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| Retrospective analysis of 29 cases of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis |
| QIAN Ying1 CAO Bingzhen2 |
1.Department of Endocrinology, Ji′nan Military General Hospital, Shandong Province, Ji′nan 250031, China;
2.Department of Neurology, Jin′an Military General Hospital, Shandong Province, Ji′nan 250031, China |
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Abstract Objective To explore the clinical characteristics of anti-NMDAR encephalitis, including clinical manifestation, magnetic resonance imaging (MRI) findings, cerebrospinal fluid changes (CSF) and treatment outcome. Methods Clinical data of 29 cases with anti-NMDAR encephalitis from January 2011 to March 2017 were collected in four medical centers, including Ji′nan Military General Hospital, Qilu Hospital of Shandong University, Shandong Provincial Hospital, and the Second Hospital of Shandong University. The characteristics of cases were analyzed and the patients were followed up. Results The age of 29 patients was ranged from 2 to 70 years, with a mean value of (27.8±13.46). There were 12 males and 17 females. Tumors were detected in 2 males and 3 females during the follow-up periods. The clinical manifestations included psychiatric symptoms (89.7%), seizures (69.0%), dyskinesia (65.5%), hypoventilation (20.7%), recognizant levels drop (44.8%) and autonomic instability (58.6%). Positive rate of CSF routine and biochemistry tests was 70.0%, 96.6% patients with positive anti-NMDA receptor antibodies in serum, and all patients with positive anti-NMDA receptor antibodies in CSF. The EEG records were abnormal in 82.8% patients, with mild degree (20.7%) and middle and serious degree (62.1%) in generalized or frontal to temporal areas. Abnormal MRI changes were observed in 55.2% patients, in which the most common manifestations were T2-FLAIR hyper intensities in frontal and temporal lobes. All patients were treated with immunotherapy, among of whom 5 patitents received immunosuppressive agent (Rituximab, Cyclophosphamide or Azathioprine), 1 patitent received plasmapheresis, 1 patitent diagnosed with small cell carcinoma received chemotherapy, 2 patitents were diagnosed with teratoma and received surgical resection. Death occurred in 3 patitents - during the follow-up period, the remaining 26 (89.7%) cases experienced well recoveries (modified Ranking scale 0-2). Conclusion In anti-NMDAR encephalitis patients, the positive anti-NMDAR antibody in CSF is the most common laboratory findings. The abnormal electroencephalogram (EEG) records occur in most cases and correlated with the severity of anti-NMDAR encephalitis. Most patients receive a substantial recovery by early stage first-line immunosuppressive treatments. Surgical resection and chemotherapy improve prognosis for the cases with tumors.
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