[P1-115] Clinical Features and Cerebrospinal Fluid Cytokine/Chemokine Profiles of Anti-NMDAR Encephalitis in Children
Objective: To report the clinical features and cerebrospinal fluid (CSF) cytokine/chemokine profiles of pediatric patients with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis
Patients and Methods: From 2013 to 2016, in the preliminary study, antineuronal antibodies (Abs) in sera and CSFs were screened in 200 pediatric patients with suspected autoimmune or inflammatory neuronal disorders. Ten patients were positive for anti-NMDAR Ab by cell based assay (Euroimmun) and diagnosed as anti-NMDAR encephalitis. Clinical features, laboratory data, neuroradiological findings, and therapies were assessed, and 6 CSF cytokines/chemokines were measured by bead based multiplex assay.
Results: The median age at onset was 7 years (range, 2-14 years), 90% were female. Clinical manifestations included psychiatric symptoms in 9; movement disorders in 7; seizure in 6; behavioral problems in 7; memory deficit in 2. All patients aged 0-6 years had speech dysfunction, and all aged 7- years had stereotypic movement. Ovarian teratoma was found in 2 patients. The mean CSF cell count and protein level were 14.9±13.4 (±SD) /μL and 25.1±19.2 (±SD) mg/dL, respectively. Electroencephalography was abnormal in 7 patients. Only one patient had abnormality on brain image. Most patients were treated methyl-prednisolone pulse therapy and intravenous immunoglobulin. One patient received rituximab, 2 necessitated assisted ventilation. The CSF levels of CXCL10 were significantly elevated in some patients compared to control.
Conclusions: Clinical features of NMDAR encephalitis were more neurologic in young children and more psychiatric in adolescents. Increased production of some chemokines in CSF may reflect local production of anti-NMDAR Ab.
Patients and Methods: From 2013 to 2016, in the preliminary study, antineuronal antibodies (Abs) in sera and CSFs were screened in 200 pediatric patients with suspected autoimmune or inflammatory neuronal disorders. Ten patients were positive for anti-NMDAR Ab by cell based assay (Euroimmun) and diagnosed as anti-NMDAR encephalitis. Clinical features, laboratory data, neuroradiological findings, and therapies were assessed, and 6 CSF cytokines/chemokines were measured by bead based multiplex assay.
Results: The median age at onset was 7 years (range, 2-14 years), 90% were female. Clinical manifestations included psychiatric symptoms in 9; movement disorders in 7; seizure in 6; behavioral problems in 7; memory deficit in 2. All patients aged 0-6 years had speech dysfunction, and all aged 7- years had stereotypic movement. Ovarian teratoma was found in 2 patients. The mean CSF cell count and protein level were 14.9±13.4 (±SD) /μL and 25.1±19.2 (±SD) mg/dL, respectively. Electroencephalography was abnormal in 7 patients. Only one patient had abnormality on brain image. Most patients were treated methyl-prednisolone pulse therapy and intravenous immunoglobulin. One patient received rituximab, 2 necessitated assisted ventilation. The CSF levels of CXCL10 were significantly elevated in some patients compared to control.
Conclusions: Clinical features of NMDAR encephalitis were more neurologic in young children and more psychiatric in adolescents. Increased production of some chemokines in CSF may reflect local production of anti-NMDAR Ab.