Characterization of neuromyelitis optica and neuromyelitis optica spectrum disorder patients with a late onset

• Verfasst von: Collongues, Nicolas [VerfasserIn]
• Verfasst von: Jarius, Sven [VerfasserIn]
• Verfasst von: Wildemann, Brigitte [VerfasserIn]
• Titel: Characterization of neuromyelitis optica and neuromyelitis optica spectrum disorder patients with a late onset
• Titelzusatz: $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
• Jahr: 2014
• Jahr des Originals: 2013
• Umfang: 9 S.
• Fussnoten: First Published: December 9, 2013 ; Gesehen am 21.08.2020
• Titel Quelle: Enthalten in: Multiple sclerosis journal
• Ort Quelle: London : Sage, 1995
• Jahr Quelle: 2014
• Band/Heft Quelle: 20(2014), 8, Seite 1086-1094
• ISSN Quelle: 1477-0970
• DOI: doi:10.1177/1352458513515085
• Datenträger: Online-Ressource
• Sprache: eng
• K10plus-PPN: 1727574109

Abstract

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.