Clinical Features Analysis Of Central Nervous System Demyelinating Diseases With Positive MOG Antibody In Children

Objective: To explore the clinical features of the demyelinating diseases of the central nervous system with MOG antibody positive in children.Methods: We retrospectively analyzed the clinical features of 12 ADS patients with MOG antibody positive,including the onset age,gender,clinical manifestations,imaging features,treatment and prognosis.Results :(1)Age at onset: The patients were 2-15.3 years old,and the median age was 6.3 years old,including 9 males and 3 females,with a ratio of 3:1.(2)Presentation at onset: Encephalopathy and cerebral syndrome are the most common symptoms.(3)Clinical symptoms: Most common in encephalopathy and cerebral syndrome,followed by myelitis,optic neuritis,cerebellar symptoms and brainstem symptoms.(4)Cerebrospinal fluid examination: There are 7 cases of cerebrospinal fluid protein increased,8 cases of cerebrospinal fluid leukocyte count were increased,7 cases were <500*106/l.All patients had normal glucose and chloride.(5)Antibody test: serum: 12 patients with MOG antibody were positive,and the titer was 1:10-1:100.Cerebrospinal fluid: 3 cases(3/10)of MOG antibody positive,the titer is 1:1.one case was combined with anti-NMDAR antibody positive.(6)MRI: All cases are visible intracranial lesions,all of them involving the brain white matter,mainly involving the periventricular,subcortical white matter,second involving the basal ganglia region,the brain stem,thalami,cerebellum,optic nerve,gray matter,corpus callosum;In 10 children,MRI was performed on the spinal cord,and in 6 cases,the lesions were seen in the spinal cord.1 case only involved in the cervical spinal cord,5 cases were involved in the cervical and thoracic spinal cord,and 3 cases were long segment lesions.(7)Final diagnose: According to the clinical diagnostic criteria of ADEM,NMOSD,CIS and MS,12 patients were diagnosed as ADEM 4,NMOSD 4,CIS 2,multiple sclerosis 1 case,and 1 case could not be classified.(8)Treatment: after the first onset of the disease,10 patients were treated with methylprednisolone and prednisone,and 10 patients were given IVIG,8 of which were combined with high-dose methylprednisolone.One of the patients was transferred to PICU due to the critical illness,and the plasma exchange was performed.All patients showed improvement after treatment.(9)Followed up: Patients who had been followed up for more than 3 months since the onset of the disease were followed up with 8 patients with no symptoms,and the follow-up time was 9 to 25 months,median 15.5 months.There were 5 cases of recurrence,the interval between the initial relapse and the first onset was 6-14 months,and the median was 9 months.Conclusion:(1)In the ADS with positive MOG antibody,ADEM,NMOSD and CIS are the most common,and MS is rare.(2)In ADS with positive MOG antibody,the onset age is different,the clinical manifestation is different,the onset age is younger,and the clinical manifestation is more likely to be ADEM.The older the onset age,the more likely the clinical manifestation is to be NMOSD.(3)The positive rate of MOG antibody in serum is significantly higher than that of cerebrospinal fluid,and the detection of serum MOG antibody is more sensitive.(4)Efficacy of glucocorticoid and gamma globulin therapy for MOG antibody positive patients,if glucocorticoid and gamma globulin were not effective,plasma exchange and immunosuppressive agents were feasible.(5)In the ADS with positive MOG antibody,the prognosis was mostly good,but some of them had recurrence.