Clinical analysis of anti-N-methyl-D-aspartate receptor encephalitis in 15 children

doi:10.19538/j.ek2017070613

Abstract：Objective To explore the clinical characteristics of anti-N-methyl-D-aspartate receptor(anti-NMDAR) encephalitis in children.Methods Between April 2015 and December 2015,fifteen children were diagnosed with this disorder at the Department of Neurology,Children's Hospital of Fudan University.We retrospectively analyzed the data of clinical characteristics and laboratory examinations.Results (1)There were seven females and eight males,the median age of onset was 7 years old,ranging from 8 months to 12 years.(2) Clinical characteristics were as follows:3/15 (20％) had impaired consciousness along with convulsion,and 4/15 (26.7％) had emotional and behavioral changes at the onset of disease.Seizures occurred in all the 15 children and status epilepticus in 3 cases.Facial and limb involuntary movements occurred in 12/15 (80％).Seven cases(46.7％) demonstrated uroschesis.Three had hypoventilation.Two (13.3％) were secondary to virus infection.(3) Imaging and laboratory examination showed that the white blood cell of cerebrospinal fluid (CSF) in 5/15 cases (33.3％) were moderately elevated.The protein of cerebrospinal fluid (CSF) was obviously increased in 2 patient(13.3％).IgG index was detected in 10 patients and increased in 4/10(40％).All had cerebrospinal fluid antibodies.MRI was abnormal in 10/15 (66.7％).EEG typically showed diffuse background slowing,while no extreme delta brush was observed.One case (6.7％) had a mass in ovary.All patients received intravenous methylprednisolone and immunoglobulins (IVIG).One case (6.7％) received plasma exchange.Follow-ups lasted for 1 to 6 months.The functional outcomes of 9 patients were favorable,mRS being 0 to 2.Conclusion Due to the lack of specificity of clinical symptoms and laboratory examination,this disorder is difficult to diagnose at the onset.The disease can be diagnosed by the specific anti-NMDAR antibody in the cerebrospinal fluid.Tumors and delt brush are rarely found in children with anti-NMDAR encephalitis.Early diagnosis and timely initiation of immunosuppressive treatment will result in favorable outcome in the majority of the patients.