| Purpose:Analyze the clinical manifestations,laboratory examinations,and MRI data of patients with myelin oligodendrocyte glycoprotein antibody-associated diseases(MOGAD),and follow up their treatment and prognosis,to figure out the similarity and difference of the onset characteristics of different age groups,so as to optimize the diagnosis process,treatment,and follow-up plan of patients of different age groups,to optimize or even simplify the procedure of diagnosis,therapy and follow-up plan,thereby to improve the prognosis of patients.Materials and Methods:A total of 63 patients diagnosed with MOGAD who were taken therapy in the Department of Neurology and Pediatric Neurology of Shandong Provincial Hospital from November 2017 to September 2021 were retrospectively collected.They were divided into two groups,adulthood-onset group(≥ 14 years old)and childhood-onset group(<14 years old).The clinical data(genders,ages,durations,onset times,clinical manifestations and prognosis),laboratory test indicators(MOG-IgG titer,oligoclonal zone,humoral immunity,cellular immunity indicators,etc.),and MRI images(location,morphology,quantitative characteristics of affected lesions,etc.)were collected for analysis,and the treatment and prognosis of the patients were followed up for statistical analysis.Results:1.There were 39 cases in the childhood-onset group and 24 cases in the adulthood-onset group;the ages of first onset were(7.20±2.89)and(29.42± 15.74)years old,respectively.2.Course of disease:The average time of attacks in the childhood-onset group was 1 time,and the average number of attacks in the adult-onset group was 1 time.At the same time,the proportion of patients with only one attack(monophasic course)in the childhood-onset group was higher than that in the adulthood-onset group(22/39,56.4%vs.14/24,58.3%),and the difference between the two groups was statistically significant(χ2=63.132,P<0.001).3.The clinical phenotype of the first attack:encephalitis or meningoencephalitis were the main first attack phenotypes in the childhood and adult onset groups(34/39,87.2%vs.18/24,75.0%).The proportion of myelitis type in the adult-onset group(9/24,37.5%)was significantly higher than that in the childhood-onset group(4/39,10.3%),and the difference between the two groups was statistically significant(χ2=5.173,P=0.023).4.Recurrent phenotype:the proportion of relapse phenotype consistent with the first phenotype in the childhood and adult-onset groups was(6/9,66.7%vs.9/15,60.0%),and the difference betweel the two groups was statistically significant Significance(χ2=25.111,P<0.001).The recurrence type in the childhood and adult onset groups were encephalitis or meningoencephalitis(6/9,60.0%vs.10/15,66.7%),and optic neuritis(2/9,22.2%vs.5/15),33.3%),myelitis type(1/9,66.7%vs.0/15,0.0%).5.MOGAD often involves cranial brain,optic nerve,and spinal cord,among which craniocerebral involvement accounts for the most in both childhood-onset and adult-onset patients.There was no significant difference between the two groups in the lesions involved(χ2=1.311,P=0.252;χ2=1.529,P=0.216;χ2=3.817,P=0.051).6.MOGAD can involve many parts of the brain.There are some differences in the distribution of brain lesions between the two groups.The proportion of deep white matter involvement is higher in the childhood-onset group.The proportion of deep white matter involvement in the childhood-onset group was significantly higher than that in the adult-onset group(13/39,33.3%vs.2/24,8.3%),and the difference between the two groups was statistically significant(χ2=5.119,P=0.024).7.It shows that encephalitis and craniocerebral involvement,myelitis and spinal cord involvement,and optic neuritis and optic nerve involvement were all consistent(t=4.683,t=8.316,t=12.679;all P<0.001)8.There were more patients significantly with abnormal humoral immunity in the childhood-onset group than in the adult-onset group(18/24,75.0%vs.3/8,37.5%),and there are statistically significantly difference between the two groups(χ2=7.572,P=0.006).9.The average EDSS score before leaving hospital at the first hospitalization was(0.54± 1.70),(2.38±2.38),and the EDSS score of the childhood-onset group was significantly lower than that of the adult-onset group after treatment and the difference between the two groups was statistically significant(t=3.57,P=0.001).In the scores of pyramidal tract function(system 1),brainstem function(system 3),and sensory system(system 4),the EDSS scores of the patients in the childhood-onset group were significantly lower than those in the adult-onset group after treatment,and there are statistically significantly difference between the two groups(t=2.435,_P=0.018;t=2.191,P=0.032;t=3.570,P=0.001).Conclusions:1.MOGAD can develop in childhood and adulthood.The prevalence is higher in children than in adults.2.Encephalitis or meningoencephalitis were the main onsets in the childhood and adult onset groups.The adult group had a higher rate of myelitis.3.The recurrence rate was higher in the childhood-onset group.There was no significant difference in the recurrence pattern between the childhood and adult onset groups,but the recurrence pattern in the childhood onset group was more consistent with the initial onset.4.It is recommended that routine MRI examinations and regular follow-up as a priority,especially cranial MRI.The incidence of MOGAD brain lesions is higher;if there are corresponding symptoms of optic neuritis and myelitis,orbital MRI and spinal cord MRI should be carried out in time.5.There are significantly more children with abnormal humoral immunity than adults.It is estimated that this may be related to the pathogenic mechanism of MOG antigen.MOG antigen leaks into peripheral blood and activates its T cells and B cells to produce MOG antibodies correspondingly and the binding of antigen and antibody causes pathological changes.At the same time,due to the blood-brain barrier being destroyed,B cells in peripheral blood flow into the central nervous system,which can lead to the production of specific MOG antibodies.6.The overall prognosis of patients with childhood onset is better,especially the function of the pyramidal tract,brainstem and sensory system. |
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