Paediatric Anti-N-methyl-D-aspartate Receptor Encephalitis:The First Chinese Case Series

Objective:Since it was first defined in 2007 by Dalmau and colleagues,anti-N-methyl-D-aspartate receptor(NMDAR)encephalitis has been increasingly recognized in children.Europea country and the United States have already launched large-scale clinical observational studies. In China, the anti-NMDAR encephalitis was not reported until 2010,at present,there are no empirical study about large numbers of Paediatric anti-NMDAR encephalitis.By analyzing the clinical data of 46 children who were diagnosed and hospitalized in Children’s Hospital of Chongqing Medical University, We summarized the clinical features and prognosis of Chinese paediatric anti-NMDAR encephalitis for the first time.This study will contribute to the understanding of Paediatric anti-NMDAR encephalitis in Chinese population.Methods:To contribute to define the features of this condition, we describe retrospectively a case series of 46 children who were diagnosed and hospitalized in Children’s Hospital of Chongqing Medical University during the time from Sep 2012 to June 2015.Besides,the SPSS 16.0 was used for statistical analysis.Results:1.Most patients were 3-12 y, but Paediatric anti-NMDAR encephalitis can happen at any age.It is more prevalent in females.2.Oncologic searches were given in 36 cases(78%)and all are negative.Prodromal symptoms were observed in 50% patients.seizures are the first symptoms in approximately two third of children.During the first month of the disease,most patients developed four or more of the 8 categories of symptoms.During the course of their illness,over 90% patients developed changes in consciousness,over 80% patients developed movement disorder,seizresand behavioural changes and/or psychiatric disturbances. sleepewake cycle disturbances and speech disturbances were observed over 50%.A few patients have suicidal tendency.Complications include but are not limited to hospital acquired pneumonia,rhabdomyolysis and paralutic ileus.3.The youngest group(<3 years) seems to have a less severe phenotype and a better outcome compared to older groups.There is no significant difference between other two groups in severity and outcome( P<0.05).4.The rate of positivity of anti NMDAR Ab was higher in the CSF as compared to the serum.The MRI tests revealed no specific results,however,some patients can presented with reversible brain atrophy,which doesn’t have to connect to a poor outcome.An electroencephalogram(EEG) study of a patient with anti-NMDAR encephalitis usually shows generalized diffuse slowing.Focal slowing and focal status epilepticus also may be observed.5.Patients have a poorer outcome after failing first-line immunotherapy(P<0.05).At last follow-up,72% of patients had a good recovery.Relapses occurred in 7% of our children.The most common reason of death is respiratory and circulatory failure caused by somatoform autonomic dysfunction.6.Early diagnosis and a less severe symptom decreases the likelihood of morbidity and mortality.Some patients may experience longstanding neurologic,cognitive issues.7.Rehabilitation may be helpful in paediatric anti-NMDAR encephalitis.Conclusions:The clinical manifestations of paediatric anti-NMDAR encephalitis in our country are various and they are correlated with age.There are differences between the Chinese and the foreign in anti-NMDAR encephalitis such as the age structure,the rate of combined tumor and clinical manifestations. Some patients may experience longstanding cognitive impairment.Early diagnosis and aggressive medical management decreases the likelihood of morbidity and mortality.Rehabilitation may be helpful in Paediatric anti-NMDAR encephalitis.