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.

Immune-Mediated Encephalitis and Virilization in Association with a Mature Cystic Ovarian Teratoma in an Adolescent Girl

“…Most cases of PLE have been described in the adult population [9], though a few cases have been described in children, all of whom were adolescent girls [11,12,13,14,15,16]. In these reports, PLE was seen in association with immature teratomas.…”

Limbic Encephalitis Associated with Primary Biliary Cirrhosis

“…Approximately 50% of paraneoplastic LE are associated with lung carcinoma, both oat cell and other histological types, 20% are associated with testicular carcinoma and 8% with breast cancer [4] . Cases associated with immature ovarian teratoma [8] , Hodgkin's lymphoma [9,10] and malignant thymoma [11,12] have also been reported. Most of these patients, up to 60%, showed positivity of onconeuronal antibodies [4] .…”

Delirium in children and adolescents: A systematic review of the literature

“…Delirium may also occur in the context of neoplastic illness in children and adolescents [33], and crucially, in occasional patients, delirium associated with paraneoplastic limbic encephalitis may be the first clinical manifestation of an underlying cancer [34,35]. Delirium in childhood is also well described in nonconvulsive status epilepsy [36], childhood confusional migraine [37][38][39][40], cerebral systemic lupus erythematosus [41], and HIV/AIDS [12,13].…”