Clinical Study On Pediatric Neuromyelitis Optica Spectrum Disorder

Objective:Pediatric neuromyelitis optica spectrum disorder?p-NMOSD?is a kind of rare disease in pediatric neurology with diverse manifestations,which is likely misdiagnosed.Our aim is to summarize the clinical characteristics of p-NMOSD and deeply understand it comparing with the pediatric multiple sclerosis?p-MS?.Methods:Twelve cases of p-NMOSD were recruited at the department of pediatric neurology of the first hospital Jilin university from October 1^st,2015 to October 1^st,2018.A retrospective study on p-NMOSD clinical characteristics was carried,meanwhile,a comparative analysis between the p-NMOSD patients and 15 cases of p-MS collected at the same time window was conducted.Clinical characteristics include commen information,clinical manifestation,cerebrospinal fluid test,autoantibody,imaging examination,treatment and prognosis.Results:Among 12 p-NMOSD patients,the male to female ratio was 1:1,the average onset age was 8.6±3.8 yr,the median diagnostic time was 1.5month.Ten cases had single course,and two were the recurrent courses.Seven cases were derived from upper respiratory tract infection and five cases had no causative factors.Among the 15 cases of p-MS patients,the male to female ratio was 1:2.The average age of onset was 8.5±4.4 yr,and the median diagnostic time was 6.0 month.Eleven cases presented relapse-alleviating courses.Among the p-MS patients,eleven cases were correlated with upper respiratory tract infection and four cases had no inducement.The median diagnostic time of p-NMOSD is significantly shorter than that of the p-MS group,and the recurrence rate is significantly lower than that of the p-MS group?p<0.05?.While there is no difference between the two groups in the average age of onset,gender,and predisposing factors.The main initial symptom of the p-NMOSD patients was optic nerve lesion?50.0%?,the symptom of brain damages commonly present early in the p-MS group?53.3%?,meanwhile,both groups had spinal cord lesion?33.3%vs 26.7%?.The longitudinally extensive transverse myelitis appeared in all p-NMOSD patients,which is significantly different from the p-MS group?p<0.05?.Brain lesions present in 91.7%of the p-NMOSD group,especially in subcortical area and subcortical white matter.The leukocyte count and protein level in cerebrospinal fluid increased in some p-NMOSD patients.The aquaporin-4 antibody?AQP4-Ab?positive rate was low,and the oligoclonal band?OB?was positive in some p-NMOSD patients,but there is no significant difference compared with the p-MS group?p>0.05?.Some p-NMOSD patients who were negative aquaporin-4 antibody could detect myelinoligodendrocyte glycoprotein antibody?MOG-IgG?.Conclusions:1.Compared with p-MS group,the median diagnostic time of the p-NMOSD group is significantly shorter,and the recurrence course is also significantly lower.There is no statistically difference in the average age of onset,gender,and predisposing factors between the two groups.2.The main initial symptom of the p-NMOSD was optic nerve lesion,while the symptom of brain damages commonly present early in the p-MS group,meanwhile,both groups had spinal cord lesion.3.The longitudinally extensive transverse myelitis was the specific symptom of p-NMOSD patients.The brain lesions occur mainly in the subcortical area and subcortical white matter,but there is no specificity.4.The p-NMOSD patients had lower rate of positive AQP4-Ab than the adult.Some patients who were negative with AQP4-Ab could detect MOG-IgG.The leukocyte count and protein level in cerebrospinal fluid increased in some p-NMOSD patients.