SESSION TITLE: Miscellaneous Student/Resident Case Report Posters II
SESSION TYPE: Medical Student/Resident Case Report
PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM
INTRODUCTION: Status epilepticus can be a difficult entity to manage in the ICU. Some etiologies are rare and require a high index of suspicion.
CASE PRESENTATION: A 20-year-old woman without significant past medical history presented with severe mood changes prompting admission to an in-patient psychiatric facility. She developed seizures progressing to status epilepticus. She was intubated for airway protection and transferred to our ICU. Pentobarbital drip was started, as status epilepticus was refractory to a combination of four anticonvulsants. 24 hour EEG monitoring showed Stimulus Induced Rhythmic Periodic Ictal Discharges (SIRPID). Extensive workup including CSF studies showed a high titer for NMDAR antibodies. A CT scan of the abdomen was performed revealing an ovarian mass initially read as a benign cyst. However, a transvaginal ultrasound was performed revealing the lesion to be concerning for a teratoma. She underwent surgical resection of the tumor. She was treated aggressively with IV steroids, intravenous immunoglobulin, and plasma exchange in an effort to resolve her encephalitis. Despite the interventions, the seizures could not be controlled. Her hospital course was complicated with multi-drug resistant infections, which precluded further immunotherapy. Her family ultimately decided to withdraw life-sustaining treatment.
DISCUSSION: The N-Methyl-D-aspartic acid receptor (NMDAR) plays a critical role in synaptic plasticity, memory, and learning. A rare autoimmune disease has been described in the literature where antibodies against the NMDAR are formed. Clinically, these patients can present with seizures, psychiatric changes, and decreased consciousness. There have been only two previous reports of anti NMDA receptor encephalitis presenting with SIRPIDs, which is more commonly seen in patients with acute brain injury. Anti-NMDA receptor encephalitis is considered a paraneoplastic syndrome because the majority of affected patients have an underlying tumor (most common: teratoma). Treatment usually entails immunotherapy and tumor removal, though in some cases an underlying tumor is never identified. Prognosis can vary depending on cancer type and delay of appropriate diagnosis. Patients are often young and have a prolonged course of recovery, which can be complicated by hospital-acquired infections.
CONCLUSIONS: This case highlights the importance of keeping paraneoplastic syndrome as part of the differential diagnosis in cases with anticonvulsant refractory status epilepticus. Patients with this pathology often require long term critical care, mechanical ventilation, and hemodynamic support with average hospitalization around 2.5 months. 1.
Reference #1: 1. Gleichman A, et. al. Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies. Lancet Neurof 2008; 7: 1091-98
DISCLOSURE: The following authors have nothing to disclose: Aasim Afzal, Elecia Kim, Adan Mora
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