| Objective: To explore the clinical heterogeneity and survival status of optic neuromyelitis optica spectrum disease(NMOSD)with different core symptom onset;to analyze the relevant factors affecting the prognosis and survival quality of NMOSD patients.Methods: The cases of 126 patients with a diagnosis of NMO or NMOSD who visited the Department of Neurology at the Sixth Medical Center of the PLA General Hospital between2012 and 2022 were collated and screened according to the inclusion and exclusion criteria,of which 6 cases could not be counted and the remaining 93 cases were included in the study data set.Demographic,clinical,imaging and laboratory data were collected from the enrolled cases.The study subjects were divided into 4 groups according to the core symptoms at the onset of the disease: ON group,LETM group,ON+LETM group and combined brain lesion group,and the differences in clinical manifestations,imaging,laboratory indices and survival status were compared among the groups.Results: 1.General:(1)Gender: 15.1%(14 /93)in males and 84.9%(79/93)in females,male:female = 1:5.62;(2)Age of onset: mean age of onset(39.95 ± 16.81)years,minimum age of onset was 3 years and maximum was 69 years;(3)Follow-up time: 12-120 months,median follow-up time was 54(27,81);(4)Onset triggers: 26.9%(25/93)had onset triggers at first onset,37.8%(25/67)had onset triggers at relapse,common triggers at first onset were cold and fever,common triggers at relapse were exertion;(5)Core symptoms at first onset:29.0%(27/93)in the ON group,41.9%(39 /93)in the LETM group,7.5%(7/93)in the ON+LETM group and 21.5%(20/93)in the combined brain lesion group;(6)Recurrence:72.0%(67/93)recurred during the follow-up period,14.9%(10/67)in men and 85.1%(57 /67)in women,male:female = 1:5.70,and the mean age of onset of recurrence was(40.45 ± 16.68)years.2.Clinical characteristics of NMOSD with different core symptoms onset:(1)General clinical characteristics and misdiagnosis analysis:(1)there were no statistical differences between the groups in terms of gender,age,ethnicity,health insurance mode,marital status,and age at first presentation(P > 0.05);(2)there were statistical difference in terms of whether misdiagnosis exists(P < 0.001),patients with combined brain lesions have the highest misdiagnosis rate and are easily misdiagnosed as digestive system diseases or cerebral infarction;(2)Comparison of clinical characteristics of first onset:(1)there were statistical differences existed in terms of season of first onset and sensory abnormalities(P =0.046,P < 0.001);32.3%(30/93)with summer onset were the most common;57%(53/93)had sensory abnormalities,with89.7%(35/39)in the LETM group,accounting for the highest percentage;(2)the time to diagnosis was statistically different(P = 0.041),with 2.0(1.0,10.0)months in the combined brain lesion group,which was the longest time to diagnosis;(3)the shape of lesions was statistically different(P = 0.025),with patchy in the ON group,striped in the LETM and ON+LETM groups,and striped + patchy in the combined brain lesion group.(4)the treatment regimen was statistically different(P=0.019),with hormone alone being the most common in the ON group and combined brain lesion group,and hormone +immunosuppressive therapy in the LETM and ON+LETM groups,with no statistical difference in recovery after treatment.(3)Comparison of clinical characteristics of recurrence:(1)72.04%(67/93)recurred during the follow-up period,77.8%(21/27)in the ON group,with the highest recurrence rate,74.4%(29/39)in the LETM group,71.4%(5/7)in the ON+LETM group,and 60%(12/20)in the combined brain lesion group,with the lowest recurrence rate;(2)there was a statistical difference in pain,with the ON group being more likely to be associated with pain compared to the other groups.(4)Comparison of current status and quality of survival during the follow-up period:(1)10.7%(10/93)with no residual symptoms,51.6%(48/93)with improved and stable disease,17.2%(16/93)with chronic progression,and 20.4%(19/93)with death.Further comparison between groups revealed that the combined brain lesion group had the highest mortality rate(30.0%)and worse prognosis compared with other groups,and the ON group had the highest rate of no residual symptoms(14.8%)and better prognosis.(2)quality of survival scores during the follow-up period were statistically different in terms of pain and stress(P < 0.05),and further analysis revealed that patients in the LETM group had lower scores in these two areas than the other groups.3.Analysis of prognosis-related influencing factors: Using the EDSS score during the follow-up period as the dependent variable,variables that may have an effect on patient prognosis from univariate analysis were included in the multiple linear regression analysis and found that after excluding other confounding variables,the treatment regimen for the first episode [B=-1.815,95% CI(-3.300,0.330),P=0.017] may be the prognosis of NMOSD patients independent influencing factors.4.Exploration of influencing factors related to quality of survival: Using the total quality of survival score as the dependent variable,variables that had an effect on patient quality of survival by univariate analysis were included in multiple linear regression analysis and found that after excluding other confounding variables,AQP4 antibody positivity [B=10.117,95% CI(2.443,17.791),P=0.011],sleep disturbance [B= 2.030,95% CI(-4.898,8.958),P=0.046],anxiety-depressive state [B=-5.315,95% CI(-12.187,1.558),P=0.026],and EDSS score [B=-3.439,95% CI(-4.883,-2.045),P<0.001] may be independent influences on the quality of survival in patients with NMOSD.Conclusion: 1.Clinical characteristics of NMOSD starting with different core symptoms:(1)NMOSD starting with combined brain lesions took the longest time to confirm the diagnosis,had the highest misdiagnosis rate,and was easily misdiagnosed as gastrointestinal disease and cerebral infarction;(2)NMOSD starting with LETM was prone to sensory abnormalities;(3)the combined brain lesion group had the highest mortality rate,and the ON group had the highest rate of no residual symptoms.2.NMOSD prognosis Related influencing factors: first-onset treatment regimen may be an independent influencing factor for NMOSD prognosis,and patients with the addition of immunosuppressants had lower EDSS scores and better prognosis compared with hormone therapy alone.3.NMOSD patients survival quality related influencing factors: AQP4 antibody positivity,sleep disturbance,anxiety and depression status,and EDSS scores during the follow-up period may be an independent influencing factor for NMOSD patients survival. |
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