Anti-N-methyl-D-aspartate receptor encephalitis in adults: a systematic review and analysis.

PURPOSE: To analyze predictors of treatment outcome for anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis in adults. METHODS: We performed a comprehensive literature search of PubMed, PsycInfo, and OVID. We included 424 patients from case reports and case series. Demographics, anti-NMDAR antibodies, prodromal and presenting symptoms, diagnostic workup, and treatment variables were recorded. Inferential analyses were performed in the subset (n = 299) of those with known treatment outcomes. Multivariate multinomial logistic regression analysis for treatment outcome compared full recovery versus partial recovery and full recovery versus death. RESULTS: Treatment outcomes consisted of 34.67% full recovery (n = 147), 30.90% partial recovery (n = 131), 4.95% death (n = 21), and 29.48% unknown (n = 125). Speech/language abnormality and abnormal electroencephalogram (EEG) were each significantly associated with a higher relative risk for a full recovery. Treatment with intravenous immunoglobulin and plasmapheresis were each significantly associated with a higher relative risk for partial recovery. The analysis comparing death to full recovery found that catatonia was significantly associated with a lower relative risk for death. Increased age, orofacial dyskinesia, and no tumor removal were each significantly associated with a higher relative risk for death. CONCLUSION: Increased age, orofacial dyskinesia, and no tumor removal were associated with a higher relative risk for death in anti-NMDAR encephalitis in adults. Clinicians should monitor and appropriately treat anti-NMDAR encephalitis with these findings to minimize the risk of death.

Anti-NMDA Receptor Encephalitis Masquerades as Psychosis: A Case Report.

A 28-year-old male patient with an unclear history of psychosis was admitted to the inpatient psychiatric unit. He presented with auditory hallucinations, agitation, and bizarre and disorganized behavior. He was treated with antipsychotic medications without improvement. Magnetic resonance imaging of the brain showed hyperintensities throughout the brain parenchyma. Investigations for infectious, metabolic, autoimmune, and malignant etiologies were negative. Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis was suspected. Cerebrospinal fluid (CSF) and serum NMDA receptor antibody testing were performed. Both tests were positive, confirming anti-NMDA receptor encephalitis. The patient was treated with intravenous immunoglobulin and methylprednisolone, which resulted in the resolution of his psychosis. In the case of unexplained psychosis associated with seizures, early screening using serum and CSF testing for anti-NMDA receptor antibodies and brain magnetic resonance imaging may be an important diagnostic tool for detecting anti-NMDA receptor encephalitis. Detailed investigations of CSF and serum should be performed to rule out infectious, metabolic, and autoimmune causes. Imaging studies should also be performed to identify any tumors such as a teratoma. This approach may help identify patients with anti-NMDA receptor encephalitis masquerading as psychosis. Early diagnosis and treatment including intravenous steroids, immunosuppressants, plasmapheresis, and removal of any teratoma if present in patients with anti-NMDA receptor encephalitis can improve the overall outcome.

Anti-N-Methyl-D-Aspartate Receptor (NMDAR) Encephalitis in Children and Adolescents: A Systematic Review and Quantitative Analysis of Reported Cases.

OBJECTIVE: This pooled analysis, from a systematic review, examines anti-N-Methyl D-Aspartate Receptor (NMDAR) encephalitis presentation in children and adolescents. METHOD: A comprehensive literature search from database inception through December 31, 2019, using PubMed, PsycInfo, and OVID was performed. Case reports and case series were included. Sample characteristics are described. Prodromal and presenting symptoms between partial recovery and full recovery are compared. The association between presenting symptoms and abnormal MRI, abnormal EEG, and tumor presence are determined. RESULTS: The sample (n=283) had a mean age of 10.8 years with 75.3% females. The most common prodromal and presenting symptom was seizure (29.7% and 63.3%, respectively). Partial and full recovery did not differ for prodromal and presenting symptoms. Multivariate logistic regression analysis found that (1) delusion were significantly associated with higher odds and aggressive behavior was associated with lower odds for abnormal findings on magnetic resonance imaging (MRI); (2) waxing and waning of symptoms were significantly associated with higher odds for abnormal electroencephalograms (EEG), and (3) increased age and psychosis were each significantly associated with increased odds, and sleep disturbance and orofacial dyskinesia with lower odds for tumor presence. CONCLUSION: Given the pattern of findings, routinely obtaining MRI and EEG should be considered for anti-NMDAR encephalitis in children and adolescents presenting with delusion and waxing and waning of symptoms, respectively. Investigation of tumors should be considered in patients with anti-NMDAR encephalitis especially when psychosis is present.

OBJECTIFS: La présente analyse combinée, tirée d'une revue systématique, examine la présentation d'une encéphalite anti-récepteur de N-méthyl-D-aspartate (NMDA) chez les enfants et les adolescents. MÉTHODE: Une recherche détaillée de la littérature à compter des débuts des bases de données jusqu'au 31 décembre 2019, dans PubMed, PsycInfo, et OVID a été menée. Les rapports de cas et les séries de cas sont inclus, et les caractéristiques de l'échantillon sont décrites. Les symptômes avant-coureurs et ceux présentés entre le rétablissement partiel et complet sont comparés. L'association entre les symptômes présentés et une IRM anormale, un EEG anormal, et la présence d'une tumeur est déterminée. RÉSULTATS: L'échantillon (n = 283) avait un âge moyen de 10,8 ans et était à 75,3 % de sexe féminin. Les symptômes avant-coureurs et présentés les plus communs étaient les convulsions (29,7 % et 63,3 %, respectivement). Le rétablissement partiel et complet ne différait pas pour les symptômes avant-coureurs et présentés. L'analyse de régression logistique multivariée a constaté que (1) le délire était significativement associé à des probabilités plus élevées, et le comportement agressif à des probabilités plus faibles de résultats anormaux à l'imagerie par résonance magnétique (IRM); (2) les variations des symptômes étaient significativement associées à des probabilités plus élevées d'électro-encéphalogrammes (EEG) anormaux; et (3) l'âge et la psychose avancés étaient chacun significativement associés à des probabilité accrues, mais le trouble du sommeil et la dyskinésie bucco-faciale étaient eux associés à des probabilités plus faibles de la présence d'une tumeur. CONCLUSION: Étant donné le modèle des résultats, obtenir automatiquement une IRM et un EEG devrait être envisagé chez les enfants et les adolescents présentant un délire et une variation des symptômes, respectivement. L'investigation de tumeurs devrait être envisagée chez les patients de l'encéphalite anti-récepteur NMDAR surtout en présence de psychose.