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

Item Type:Article
Title:Characterization of neuromyelitis optica and neuromyelitis optica spectrum disorder patients with a late onset
Creators Name:Collongues, N. and Marignier, R. and Jacob, A. and Leite, M. and Siva, A. and Paul, F. and Zephir, H. and Akman-Demir, G. and Elsone, L. and Jarius, S. and Papeix, C. and Mutch, K. and Saip, S. and Wildemann, B. and Kitley, J. and Karabudak, R. and Aktas, O. and Kuscu, D. and Altintas, A. and Palace, J. and Confavreux, C. and De Seze, J.
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.
Keywords:Aging, Aquaporin-4, Aquaporin Antibody, Late Onset, Neuromyelitis Optica, Morbidity, Mortality, Prognosis, Spectrum Disorders
Source:Multiple Sclerosis Journal
ISSN:1352-4585
Publisher:Sage Publications (U.K.)
Volume:20
Number:8
Page Range:1086-1094
Date:2014
Official Publication:https://doi.org/10.1177/1352458513515085
PubMed:View item in PubMed

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