PARANEOPLASTIC ENCEPHALITIS ASSOCIATED WITH OVARJAN TERATOMA: CLINICAL PICTURES AND N-METHYL-D-ASPARTATE RECEPTOR ANTIBODIES

Abstract

Background: Recently, a new category of paraneoplastic encephalitis associated with ovarian teratoma has been described and associates with antibodies to NRI/NR2 heteromers of the N-methyl-D-aspartate receptor (NMDAR). Methods: We describe clinical pictures of 26 patients with ovarian teratoma-associated encephalitis (OTE) and the presence of NMDAR antibodies. Results: Clinically, OTE was characterized by the development of acute prominent psy- chiatric symptoms (25 of 26 patients), seizures (19 of 26 patients), central hypoventilation (18 of 26 patients) and involuntary movements (19 of 26 patients). Ventilatory support was required for 84.5 ± 71.8 days on average. The white blood cell count in cerebrospinal fluid was 50.0 ± 56.6/mm3. Twelve patients showed abnormalities on cranial MRI, involving areas such as the temporal regions (seven patients) or brainstem (four patients). In addition to tumor resection, twenty patients received some type of immunotherapy. Twenty-three patients with OTE had neurological improvement, including 14 with full recovery. Immunocytochemical studies showed that cells expressing NR1/NR2 heteromers reacted strongly with the CSF of patients with OTE. Conclusions: Neurologists must be aware of the syndrome described above and evaluate antibodies to NR1/NR2B heteromers of the NMDAR. Detection of these antibodies should prompt the search for an ovarian teratoma and the administration of immunotherapy.