Paraneoplastic encephalitis, psychiatric symptoms, and hypoventilation in ovarian teratoma

Abstract

We report four young women who developed acute psychiatric symptoms, seizures, memory deficits, decreased level of consciousness, and central hypoventilation associated with ovarian teratoma (OT) and cerebrospinal fluid (CSF) inflammatory abnormalities. Three patients recovered with treatment of the tumor or immunosuppression and one died of the disorder. Five other OT patients with a similar syndrome and response to treatment have been reported. Our patients' serum or CSF showed immunolabeling of antigens that were expressed at the cytoplasmic membrane of hippocampal neurons and processes and readily accessed by antibodies in live neurons. Immunoprobing of a hippocampal-expression library resulted in the isolation of EFA6A, a protein that interacts with a member of the two-pore-domain potassium channel family and is involved in the regulation of the dendritic development of hippocampal neurons. EFA6A-purified antibodies reproduced the hippocampal immunolabeling of all patients' antibodies and colocalized with them at the plasma membrane. These findings indicate that in a young woman with acute psychiatric symptoms, seizures, and central hypoventilation, a paraneoplastic immune-mediated syndrome should be considered. Recognition of this disorder is important because despite the severity of the symptoms, patients usually recover. The location and function of the isolated antigen suggest that the disorder is directly mediated by antibodies.

Anti-NMDA receptor encephalitis presenting with total insomnia — A case report

“…Recently, a report analyzing brain PET scan of six patients with anti-NMDAR encephalitis detected a gradient of cerebral metabolism from frontotemporal to occipital areas [22]. The diagnosis is based on the identification of anti-NMDAR antibodies in serum or CSF [4,6,23].…”

Gait Disturbance as the Presenting Symptom in Young Children With Anti-NMDA Receptor Encephalitis

“…2 This neurologic disorder was first described in 2005 in young women who presented with psychiatric symptoms, seizures, encephalopathy, movement disorders, and autonomic instability and who were found to have ovarian teratomas. 3 Common antibodies that react against the NR1 subunit of the N-methyl-D-aspartate receptor were demonstrated to play a pathogenic role in this disease. 4 Since that time, descriptions of symptom onset, treatment, and outcome in larger cohorts have been published, and the condition has become notable for its dramatic clinical presentation with typically favorable response to therapy.…”

Anti-NMDA-reseptorencefalitt

“…Initialt ble anti-NMDA-reseptorencefalitt diagnostisert hos kvinner med ovariale teratomer (19). Dette mer enn antydet at det var en assosiasjon mellom teratomene og encefalitten.…”