Advance in diagnosis and treatment of pediatric anti-NMDAR encephalitis / 国际儿科学杂志

ABSTRACT

Anti-N-methyl-D-aspartate receptor encephalitis is severe diffuse encephalitis caused by cerebrospinal fluid(CSF)antibodies against the GluN1 subunit of the NMDAR.Pediatric NMDAR encephalitis is mainly characterized by neurological symptoms such as dyskinesia, ataxia and epilepsy.Auxiliary examinations of the patients show positive CSF IgG antibodies, EEG slow waves and metabolic changes in FDG-PET/CT.But there is lacking of clinical biomarkers for early diagnosis of children with atypical symptoms and negative antibody.Immunotherapy is effective for most children and early treatment can improve prognosis remarkably.At the same time, antipsychotic drugs and antiepileptic drugs can treat children with psycho behavioral symptoms and seizures.However, some patients are refractory to conventional therapies and have neurological sequela even after the application of multiple immunosuppressants.Therefore, more further studies are needed to clarify the pathogenesis and find biomarkers for early diagnosis of pediatric patients.This article reviews the pathogenesis, clinical characteristics, diagnosis and treatments of the disease.