OBJECTIVE: To provide the clinical features and suggest treatment guidelines for anti-NMDA-receptor encephalitis.BACKGROUND: Anti-NMDA-receptor encephalitis is the most common and best characterized antibody-mediated encephalitis. We provide the clinical features, treatment, and follow-up of 500 patients.
DESIGN/METHODS: Cohort study, analysis of demographics, onset, treatment, and long-term follow-up.
RESULTS: 82% were female. Median age was 21 years (range 1-85; 36% <18 and 4% >45 years). 42% had a tumor (95% teratomas). 55% of females >12 years had ovarian teratoma(s) versus 8% <12 years. In patients <12 years old the most frequent initial symptoms were abnormal behavior, seizures, and movement disorders (36%, 35%, 14%), while in adults were abnormal behavior and memory problems (70%, 13%). 90% of patients had >3 of the following: psychiatric symptoms, memory/speech disorder, seizures, dyskinesias, decreased level of consciousness, autonomic instability, or hypoventilation. Within the first month, movement disorders and ataxia were more frequent in children (92% and 17% vs 70% and 2%, p<0.001 both), while memory problems and hypoventilation predominated in adults (84% and 42% vs 68% and 16%, p=0.008 and p<0.001).Immunotherapy (93%) and tumor removal (when appropriate) resulted in full recovery or substantial improvement in 61% of patients at 8 months, and 77% at 24 month follow-up; 7% died. Early treatment (1st month) led to better outcome (75% vs 64%, p=0.001). If 1st line immunotherapy (steroids, immunoglobulins and/or plasma exchange) failed, 2nd line treatment (rituximab or cyclophosphamide) significantly improved outcome compared with no treatment or repeating 1st line drugs (56% vs 27%, p=0.006). Relapses occurred in 14%, 73% of them in patients without teratoma.
CONCLUSIONS: Anti-NMDA-receptor encephalitis is a severe but treatable disorder of predominantly young individuals. Prompt treatment improves outcome. If initial immunotherapy fails, second-line treatment is usually effective. 75% of patients have full/substantial improvement although the process of recovery can take >24 months.
Maarten Titulaer studied at the Utrecht University (the Netherlands) and completed his residency in Neurology at Leiden University (The Netherlands). He obtained his PhD thesis ‘cum laude’ (Prediction of Small Cell Lung Cancer in the Lambert-Eaton Myasthenic Syndrome, advisor Prof. J. Verschuuren) at Leiden University. His research on screening techniques for lung cancer in LEMS was rewarded with the Dutch ‘Prinses Beatrix Fonds Prize for Neuromuscular Diseases 2008’, and his thesis with the ‘Vera Bonta Prize 2009-2010’ for the best thesis in lung cancer. As part of his PhD, he studied at the Weatherall Institute of Molecular Immunology in Oxford (Prof. A. Vincent and B. Lang, United Kingdom), supported by a grant from the European Neurological Society. He was a clinical fellow of Neuro-oncology at the Daniel den Hoed Cancer Center, Rotterdam (Prof. M. van den Bent, NL). Currently, Dr.Titulaer is a clinical research fellow of Neuro-oncology and Immunology (Prof. J. Dalmau) at the University of Pennsylvania, Philadelphia, PA, supported by a fellowship award from the Dutch Cancer Society.
Disclaimer: The opinions expressed in this posting are those of the author only and do not represent the views of the American Academy of Neurology or any of its affiliated subsidiaries.
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