EFA6A-like antibodies in paraneoplastic encephalitis associated with immature ovarian teratoma: a case report
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We describe a 19-year-old patient with paraneoplastic encephalitis associated with immature ovarian teratoma (OT), who presented with psychiatric symptoms, prolonged disturbance of consciousness, refractory status epilepticus, central hypoventilation, and various abnormal involuntary movements. Immunological characterization of the patient’s serum and cerebrospinal fluid (CSF) demonstrated the presence of an autoantibody that colocalized with EFA6A, a brain-specific protein involved in the regulation of dendritic development of hippocampal neurons. Despite the severity of the symptoms, the patient showed significant neurological improvement following removal of the tumor and chemotherapy. This case suggests that physicians should rule out an OT in young women with encephalitis who present with the subacute-onset of psychiatric symptoms. Antibodies that colocalize with EFA6A are a valuable marker for early diagnosis of a potentially reversible paraneoplastic encephalitis associated with OT.
KeywordsEFA6A Ovarian teratoma Paraneoplastic encephalitis Status epilepticus Involuntary movement disorder
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We thank Dr. Keiko Tanaka, Department of Neurology, Brain Research Institute, Niigata University, for evaluation of the anti-neuronal antibodies, and Dr. Kimiyoshi Arimura and Dr. Osamu Watanabe, Department of Neurology, Kagoshima University, for evaluation of VGKC antibodies.
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