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Clinical Features, Treatment, and Outcome of 500 Patients with Anti-NMDA Receptor Encephalitis (PL01.001)
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2012
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Treatment GuidelinesNeurological DisorderImmunodeficienciesImmunologyClinical NeurologyPathologyClinical FeaturesNeurological ProgressImmunotherapySocial SciencesNeurobiology Of DiseaseAntibody-mediated EncephalitisNeurologyNeuropathologyNeuroimmunologyEncephalitisAnti-nmda Receptor EncephalitisAnti-nmda-receptor EncephalitisNeuroscienceMultiple SclerosisMedicine
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% 45 years). 42% had a tumor (95% teratomas). 55% of females >12 years had ovarian teratoma(s) versus 8% 3 of the following: psychiatric symptoms, memory, speech disorders, 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 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. Supported by: In part by grants from the National Institutes of Health and National Cancer Institute RO1CA89054 (Dalmau), 1RC1NS068204-01 (Balice-Gordon and Dalmau), a McKnight Neuroscience of Brain Disorders award (Balice-Gordon and Dalmau), a KWF fellowship 2009-4451 of the Dutch Cancer Society (Titulaer), and a grant from the Fondo de Investigaciones Sanitarias, FIS, Spain (Martinez-Hernandez). Disclosure: Dr. Titulaer has nothing to disclose. Dr. McCracken has nothing to disclose. Dr. Gabilondo Cuellar has nothing to disclose. Dr. Martinez-Hernandez has nothing to disclose. Dr. Graus has nothing to disclose. Dr. Balice-Gordon has nothing to disclose. Dr. Dalmau has received personal compensation in an editorial capacity for Up To Date, Inc.Dr. Dalmau has received research support from Euroimmun.