Encefalitis límbica y síndrome neuroléptico maligno secundario a teratoma ovárico maduro con componente inmaduro. Comunicación de un caso fatal y revisión de la bibliografía

This paper reports the case of a 28-year-old woman who seven days before admission showed behavioral changes, seizures, visual hallucinations, sleep wake cycle reversals and weight loss of 10 kg in 10 days. Tomography scans showed: right ovarian tumor. Tumor markers were performed in blood: anti-neuron Auto: Antibodies (NMDA-R) positive. Her hospital stay was characterized by: seizures, psychomotor agitation and persistent fever of 40 to 42oC in the absence of leukocytosis. Surgery service suggested surgical excision of ovarian tumor, but it was not possible for the patient’s condition. With impaired function: anuria, severe rhabdomyolysis, without stem reflexes (akinetic mutism and miotic pupils). Patient died 14 days after admission with the clinical diagnosis of limbic encephalitis and neuroleptic malignant syndrome secondary to ovarian teratoma. The autopsy showed 13 X 12 X 5.5 cm right ovarian lesion, heterogeneous histology confirmed mature teratoma with immature elements. Macroscopically patient had brain edema and microscopic examination of the hippocampus and amygdala showed: neuronal loss, gliosis, activation of microglial cells (rod cells) and perivascular lymphocytic cuffs. Limbic encephalitis (EL) can affect any part of the central or peripheral nervous system, or neuromuscular junction. This is a paraneoplastic syndrome associated with many tumors. Its pathogenesis is autoimmune, antibodies against antigens are formed: intracellular neuronal membrane, including potassium channels and the newly identified “hippocampal neuropil antigens”. Encephalitis associated with antibodies against these receptors predominantly affects young women who have benign-appearing ovarian cysts (mature or immature teratomas). Despite the severity of disease, recovery is very common after tumor removal and immunotherapy. On the other hand, neuroleptic malignant syndrome is an idiosyncratic reaction to neuroleptic antipsychotic drugs, is characterized by temperatures above 42oC, altered mental status, autonomic dysfunction and muscle rigidity.