | 10.1177/1352458513515085 |
| Online Access: | Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1177/1352458513515085 Verlag, lizenzpflichtig, Volltext: https://journals.sagepub.com/doi/10.1177/1352458513515085 
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| Summary: | Background: 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). Objective: To characterize LONMO and LONMOSD, and to analyze their predictive factors of disability and death. Methods: 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. Results: 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. Conclusion: LONMO/LONMOSD is particularly severe, with a high rate of motor impairment and death. |
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| Item Description: | First Published: December 9, 2013 Gesehen am 21.08.2020 |
| Physical Description: | Online Resource |
| ISSN: | 1477-0970 |
| DOI: | 10.1177/1352458513515085 |