Analysis Of Related Factors In The Relapse Of Anti-NMDAR Encephalitis In Children

Objective: The clinical data of children with anti N-methyl-D aspartate receptor encephalitis were retrospectively analyzed.The characteristics and recurrence characteristics of anti N-methyl-D aspartate receptor encephalitis in preschool and school-age children were summarized;meanwhile the related factors of recurrence were discussed.Methods: The clinical data of 88 children with encephalitis resistant to N-methyl-D-aspartate receptor encephalitis who were admitted to the children's Hospital of Medical University Of Chongqing before September 2017 were collected,including gender,age,prodrome,clinical manifestations and related auxiliary examinations.The characteristics of anti NMDAR encephalitis were described.The children were divided into Preschool group(< 6 years old)and school group(over 6 years old)according to the age of onset.Follow up for at least 6-12 months since leaving hospital.If relapse happened,the clinical features of recurrent cases were recorded.After comparing the recurrence and non recurrence group in age,sex,clinical symptoms and EEG,brain MRI and cerebrospinal fluid / blood related antibody,albumin and uric acid data,related risk factors of recurrence were summarized.Results: A total of 88 children with anti NMDAR encephalitis were collected,including 28 in preschool age group and 60 in school group.The incidence of anti NMDAR encephalitis is higher in school age group(P=0.000).The ratio of female:male is 1.6:1.48 cases(55%)had prodromal symptoms.Epileptic seizure is the commonest initial system,14 cases(50%)had epilepsy in preschool agegroup and 26 cases(47%)in the school age group.The most common clinical symptoms were mental and behavioral disorders,motor dysfunction and speech disorder.March was the most onset season in preschool group and May and November in school group.The two groups had no significant difference in prodrome,initial symptoms constituent ratio,main symptoms constituent ratio,electroencephalogram and percentage of abnormal MRI of head and NMDAR antibody titer of cerebrospinal fluid.No tumor was found in 88 cases in tumor related screening.At least 1 relepse was found in 22 cases.The interval of the recurrence was 6.1 months(February-13 months).The first symptoms of recurrence:8 cases(36%)had mental and behavioral disorders,6 cases(27%)epileptic seizures(27%),and 8 cases(36%)involuntary movement.During recurrence,the detection rate of anti NMDAR was higher in cerebrospinal fluid than that in serum antibody.There is no abnormality in tumor screening in recurrence.The average stay in hospital at the time of recurrence was significantly shorter than that in the first course.Famale(P=0.005),cerebrospinal fluid nucleated cell number abnormality(P=0.007),cerebrospinal fluid protein increase(P=0.012)and early acceptance of immunotherapy(P=0.004)were statistically different among relapse group and nonrelapse group.Random forest analysis showed that there was a relatively higher average Gini index in early immunotherapy.There was no significant difference between the two groups in the age of onset,first symptoms,major symptoms,the form and persistence of seizures,electroencephalogram,abnormal head MRI,the number of head MRI demyelinating lesions,the anti NMDAR titer of cerebrospinal fluid,the level of albumin and uric acid before the first onset immunotherapy.Conclusion: The prevalence of children with anti NMDAR encephalitis is higher in school age children.It characterized with typical symptoms:abnormal mental behavior / cognitive dysfunction,epileptic seizures,speech disorders,motor dysfunction,autonomic dysfunction,decrease of consciousness level.Female have more susceptiblity to disease.Nearly half children anti-NMDAR encephalitis had prodromal symptoms.The clinical characteristics Between preschool and school-age children are similar except different onset seasons.Tumor associated autoimmune encephalitis is relatively rare in children.There is about 25% recurrence rate of anti NMDAR encephalitis in children,and more than one relapse in the same case.Mental and behavioral dysfunction / cognitive dysfunction,involuntary movement and epileptic seizures are the first symptoms of recurrence,and the recurrent symptoms are not typical.The length of hospital stay was shorter than that of the first course.The female,increase number of nucleated cells in the cerebrospinal fluid and the abnormality in the amount of cerebrospinal fluid protein and the early immunotherapy are the related factors of recurrence,especially the early immunotherapy.For cases of atypical clinical symptoms or demyelinating lesions,MOG or AQP4 antibodies in cerebrospinal fluid should be screened;For children with recurrence,the necessity of intrathecal injections of hormones and early use of immunosuppressive inhibitors need further study.