Thursday, July 18, 2013

Anti-NMDA Receptor Encephalitis: Disease Presentation, Progression, and Treatment

Lisa Bolshin, Student, School of Psychology; Joan Read, Faculty, School of Psychology

Anti-NMDA receptor encephalitis is a rare paraneoplastic disease that has been gaining much attention in research due to the varied clinical presentation and nonstandardized treatment regimen. The disease's clinical characteristic presentation progresses through three critical phases from a flu-like malaise, to psychiatric symptomology, to unresponsiveness. Anti-NMDA receptor encephalitis is primarily found in young adult women with a median age of 22, but can occur in a wide range of ages as well as in the male gender. Anti-NMDA receptor encephalitis most commonly presents with an underlying ovarian teratoma although this may take years to be identified. Neurological discrepancies are rare but have been identified. The NMDA receptor, which binds to both glycine and glutamate is implicated in the onset, progression, and treatment response of this disease. The NMDA receptor plays a crucial role in many synaptic processes and is the core mechanism underlying this dysfunctional autoimmune antibody-induced neurological disease. The neurological basis of the disease guides the appropriate treatment regimen. The regulation of the NMDA receptor is the heart of the cellular pathway involved in the pathophysiological modification of excitatory synapse transmission and thus response to proposed interventions. Treatment options focus primarily on tumor removal and intravenous immunoglobulin as a first line response. Treatment also focuses on ameliorating symptomology at various stages of the disease via corticosteroids and antipsychotics. However, recommended effective dosage ranges have yet to be established. The majority of individuals recover fully or have limited improvement, although many report long-term neuropsychological deficits. A small number of cases have resulted in death. There is a significant correlation between time of treatment and cognitive outcome. With early tumor removal and first-line approach treatments, prognosis and recovery is superior.

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