Paraneoplastic encephalitis, psychiatric symptoms, and hypoventilation in ovarian teratoma

Annals of Neurology - Tập 58 Số 4 - Trang 594-604 - 2005
Bruno Giometto1, Warren Mason2, Beau M. Ances3, Theodore Zwerdling4, Zhilong Jiang3, Josep Dalmau3
1Division of Neuro-oncology, Department of Neurology, University of Pennsylvania, Philadelphia, PA 19104, USA.
2Princess Margaret Hospital, Toronto, Ontario, Canada
3Division of Neuro-Oncology, Department of Neurology, University of Pennsylvania, Philadelphia, PA
4Department of Pediatrics, Hematology/Oncology Section, University of California, Davis Medical Center, Sacramento, CA

Tóm tắt

AbstractWe 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. Ann Neurol 2005;58:594–604

Từ khóa


Tài liệu tham khảo

10.1093/brain/120.6.923

10.1093/brain/123.7.1481

10.1093/brain/awh203

10.1111/j.1600-0404.1997.tb00227.x

10.1016/S0140-6736(97)06208-9

10.1023/A:1005875822605

10.1186/1477-7800-1-11

10.1111/j.1651-2227.2003.tb00588.x

10.1017/S0317167100000469

10.1097/00041327-200406000-00004

10.1007/s00247-005-1402-1

10.1002/ana.10713

Dalmau J, 1992, The expression of the Hu (paraneoplastic encephalomyelitis/sensory neuronopathy) antigen in human normal and tumor tissues, Am J Pathol, 141, 881

10.1016/0361-9230(91)90003-3

10.1002/ana.10462

10.1093/brain/awh077

10.1093/emboj/18.6.1480

10.1242/jcs.115.14.2867

10.1111/j.0953-816X.2004.03195.x

10.1007/BF00313390

10.1177/088307389701200412

10.1136/jnnp.37.10.1166

10.1242/jcs.01090

10.1038/sj.embor.7400292

10.1523/JNEUROSCI.21-19-07491.2001

10.1093/emboj/16.17.5464

10.1212/01.WNL.0000122648.19196.02

10.1111/j.1471-4159.2005.03072.x

10.1016/S1569-9048(03)00185-X

10.1016/S0034-5687(01)00288-2

10.1016/S0140-6736(05)66376-3

Thông tin
Thông tin xuất bản

Annals of Neurology

Tập 58 Số 4

594-604

Thông tin tác giả