Paraneoplastic Anti-N-Methyl-D-Apartate-Receptor Encephalitis Associated With an Immature Teratoma
摘要:
Clinical Practice Points • Paraneoplastic syndromes are defined as a constellation of symptoms and signs that are not directly caused by the primary or metastatic cancer. • Anti- N -Methyl-D-Apartate (NMDA) receptor-mediated encephalitis is a paraneoplastic syndrome that has been associated with ovarian teratoma. It is believed that ectopic neural tissues in teratomas can stimulate the immune system to produce antibodies to the NMDA receptor with resulting encephalitis. • Anti-NMDA encephalitis is emerging as a more frequently recognized but likely underdiagnosed cause of neuropsychiatric symptoms and fever of unknown origin. This disease has high morbidity and mortality secondary to its often-late diagnosis, but with early detection and prompt treatment, many of the manifestations are reversible. Introduction Paraneoplastic syndromes are defined as a constellation of symptoms and signs that are not directly caused by the primary or metastatic cancer. Anti- N -Methyl-D-Apartate (NMDA) receptor-mediated encephalitis is a paraneoplastic syndrome that has been associated with ovarian teratoma. The NMDA receptor is located in the hippocampus, and persistent activation has been studied as an origin of epilepsy, dementia, and stroke. It is believed that ectopic neural tissues in teratomas can stimulate the immune system to produce antibodies to the NMDA receptor with resulting encephalitis.3-5 Here we present a case of an immature ovarian teratoma associated with paraneoplastic encephalitis. Case Report A 22-year-old woman (G3P1021) with a past medical history of bipolar disorder began experiencing hallucinations and demonstrating combative behavior at home. After jumping from the family vehicle en route to the local emergency room, the patient was taken to a nearby inpatient psychiatric facility. On arrival, the patient was complaining of headache, actively hallucinating, and treated with the neuroleptic medications, haldol and thorazine. She proceeded to exhibit clonic rhythmic movements, muscle rigidity, and became unresponsive. She was subsequently transferred to our medical center's intensive care unit. On arrival, she was obtunded, febrile at 103.0°F, and demonstrating the same movements as already described. She required intubation for airway protection. Initial laboratory results revealed a markedly elevated creatine phosphokinase. A febrile work-up was performed, including blood cultures, urine culture, and chest x-ray, and all were negative. Neurologic workup including computed tomography (CT) and magnetic resonance imaging (MRI) of the brain and lumbar puncture were unrevealing. Electroencephalography showed theta range slowing consistent with toxic or metabolic encephalopathy. Intensive care measures including broad spectrum antibiotics, mechanical ventilation, and nutritional support were instituted. The patient was given dantrolene and bromocriptine for the presumed diagnosis of neuroleptic malignant syndrome. She continued to require ventilator support and developed complications including ventilator-associated pneumonia and dantrolene-induced hepatitis. Her
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DOI:
10.1016/j.cogc.2013.02.002
被引量:
年份:
2012
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