Anti-NMDA Receptor Encephalitis Presenting as an Acute Psychotic Episode in a Young Woman: An Underdiagnosed yet Treatable Disorder

Abstract

Anti-NMDA receptor (NMDAR) encephalitis is a recently identified autoimmune disorder with prominent psychiatric symptoms. Patients usually present with acute behavioral change, psychosis, catatonic symptoms, memory deficits, seizures, dyskinesias, and autonomic instability. In female patients an ovarian teratoma is often identified. We describe a 32-year-old woman who presented with acute psychosis. Shortly after admission, she developed generalized seizures and deteriorated into a catatonic state. Although ancillary tests including MRI, electroencephalogram, and cerebrospinal fluid (CSF) analysis were unremarkable, the presentation of acute psychosis in combination with recurrent seizures and a relentless course suggested autoimmune encephalitis. The patient underwent pelvic ultrasound which disclosed a dermoid cyst and which led to an urgent cystectomy. Plasmapheresis was then initiated, yielding partial response over the next two weeks. Following the detection of high titers of anti-NMDAR antibodies in the CSF, the patient ultimately received second line immunosuppressive treatment with rituximab. Over several months of cognitive rehabilitation a profound improvement was eventually noted, although minor anterograde memory deficits remained. In this report we call for attention to the inclusion of anti-NMDAR encephalitis in the differential diagnosis of acute psychosis. Prompt diagnosis is critical as early immunotherapy and tumor removal could dramatically affect outcomes.

Neuropsychological characterization of three adolescent females with anti-NMDA receptor encephalitis in the acute, post-acute, and chronic phases: an inter-institutional case series

“…memory loss and/or difficulty using everyday objects), and unusual thoughts and behaviors (e.g. delirium, disorganized thinking, obsessive compulsive behaviors, hypersexuality, delusional or paranoid thoughts, and/or auditory and visual hallucinations) within 2 weeks of the flu-like prodrome (Bach, 2014;Dalmau et al, 2008;Guo et al, 2014;irani et al, 2010;Keller, Roitman, Ben-Hur, Bonne, & lotan, 2014;Titulaer et al, 2013;Vahter et al, 2014). Cataplexy-like symptoms (e.g.…”

“…During its later stages, movement disorders (e.g. orofacial dyskinesias, sustained jaw movements, teeth clenching, jaw-opening dystonia, grimacing, intermittent ocular deviation or disconjugation, athetoid dystonic movements, and/or dancing-like movements of the arms), alterations of consciousness, speech problems, and autonomic instability may be present (Bach, 2014;Dalmau et al, 2008;Guo et al, 2014;irani et al, 2010;Keller et al, 2014;Titulaer et al, 2013;Vahter et al, 2014). Seizures are also present in 78% of patients, but are rarely the first presenting symptom (Dalmau et al, 2008).…”

“…Anti-NMDA receptor encephalitis is an autoimmune reaction primarily against the NR1 subunit of NMDA receptors, which plays a critical role in synaptic transmission and learning-related plasticity and is highly concentrated in the forebrain, limbic system, and hypothalamus (Bach, 2014;Chapman & Vause, 2011;li & Tzien, 2009;Onur et al, 2012). The onset of symptoms of anti-NMDA receptor encephalitis is associated with a unique antibody binding to the extracellular portion of the NMDA receptor, resulting in the capping and internalization of receptors from the synaptic surface (Dalmau et al, 2007;Hughes et al, 2010).…”

“…Clinical improvement is also associated with a decrease in cerebrospinal fluid (CSF) and serum antibody titers, though the sensitivity of NMDA receptor antibody testing is higher in CSF than in serum (Dalmau et al, 2008;GresaArribas et al, 2014). Anti-NMDA receptor encephalitis is fatal in roughly 5% of cases, typically the result of neurological and autonomic dysfunction (Keller et al, 2014).…”

Dermoid cysts in adolescents

“…Finally, anti-NMDA receptor encephalitis is another autoimmune disorder usually presenting as acute psychosis and recurrent, relentless seizures. This disorder is more common among adolescents and young women and is effectively treated with immunotherapy and tumour removal [20].…”