EFA6A-like antibodies in paraneoplastic encephalitis associated with immature ovarian teratoma: a case report.
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.
Koide
Reiji
R
Department of Neurology, Tokyo Metropolitan Neurological Hospital, 2-6-1 Musashidai, Fuchu, Tokyo 183-0042, Japan. reiji@tmnh.fuchu.tokyo.jp
Shimizu
Toshio
T
Koike
Kazunori
K
Dalmau
Josep
J
D000328:Adult; D001323:Autoantibodies / Q000276:immunology; D001921:Brain / Q000276:immunology / Q000473:pathology; D005260:Female; D020662:Guanine Nucleotide Exchange Factors; D006801:Humans; D020363:Limbic Encephalitis / Q000097:blood / Q000134:cerebrospinal fluid / Q000150:complications / Q000276:immunology*; D009419:Nerve Tissue Proteins / Q000097:blood / Q000134:cerebrospinal fluid / Q000276:immunology*; D010051:Ovarian Neoplasms / Q000097:blood / Q000134:cerebrospinal fluid / Q000150:complications / Q000276:immunology*; D013226:Status Epilepticus / Q000097:blood / Q000134:cerebrospinal fluid / Q000209:etiology* / Q000276:immunology; D013724:Teratoma / Q000097:blood / Q000134:cerebrospinal fluid / Q000150:complications / Q000276:immunology*; D016896:Treatment Outcome
D001323:Autoantibodies; D020662:Guanine Nucleotide Exchange Factors; D009419:Nerve Tissue Proteins; C109970:PSD protein, human