Anti–N-methyl-D-aspartate receptor encephalitis is an emerging disease that affects young women. Its diagnosis can be delayed because of the neuropsychiatric symptoms in the foreground, but early removal of the associated teratoma improves the prognosis. We report the treatment of a patient with anti–N-methyl-D-aspartate receptor encephalitis that was related to an ovarian teratoma.
Anti–N-methyl-D-aspartate (NMDA) receptor encephalitis consists of the association of acute psychiatric disorders and seizures that are preceded by a flu-like syndrome. Over 400 cases have been reported since the seminal description in late 2007 that led to the idea that the prevalence of this disease may be underestimated. The association with teratoma was reported only 4 years ago, and the early removal of the tumor is associated with a favorable outcome in most cases. Briefly, anti-NMDA receptor antibodies react against glutamate receptor that is located at the surface of neurons and result in an impaired glutamatergic neurotransmission. We report the case of a woman who had “paraneoplastic” limbic encephalitis that was related to a mature ovarian teratoma.
Case Report
A 27-year-old Mauritanian woman came to our institution because of hallucinations and confusion that had been preceded by a flu-like illness 10 days earlier. Neurologic examination revealed anterograde memory deficit and trouble walking. She was taken to the reanimation department and required mechanical ventilation because of impaired vigilance with rapid worsening of the Glasgow coma score, which decreased from 10 to 6.
Blood cell count, blood electrolytes, and the brain computed tomography (CT) scan were normal. Increased fluid attenuation inversion recovery signals of both temporal lobes were seen on cerebral magnetic resonance imaging ( Figure 1 ). Cerebrospinal fluid showed mild lymphocytosis but was sterile. Electroencephalography showed an intermittent complex partial seizure that responded well to intravenous sodium valproate, and febrile limbic encephalopathy was diagnosed. Empiric therapy that consisted of acyclovir and cefotaxime was administered. Once infection (including herpetic meningitis) had been discounted, paraneoplastic causes were explored. Screening for classic onconeuronal antibodies (anti-Hu, Yo, Ma2, amphiphysin, CV2/CRMP5, and Ri) was negative, but anti-NMDA receptor antibodies were found in the cerebrospinal fluid. A total body CT scan that was performed to look for a possible primary tumor revealed a 4-cm unilocular cyst on the right ovary. Ultrasound examination confirmed an anechogenic ovarian cyst with septa.
We performed laparoscopic cystectomy without cyst rupture ( Figure 2 ). Histologic examination indicated a mature teratoma with a cutaneous component. Immunofluorescence confirmed the presence of antibodies against the NR1 and NR2B heteromers of NMDA receptor ( Figure 3 ).
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