Analysis Of Clinical Features And Related Factors Of Recurrence Between Isolated Optic Neuritis And Central Nervous System Demyelinating Disease With Optic Neuritis As The First Manifestation In Children

Objective: To investigate the clinical features and related factors of recurrence of isolated optic neuritis and central nervous system d emyelinating disease with optic neuritis as the first manifestation in c hildren.Methods: Clinical data of 44 patients 72 eyes were enrolled in the study,in Children’s Hospital of Chongqing Medical University from January 2013 to April 2020.The clinical features,auxiliary examination and treatment were retrospectively analyzed,and the prognosis was followed up by telephone or return.Children with brain and/or spinal MRI abnormalities and/or demyelinating disease-related antibodies positive(AQP4/MOG/OB)were classified as central nervous system demyelinating disease with optic neuritis as the first manifestation group,and those with both negative MRI and demyelinating disease-related antibodies were classified as isolated optic neuritis group.The differences between the twogroups were analyzed and compared,and according to whether the recurrence is divided into single phase group and recurrence group,further explore the related risk factors affecting the recurrence of idiopathic ON in children.Results: 1.Among the 44 patiens of ON,there were 26 males and 18 females,males: females is 1.4:1.The onset age ranged from 2 years and 3months to 14 years,with an average age of 7.8±3.0 years.21 patients(47.7%)had prodromal infection.There was no difference between the seasons.2.Among the clinical manifestations,all the children had visual impairment,which was the main manifestation.Secondly,16 cases(36.4%)had ocular pain and eye roll pain,and 12 cases(27.3%)had headache.The brain MRI abnormality rate was 54.5% in the children with headache,and37.9% in the children without headache.There were 20 cases of monocular onset(45.5%),including 8 cases of right eye and 12 cases of left eye.24cases(54.5%)of binocular or monocular successive binocular onset;Binocular involvement was predominant(69.6%)in children < 7 years old,monocular involvement was predominant(61.9%)in children ≥ 7 years old.61 eyes(84.7%)in 72 eyes had initial visual acuity ≤0.1.3.47.5%(19/40)of the children had imaging abnormalities,including 17 brain MRI abnormalities and 6 spinal MRI abnormalities;40.6%(13/32)of the children were positive for demyelinating disease-related antibodies,including AQP4(+)in 3 cases,MOG(+)in 10 cases,and OB(+)in 1 case.According to the grouping method,12 cases were isolated ON and 24 cases were demyelinating disease group.4.Fundus examination was completed in 42 eyes,25 eyes(59.5%)showed papilloma,and 10 eyes(23.8%)showed papilloma pallor/optic atrophy.The abnormal rate of VEP was95.3%(41/43),in which P100 wave absence or amplitude decrease was more common,33 cases(76.7%).5.In the demyelinating disease group,according to imaging findings and demyelinating disease-related antibodies,CIS11 cases,MOG antibody disease 10 cases and AQP4(+)NMOSD3cases were diagnosed.6.Compared with the isolated ON group,the demyelinating disease group was mostly female,and more prodromal infection,P100 wave absence or decrease in amplitude.However,as with other clinical features,there was no statistical difference between the two groups.7.During follow-up,7 children(18.4%)experienced recurrence,all of them in the demyelinating disease group,including 4 NMOSD cases,1MS case,1 central nervous system tumor-like demyelinating disease,and 1MOG antibody related demyelinating disease.8.Compared with the monophasic group,there was no statistical difference in the imaging abnormality rate of the recurrence group.But the rate of demyelinating disease-related antibodies was higher(83.3% VS 30.8%,P=0.029).All the children with positive AQP4 had recurrence,while 20% of the children with positive MOG had recurrence.Conclusion: 1.Among the children only with the manifestationof ON,about 33.3% were isolated ON and 66.7% were central nervo us system demyelinating disease with optic neuritis as the first manif estation.2.Isolated ON is more common in male children,and centr al nervous system demyelinating disease with optic neuritis as the fir st manifestation is more common in female children.3.Binocular inv olvement was more common in children < 7 years old,and monocul ar involvement was more common in children ≥7 years old.4.Mos t of the children with ON recovered well after glucocorticoids and I VIG treatment,among which the recurrence rate of isolated ON child ren was low,while the central nervous system demyelinating disease with optic neuritis as the first manifestation was more likely to relap se.5.Positive antibody for demyelinating disease is a high risk facto r for recurrence.All children with positive AQP4 had recurrence,wh ile 20% of children with positive MOG had recurrence.6.In clinical practice,for children presenting only ON,we should be highly alert to central nervous system demyelinating disease with ON as the firs t manifestation,and actively complete demyelinating disease-related an tibodies and brain/spinal MRI to standardize treatment and prevent recurrence.