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

Tools

Item Type:	Article
Title:	Characterization of neuromyelitis optica and neuromyelitis optica spectrum disorder patients with a late onset
Creators Name:	Collongues, N., Marignier, R., Jacob, A., Leite, M., Siva, A., Paul, F., Zephir, H., Akman-Demir, G., Elsone, L., Jarius, S., Papeix, C., Mutch, K., Saip, S., Wildemann, B., Kitley, J., Karabudak, R., Aktas, O., Kuscu, D., Altintas, A., Palace, J., 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
Volume:	20
Number:	8
Page Range:	1086-1094
Date:	2014
Official Publication:	https://doi.org/10.1177/1352458513515085
PubMed:	View item in PubMed

Repository Staff Only: item control page