title: Paraneoplastic limbic encephalitis in a teenage girl with an immature ovarian teratoma.
abstract: Paraneoplastic limbic encephalitis (PLE) is an unusual disorder that is characterized by the association of clinical limbic system abnormalities with neoplasia, usually malignancy. It has rarely been reported in children and then manifests during the teenage years. Diagnosis is often delayed, especially when the tumor has not been recognized. In adults, the diagnosis can be revealed by the presence of antineuronal antibodies. We describe an unusual case of behavioral disturbance leading rapidly to coma in a 14-year-old girl with CSF pleocytosis who was found 10 weeks later to have an immature ovarian teratoma. Although her symptoms eventually improved slightly after tumor excision, she died while in rehabilitation. PLE is an important diagnosis to consider in the teenage girl with symptoms of a progressive limbic disorder and CSF pleocytosis, and whose brain MR imaging demonstrates no abnormality or mild T2-weighted temporal lobe signal abnormality. When this constellation of findings presents in a teenage girl, the possibility of an underlying ovarian teratoma should be considered.
authors.affiliation: Department of Radiology, University of California, Davis Medical Center and U.C. Davis Children's Hospital, Sacramento, CA 95817, USA. rebecca.steinwexler@ucdmc.ucdavis.edu
authors.forename: Rebecca
authors.initials: R
authors.lastname: Stein-Wexler
authors.forename: Sandra L
authors.initials: SL
authors.lastname: Wootton-Gorges
authors.forename: Claudia M
authors.initials: CM
authors.lastname: Greco
authors.forename: James A
authors.initials: JA
authors.lastname: Brunberg
mesh_terms: D000293:Adolescent; D001921:Brain / Q000473:pathology; D003128:Coma / Q000209:etiology; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007964:Leukocytosis / Q000134:cerebrospinal fluid; D020363:Limbic Encephalitis / Q000134:cerebrospinal fluid / Q000209:etiology*; D008279:Magnetic Resonance Imaging; D001523:Mental Disorders / Q000209:etiology; D010051:Ovarian Neoplasms / Q000150:complications* / Q000175:diagnosis; D013724:Teratoma / Q000150:complications* / Q000175:diagnosis
references.citation: Acta Paediatr. 2003 Apr;92(4):506-9
references.pmid: 12801123
references.citation: N Engl J Med. 1985 Jul 25;313(4):249-57
references.pmid: 4010727
references.citation: N Engl J Med. 2003 Oct 16;349(16):1543-54
references.pmid: 14561798
references.citation: Acta Neurol Scand. 1997 Jun;95(6):367-73
references.pmid: 9228272
references.citation: Mayo Clin Proc. 2003 Nov;78(11):1363-8
references.pmid: 14601695
references.citation: Can J Neurol Sci. 1999 Nov;26(4):317-20
references.pmid: 10563220
references.citation: J Neurooncol. 1998 Mar;37(1):63-6
references.pmid: 9525839
references.citation: Neuropediatrics. 1998 Jun;29(3):159-62
references.pmid: 9706628
references.citation: Lancet. 1997 Aug 16;350(9076):488
references.pmid: 9274586
references.citation: Brain. 2000 Jul;123 ( Pt 7):1481-94
references.pmid: 10869059
references.citation: Brain Pathol. 1999 Apr;9(2):251-60
references.pmid: 10219743
references.citation: Lancet. 1982 Apr 10;1(8276):844-5
references.pmid: 6122069