Virus infection and limbic encephalitis: Clinical analysis of 5 cases of anti-N-methyl-D-aspartic receptor encephalitis and literature review

Abstract

Objective To have a profound understanding of anti-N-methyl-D-aspartic receptor (anti-NMDAR) encephalitis, through the clinical analysis of 5 cases of anti-NMDAR encephalitis, and literature review. Methods This is a retrospective analysis. Five cases of anti-NMDA receptor encephalitis treated from May 2010 to June 2015, in the Department of Neurology, Beijing Friendship Hospital affiliated to Capital Medical University, were included in this study. The clinical data, including clinical manifestation, past history, radiological features, serum and cerebral spinal fluid examinations, treatment and prognosis, were analyzed. Results Among the 5 cases, 3 young female and 2 middle-to old-aged male. The clinical features of the onset was mental and behavior disorder, as well as seizure and extrapyramidal features, like facial and limbic involuntary movements or tremor. Coma and hypopnea was severe in 3 young female cases, needing assistance of mechanical ventilator, while the manifestation of 2 male patients was much mild, need not assisted respiration. 1 case had teratoma of ovary, 1 case had Vogt-Koyanagi-Harada syndrome. The anti-NMDA receptor antibody was positive in cerebraospinal fluid of all 5 cases, but in serum of 3 cases, serum and CSF Epstein-Barr virus (EBV) IgM antibody was positive in 1 case, while herpes simplex I virus (HSV-1) IgM antibody positive in another case, and anti-myelin oligodendrocyte glycoprotein (MOG) antibody was seen in serum and CSF in 1 case. The time interval from the onset to treatment was 10-37 d (18.8±9.8 d). IVIG was used in all of the 5 cases, glucocoticoid in 4 cases, and plasma exchange in 3 cases. One case with Vogt-Koyanagi-Harada syndrome, having a long time before diagnosis and treatment, died, while the other 4 cases had good prognosis, and had no relapse. Conclusions Mental and behavior disturbance is common at onset of anti-NMDAR encephalitis. The radiological and lab examination may be normal. It may be accompanied with HSV-1 or EBV infection, anti-MOG antibody may be positive in this disease. Active treatment is important. Key words: Anti-NMDA receptor encephalitis; Teratoma; EB virus; Herpes-simplex virus I; Vogt-Koyanagi-Harada syndrome