Anti-N-methyl-D-aspartate-receptor (NMDAR) encephalitis (Updated – Mar 2014.)

van de Riet EH, Esseveld MM, Cuypers L, Schieveld JN. Anti-NMDAR encephalitis: a new, severe and challenging enduring entity. Eur Child Adolesc Psychiatry. 2013 May;22(5):319-23.

“Two girls, 15- and 17-year-old, were consecutively and involuntarily admitted to the local child and adolescent psychiatric hospital with severe first onset psychosis. Due to refractory agitation, ongoing psychosis and insomnia, catatonic features, autonomic instability and the need for one-on-one guidance, the first girl was transferred to the PICU of an academic tertiary hospital and anti-NMDA receptor encephalitis was diagnosed. Given this experience nursing staff suspected, due to similarities in the clinical presentation and course, anti-NMDA receptor encephalitis in the second girl also and this proved to be true. The main clinical features, pharmacological and non-pharmacological treatment strategies and outcomes are presented and discussed. Perhaps, one ought to suspect anti-NMDA receptor encephalitis in every case of severe first onset psychosis with catatonic features.”

Full-text for Emory users.

Armangue T, Titulaer MJ, Málaga I, Bataller L, Gabilondo I, Graus F, Dalmau J; Spanish Anti-N-methyl-D-Aspartate Receptor (NMDAR) Encephalitis Work Group. Pediatric anti-N-methyl-D-aspartate receptor encephalitis-clinical analysis and novel findings in a series of 20 patients. J Pediatr. 2013 Apr;162(4):850-856.e2.

“Findings from this study suggest that in children the first symptom of anti-NMDAR encephalitis may be different from that of the adults (more neurologic in children, more psychiatric in adults); the development of a monosymptomatic illness is extremely rare (except in relapses), and although the disease is potentially lethal, most patients respond to immunotherapy. Moreover, second line immunotherapy, mostly including rituximab, is often effective and well tolerated. A notable feature not previously reported in children is the EEG pattern named extreme delta brush. Further studies are needed to determine the frequency of NMDAR antibodies in patients with post-HSE choreoathetosis and whether this disorder is, in fact, anti-NMDAR encephalitis.”

Full-text for Children’s and Emory users.

Mann A, Machado NM, Liu N, Mazin AH, Silver K, Afzal KI. A multidisciplinary approach to the treatment of anti-NMDA-receptor antibody encephalitis: a case and review of the literature. J Neuropsychiatry Clin Neurosci. 2012 Spring;24(2):247-54.

“Psychiatrists should have a high suspicion for anti-NMDAR encephalitis in young, female patients with acute seizures, associated with abrupt behavioral changes or dyskinesias. These symptoms, coupled with evidence of CSF lymphocytic pleocytosis or oligoclonal bands, an EEG with generalized slow-wave activity, and nonspecific, hyperintensities on brain MRI help support clinical suspicions. Full recovery is obtained more often in patients who are started early on immune-suppressant therapy. Benzodiazepines and ECT may be warranted for treatment of progressive, life-threatening autonomic instability and muscle rigidity associated with malignant catatonia. Additional research is necessary to determine the optimal treatment for this disease and its psychiatric manifestations. The best outcomes result from cooperation within a multidisciplinary team to address the needs of such medically complex patients.”

Full-text for Emory users.

Peery HE, Day GS, Dunn S, Fritzler MJ, Prüss H, De Souza C, Doja A, Mossman K, Resch L, Xia C, Sakic B, Belbeck L, Foster WG. Anti-NMDA receptor encephalitis. The disorder, the diagnosis and the immunobiology. Autoimmun Rev. 2012 Oct;11(12):863-72.

“Anti-NMDAR encephalitis is a newly characterized syndrome with a progressive, predictable clinical course and the possibility of effective treatment. Accurate and timely diagnosis is critical to selection and implementation of treatments, and optimal patient outcomes. Outcomes are improved with early diagnosis via indirect immunofluorescence or cell-based assays, and the rapid and appropriate administration of immunosuppressant and anti-psychotic therapies. Three possible scenarios accounting for the immunopathogenesis of anti-NMDAR encephalitis are presented, with the most probable one being that of paraneoplastic autoimmunity. Future efforts in this disorder should focus on elucidating the mechanisms that contribute to initiation of this antibody response, as well as exploring the role of tumors, infectious triggers and immune-reactivation. Finally, accessible tools need to be developed that allow for reliable identification of specific antibody markers against synaptic proteins.”

Full-text for Emory users.

Day GS, High SM, Cot B, Tang-Wai DF. Anti-NMDA-receptor encephalitis: case report and literature review of an under-recognized condition. J Gen Intern Med. 2011 Jul;26(7):811-6.

“Anti-N-methyl-D-aspartate receptor (anti-NMDA-R) encephalitis is an immune-mediated syndrome that remains under-recognized despite a growing body of literature. This syndrome has been predominantly described in young females with a constellation of symptoms, including personality changes, autonomic dysfunction and neurologic decompensation. It is commonly associated with mature ovarian teratomas. We describe the classic presentation of anti-NMDA-R encephalitis in three dramatically different patients: Case A, a young woman with ovarian teratoma; Case B, the eldest case reported to date; and Case C, a young male with no identifiable tumor. We review the literature summarizing the differential diagnosis, investigative approach, treatment options and challenges inherent to this disorder. We advocate good supportive care, involvement of multiple health disciplines and use of immune-modulating therapies in patient management. These cases underscore the need for increased awareness and high diagnostic suspicion when approaching the patient with suspected viral encephalitis.”

Free full-text.

Chapman MR, Vause HE. Anti-NMDA receptor encephalitis: diagnosis, psychiatric presentation, and treatment. Am J Psychiatry. 2011 Mar;168(3):245-51.

“A growing literature describes the clinical presentation, diagnosis, and pathophysiology of anti-NMDA (N-methyl-d-aspartic acid) receptor encephalitis. Once conceptualized as a condition primarily affecting adult women and frequently associated with tumors, anti-NMDA receptor encephalitis is increasingly recognized in males, in children, and in the absence of tumors. Despite the expanding knowledge base, much remains to be understood about effective treatments. The little available information on management focuses primarily on tumor management and immunotherapy to control the antibody response. Limited information is available on the management of psychiatric symptoms in these complex, often critically ill patients. Here we describe, through an illustrative case example, the clinical characteristics of anti-NMDA receptor encephalitis in an adolescent male. We also outline other disease processes to be considered in the differential diagnosis and describe currently used interventions, including psychotropic medications.”

Free full-text.

More PubMed results on anti-NMDA-R encephalitis.

Created 6/10/13, revised 3/13/14. 

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