Analysis Of Clinical Features And Severity-related Factors Of Neuromyelitis Optica Spectrum Disorders

Objective: The study aimed to evaluate the clinical characteristics of Relatively late-onset(the age of onset>40 years)neuromyelitis optic spectrum disorder(RLO-NMOSD),and analyze the independent risk factors affecting the severity of the NMOSD in order that doctors could improve the awareness of clinical characteristics of RLO-NMOSD,and treat the NMOSD patients individually.Methods: 265 NMOSD patients newly diagnosed in the Department of Neurology in the Second Hospital of Lanzhou University From January 2015 to December 2022.We retrospectively reviewed their medical records.(1)According to the age of onset patients were divided into two groups: RLO-NMOSD(the age of onset>40years)and REO-NMOSD(18 ≤ the age of onset ≤ 40),we analyzed the clinical characteristics of two grups.(2)Patients in the study were divided into those with mild disability(EDSS< 4)or moderate/severe disability(EDSS≥4)according to the EDSS score at admission.We explored the independent risk factors affecting the severity of the disease by performing univariate analysis and multivariate Logistic regression analysis.results:(1)A total of 265 patients with NMOSD were enrolled in the retrospective study.151 patients(56.9%)were RLO-NMOSD and 114 patients(43.1%)were RLO-NMOSD,RLO-NMOSD patients first attacked was older than that of REO-NMOSD(51 vs 29,P<0.001),Prodromal symptoms occured less in RLO-NMOSD patients before first attacked(21.9% vs 34.2%,P = 0.025),RLO-NMOSD patients first attacked was worse than that of REO-NMOSD(29.8% vs18.4%,P = 0.034).There were statistically significant differences in the onset symptom and clinical core syndrome between two groups.RLO-NMOSD patients suffered less visual dysfunction than REO-NMOSD patients at onset(15.2% vs31.6%,P=0.002),while motor and sensory dysfunction occured frequently(25.8% vs12.3%,P=0.006).RLO-NMOSD patients suffered more acute myelitis(66.2% vs43.9%,P=0.001)and sphincter dysfunction than REO-NMOSD patients(41.1% vs28.9%,P = 0.042),while optic neuritis occured less(21.9% vs 40.3%,P=0.001).Abnormal brain MRI in RLO-NMOSD patients was less than REO-NMOSD patients(40.4% vs 60.5%,P = 0.001).The levels of Cys-C,γ-GGT,TSH,TG of RLO-NMOSD patients were higher than those of REO-NMOSD.There was no statistical significance differences in AQP4-Ig G status,the incidence of autoimmune diseases and autoimmune antibodies between the two groups(P >0.05).(2)There was statistically significant differences in the age of onset,onset symptom,abnormal brain MRI,the location lession spinal cord,length of spinal cord,total-EPVS,AQP4-Ig G positive,MAO,γ-GGT,NLR between the mild and moderate/severe groups.Multivariate logistic regression analysis showed that: Age of onset(OR=1.030,95%CI 1.004-1.055,P=0.022),motor dysfunction at onset(OR=2.576,95%CI 1.075-6.173,P=0.034),length of spinal cord(OR=1.861,95%CI 1.084-3.194,P=0.024),total-EPVS(OR=1.062,95%CI 1.010-1.117,P=0.019)were independent risk factors for moderate/severe disability.A nomogram using the above factors showed good discrimination and calibration abilities,the concordance indexes in the cohort were 0.782.Conclusion:(1)RLO-NMOSD patients suffered more motor and sensory dysfunction at onset than REO-NMOSD patients,while visual dysfunction occured less.RLO-NMOSD patients suffered acute myelitis than REO-NMOSD patients,while optic neuritis occured less.Sphincter dysfunction occured frequently in RLO-NMOSD patients.RLO-NMOSD patients suffered worse than REO-NMOSD patients.Prodromal symptoms and abnormalities brain MRI occured less in RLO-NMOSD patients.The levels of Cys-C,γ-GGT,TSH,TG of RLO-NMOSD patients were higher than those of REO-NMOSD.(2)Age of oset,motor dysfunction at onset,length of spinal cord and total-EPVS were independent risk factors for the moderate/severe disability.