Clinical Characteristics Of Pediatric Myelin Oligodendrocyte Glycoprotein Antibody-associated Disorders

objective:To explore the clinical characteristics and differences between children with positive and negative MOG antibodies in idiopathic inflammatory demyelinating diseases(IIDDs)of central nervous system.Methods:The 42 children with IIDDs who were admitted to the Department of Neuroendocrinology and Pediatrics of Qingdao University Hospital from January 2015 to December 2020 were divided into two groups:MOG+group(18 cases)and MOG-group(24 cases)according to the presence of MOG antibodies.Retrospective analysis of its clinical characteristics,including age,sex ratio,symptoms,signs,clinical phenotypes,magnetic resonance(MRI)and electroencephalogram,characteristics of cerebrospinal fluid,treatment measures,efficacy and follow-up outcome.MOG antibody is detected by cell transfection technology(CBA).The quantitative data were compared by independent sample T-test or Mann-Whitney U-test,and the qualitative data were compared by quartileχ2 test or Fisher exact test.Results:(1)The age of onset in the MOG+group was 7.77±3.59 years,the male-to-female ratio was 1:0.8,and the follow-up duration ranged from 8-42.5 months.MOG-group was 6.66±3.96 years old,the male-to-female ratio was 1:0.4,and the follow-up duration ranged from 4-71 months,there was no statistically significant difference(P>0.05).(2)The most common symptoms of the two groups of children were neurological symptoms,and the most common clinical phenotype was acute disseminated encephalomyelitis(ADEM).Mental and behavioral abnormalities and visual symptoms were more common in the positive group than in the MOG-group(χ2values were5.948,5.537,P<0.05).Encephalitis was mostly diagnosed in the MOG+group,including unilateral cortical encephalitis and encephalitis with reversible lesions of the corpus callosum,and demyelinating encephalopathy was mostly diagnosed in the MOG-group,the difference were statistically significant(P<0.05).(3)The cerebral cortex and subcortical white matter were extensively affected in the two groups,deep white matter and gray matter could also be involved.Among them,thalamic lesions were more common in the MOG+group,and the difference was statistically significant(χ2=6.300,P<0.05).(4)In the two groups,the EEG of the children with epileptic seizures may have abnormalities such as slow waves and spikes,and the EEG of the children without seizures was normal.Brain magnetic resonance examination showed that all children with cortical lesions in the MOG+group had epileptic seizures,while 3 children with cortical lesions in the MOG-group had only 1 epileptic seizure.(5)In MOG+group,18 children were positive for MOG antibody in serum and 10 in cerebrospinal fluid.Antibody titer in cerebrospinal fluid was lower than that in serum.(6)The cerebrospinal fluid routine and biochemical results of the two groups showed mild to moderate increase in white blood cell count and protein level.The MOG+group’s cerebrospinal fluid white blood cell count was ranged from 0-170×10~6/L,the average protein level was 321.09 mg/L,and the MOG-group’s cerebrospinal fluid white blood cell count was ranged from 0-141×10~6/L,the average protein level is 353.42mg/L.Chloride levels in the two groups may decrease slightly,and glucose levels may be normal or slightly elevated.The above data is not statistically different between the two groups(t value-1.534—1.862,P>0.05).(7)Most of the children were given high-dose IVIG and/or methylprednisolone therapy in acute phase.More than 70%of the children’s symptom disappeared rapidly in acute phase,and only 4cases in the MOG+group remained Occasional headaches and dizziness at the end of the follow-up,and 2 cases in the MOG-group had severe sequelae such as hemiplegia and ataxia,suggesting that pediatric MOGAD have a better outcome.(8)The recurrence rate of MOG+group was 22.2%(4/18).The average relapse interval is 13.75 months,when the recurrence occurs,the MOG antibody is still positive.The recurrence rate of MOG-group was 12.5%(3/24),the average relapse interval is 10.60 months.Conclusion:In IIDDs,children with encephalitis should be screened for MOG antibodies;Children with MOGAD have more prominent mental behavior and visual symptoms;The phenotype of encephalitis is more common;Thalamic lesions in MOGAD are more common than those in children with MOG antibody negative IIDDs;Children with cortical lesions in MOGAD are more likely to have seizures.