Characterization of neuromyelitis optica and neuromyelitis optica spectrum disorder patients with a late onset.
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...
| Main Authors: | , , , , , , , , , , , , , , , , , , , , , |
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| Format: | Journal article |
| Language: | English |
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SAGE Publications
2013
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| _version_ | 1826282418347180032 |
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| author | 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 De Seze, J |
| author_facet | 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 De Seze, J |
| author_sort | Collongues, N |
| collection | OXFORD |
| description | 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. |
| first_indexed | 2024-03-07T00:43:33Z |
| format | Journal article |
| id | oxford-uuid:83ddeff3-b3bc-42e2-b2a5-c795b7d3cbf7 |
| institution | University of Oxford |
| language | English |
| last_indexed | 2024-03-07T00:43:33Z |
| publishDate | 2013 |
| publisher | SAGE Publications |
| record_format | dspace |
| spelling | oxford-uuid:83ddeff3-b3bc-42e2-b2a5-c795b7d3cbf72022-03-26T21:47:24ZCharacterization of neuromyelitis optica and neuromyelitis optica spectrum disorder patients with a late onset.Journal articlehttp://purl.org/coar/resource_type/c_dcae04bcuuid:83ddeff3-b3bc-42e2-b2a5-c795b7d3cbf7EnglishSymplectic Elements at OxfordSAGE Publications2013Collongues, NMarignier, RJacob, ALeite, MSiva, APaul, FZephir, HAkman-Demir, GElsone, LJarius, SPapeix, CMutch, KSaip, SWildemann, BKitley, JKarabudak, RAktas, OKuscu, DAltintas, APalace, JConfavreux, CDe Seze, JBACKGROUND: 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. |
| spellingShingle | 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 De Seze, J Characterization of neuromyelitis optica and neuromyelitis optica spectrum disorder patients with a late onset. |
| title | Characterization of neuromyelitis optica and neuromyelitis optica spectrum disorder patients with a late onset. |
| title_full | Characterization of neuromyelitis optica and neuromyelitis optica spectrum disorder patients with a late onset. |
| title_fullStr | Characterization of neuromyelitis optica and neuromyelitis optica spectrum disorder patients with a late onset. |
| title_full_unstemmed | Characterization of neuromyelitis optica and neuromyelitis optica spectrum disorder patients with a late onset. |
| title_short | Characterization of neuromyelitis optica and neuromyelitis optica spectrum disorder patients with a late onset. |
| title_sort | characterization of neuromyelitis optica and neuromyelitis optica spectrum disorder patients with a late onset |
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