Multiple sclerosis (Houndmills, Basingstoke, England) · 2013
Characterization of neuromyelitis optica and neuromyelitis optica spectrum disorder patients with a late onset.
СтатьяМногоцентровое исследованиеПоддержка исследования: вне правительства СШАНазвание и аннотация: Перевод пока не подготовлен
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Collongues N, Marignier R, Jacob A, Leite M I, 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
Аннотация
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.
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.
Предметные рубрики MeSH
Age of OnsetAgedAged, 80 and overAquaporin 4AutoantibodiesBiomarkersCause of DeathChi-Square DistributionDisability EvaluationDisease ProgressionEuropeFemaleHumansKaplan-Meier EstimateMaleMiddle AgedMotor ActivityMultivariate AnalysisNeuromyelitis OpticaPredictive Value of TestsPrognosisProportional Hazards ModelsRetrospective StudiesRisk FactorsSeverity of Illness IndexTime Factors