| Objective: To analyze the clinical characteristics and heterogeneity of late-onset optic neuromyelitis spectrum disease(LO-NMOSD)and provide a basis for clinical diagnosis and treatment of LO-NMOSD.Methods: We retrospectively collected the data of 128 first-onset NMOSD cases who hospitalized in the department of neurology of the second hospital of Lanzhou university from January 2015 to May 2019.General,clinical,laboratory,brain and spinal MRI data were collected and collated.With reference to previous studies,all patients who met the inclusion and exclusion criteria were divided into two groups according to the age of onset: EO-NMOSD(14years ?The age of onset <50years),and LO-NMOSD(The age of onset ?50 years).The clinical characteristics of the two groups were compered to analyze the clinical features and heterogeneity of LO-NMOSD,the potential risk factors and pathogenesis of which were further discussed.Results: Among the 128 patients with NMOSD,31 were male(24.2%)and 97 were female(75.8%),with a male-female ratio of 1:3.12.The median age of onset was 44 years(33.25,53).Besides,there were 78 patients(60.9%)with EO-NMOSD and 50 patients(39.1%)with LO-NMOSD.Fifteen patients(11.7%)were associated with autoimmune disease.But there was no significant difference between the two groups in gender,cause of disease and the co-occurrence rate of autoimmune diseases(P>0.05).The most common core symptom of LO-NMOSD was acute myelitis(70.0%).A small number of patients presented with ON(12.0%),terminal medulla syndrome(2.0%),acute brain or brainstem syndrome(4.0%)and acute myelitis combined with ON(6.0%).Compared with EO-NMOSD,the proportion of acute myelitis in the first core symptoms of LO-NMOSD was relatively higher(70.0%),while ON(12.0%)was relatively rare(P<0.05).The differences in other core symptoms were not statistically significant(P>0.05).Correspondingly,the incidence of limb weakness(76.0%)in the first clinical manifestations of LO-NMOSD washigher,and the symptoms of visual impairment were relatively few(20.0%)(P<0.05).In analysis of the brain and spinal cord MRI data,there were no significant difference in the brain MRI lesions of EO-NMOSD and LO-NMOSD in the fourth ventricle,hypothalamus and aqueduct,area postrema,juxtacortical,and infratentorial(P>0.05),but LO-NMOSD patients had more lesions in the lateral ventricle(46.2%),and the segments of the spinal cord lesion were longer(P<0.05),while no difference in the distribution of spinal cord lesions(P>0.05).In addition,there were no significant differences in serum AQP4-Ab positive rate,antibody titer,blood white blood cell count and various granulocyte ratios between the two groups(P>0.05).However,the titers of serum complement C3 and C4 in LO-NMOSD patients were(1.17±0.06g/l)and(0.29±0.02g/l),respectively,and the titers of serum complement C3(0.95±0.04g/l)and C4(0.19±0.01g/l)in EO-NMOSD patients were significantly lower than those of LO-NMOSD.Serum ?-GGT(28.00(16.00,42.00)U/L)and TG(1.38(0.97,1.74)mmol/L)in LO-NMOSD patients were significantly higher than those in EO-NMOSD(P<0.05).Conclusions:(1)LO-NMOSD were more common in females,and there is no significant difference in gender distribution between LO-NMOSD and EO-NMOSD patients.(2)The most common initial symptom of LO-NMOSD was acute myelitis,and long-term spinal cord lesions were common.There was certain heterogeneity in the clinical manifestations and MRI findings of brain and spinal cord in the two groups.(3)There was no difference between LO-NMOSD and EO-NMOSD in serum AQP4-Ab positive rate and its titer.(4)The risk factors and pathogenesis of the two groups may be different. |
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