SESSION TITLE: Critical Care Cases
SESSION TYPE: Medical Student/Resident Case Report
PRESENTED ON: Sunday, October 27, 2013 at 07:30 AM - 08:30 AM
INTRODUCTION: We describe a case of severe anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis that required intensive, multi-modality treatment.
CASE PRESENTATION: A 29 year old female was transferred to our facility with recurrent episodes of sinus arrest. Four years prior she underwent a right oophorectomy for an ovarian mass. Two weeks prior, she noted a flu-like illness, headaches and emotional lability which progressed to an acute delusional psychosis requiring inpatient psychiatric treatment. She developed fevers, tonic-clonic movements, recurrent and prolonged episodes of sinus arrest requiring temporary pacing, and respiratory depression requiring intubation. She was unresponsive to painful stimuli with facial grimacing, tongue thrusting, hypersalivation, hypertonia, hyperreflexia and sustained ankle clonus. Lumbar puncture revealed an opening pressure of 34 cm H20, 62 WBCs/HPF with 95% lymphocytes. Extensive workup for encephalitis including mycoplasma, HSV, rabies, arbovirus, and HIV was negative. Recognizing that 8-15% of ovarian teratomas are bilateral, a CT abdomen/pelvis was ordered. A left adnexal complex cystic mass, later determined to be a mature cystic teratoma (figures 1-2), was discovered and urgently resected. High dose methylprednisolone and IVIG were started for presumed anti-NMDAR encephalitis, later confirmed by positive CSF anti-NMDAR antibody titers. Plasmapheresis and rituximab therapy were initiated nine days later due to lack of improvement. Subsequent signs of recovery noted day 30, and by day 68, only mild neurological sequelae remained.
DISCUSSION: Anti-NMDA receptor encephalitis is a paraneoplastic syndrome caused by autoantibodies against the NMDA receptor. It is commonly associated with teratomas but can occur in the absence of tumor. Phases include: 1) Prodromal phase - Headaches, fevers, flu-like symptoms. 2) Psychotic phase - Agitation, psychosis. 3) Unresponsive phase - Mutism, akinesia, unresponsiveness, catatonia. 4) Hyperkinetic phase - Orofacial dyskinesias, autonomic instability, hypersalivation and seizures. Diagnosis is established by anti-NMDAR antibodies in CSF(1). CSF is clinically consistent with aseptic meningitis. First line treatment consists of high dose steroids, IVIG or plasma exchange. Recovery rates improve dramatically with tumor detection and removal. Second line therapy includes rituximab +/- cyclophosphamide. Recovery may require several months. Over 75% of patients will have a full recovery or mild sequelae.
CONCLUSIONS: Anti-NMDA receptor encephalitis is an important consideration, especially in patients with pronounced psychosis, autonomic instability, hypoventilation, oculogyric symptoms or orofacial dyskinesias. Early diagnosis and tumor removal significantly improves recovery time and response.
Reference #1: Dalmau et al. Clinical experience and laboratory investigations in patients with anti-NMDAR encephalitis. Lancet Neurol 2011;10:63-74.
DISCLOSURE: The following authors have nothing to disclose: Stacie Cook, Joseph Pitcher, Alicia Zukas, Hope Richard, Lisa Brath
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