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.
Similar content being viewed by others
References
Scaravilli F, An SF, Groves M, et al (2000) The neuropathology of paraneoplastic syndromes. Brain Pathol 9:251–260
Lawn ND, Westmoreland BF, Kiely MJ, et al (2003) Clinical, magnetic resonance imaging, and electoencephalographic findings in paraneoplastic limbic encephalitis. Mayo Clin Proc 78:1363–1368
Nokura K, Yamamoto H, Okawara Y, et al (1997) Reversible limbic encephalitis caused by ovarian teratoma. Acta Neurol Scand 95:367–373
Lee ACW, Ou Y, Lee WK, et al (2003) Paraneoplastic limbic encephalitis masquerading as chronic behavioural disturbance in an adolescent girl. Acta Paediatr 92:506–509
Okamura H, Oomori N, Uchitomi Y (1997) An acutely confused 15-year-old girl. Lancet 350:488
Carr I (1982) The Ophelia syndrome: memory loss in Hodgkin’s disease. Lancet 1:844–845
Rosenbaum T, Gartner J, Korholz D, et al (1998) Paraneoplastic limbic encephalitis in two teenage girls. Neuropediatrics 29:159–162
Darnell RB, Posner JB (2003) Paraneoplastic syndromes involving the nervous system. N Engl J Med 349:1543–1554
Taylor RB, Mason W, King K, et al (1999) Reversible paraneoplastic encephalomyelitis associated with benign ovarian teratoma. Can J Neurol Sci 26:317–320
Aydiner A, Gurvit H, Baral I (1998) Paraneoplastic limbic encephalitis with immature ovarian teratoma. J Neurooncol 37:63–66
Gultekin SH, Rosenfeld MR, Voltz R, et al (2000) Paraneoplastic limbic encephalitis: neurological symptoms, immunological findings and tumour association in 50 patients. Brain 123:1481–1494
Case# 30-1985 (1985) Case records of the Massachusetts General Hospital. N Engl J Med 313:249–257
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Stein-Wexler, R., Wootton-Gorges, S.L., Greco, C.M. et al. Paraneoplastic limbic encephalitis in a teenage girl with an immature ovarian teratoma. Pediatr Radiol 35, 694–697 (2005). https://doi.org/10.1007/s00247-005-1402-1
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00247-005-1402-1