paraneoplastic syndromes are a heterogeneous collection of disease manifestations caused by underlying neoplasms , which can include adnexal masses . we report a case of paraneoplastic limbic encephalitis consisting of confusion , agitation , and aggression , which subsequently progressed to a comatose state , in the presence of an adnexal mass . female patients who present with symptoms of limbic encephalitis should be thoroughly screened for malignancy . gynecologic consultation is an important component of the diagnostic workup when an adnexal mass has been identified . paraneoplastic limbic encephalitis is a serious condition , yet it has a good prognosis if treated promptly with surgical resection of the adnexal mass . . this syndrome may involve the central nervous system , peripheral nervous system , or neuromuscular junction . the classic symptoms of le include short - term memory impairment , seizures , confusion , irritability , depression , sleep disturbances , and psychiatric disturbances , with onset ranging from days to 12 weeks . paraneoplastic limbic encephalitis ( ple ) is associated with a number of tumors , with ovarian teratomas accounting for 3% to 4% of cases . the presence of immature features can be as high as 50% to 60% in ovarian teratomas associated with ple . in contrast , in the general population , the majority of ovarian teratomas are mature , with only 3% possessing immature features . gynecologic consultation and prompt surgical management are essential to prevent permanent neurologic sequela , as illustrated in this case report . a 29-year - old caucasian multiparous female with a past medical history of epilepsy and vitamin b12 deficiency , and no prior history of mental illness , presented with complaints of confusion , agitation , and aggression . shortly after the development of these symptoms , she was placed on a voluntary 72-hour hold at an outside hospital for psychotic behavior . polysubstance abuse was initially suspected as the cause of her behavior ; however , the patient denied any history of substance abuse and a urine drug screen was negative . she was discharged to home , but was readmitted several days later to the medicine service at the same hospital with similar complaints of confusion , agitation , and aggression . at that time , she also complained of mild abdominal pain , and an abdominal ct scan was obtained revealing a heterogeneous enhancing mass ( 15 cm  15 cm  12 cm ) arising from the right ovary , which contained fat , calcium , fluid , and septations . no further workup of this ovarian mass was performed at the outside hospital , as the mass was suspected to be a mature ovarian teratoma . the patient was treated with acyclovir , vancomycin , and ceftriaxone . over the next 3 days , the patient 's mental status continued to decline ; she would not follow simple commands and had no withdrawal to painful stimuli . at this time , 8 days after her initial presentation , she was transferred to our facility for further evaluation and treatment of presumed viral encephalitis . a ct scan of the abdomen and pelvis was repeated , confirming the presence of a mass ( 17 cm  13 cm  11 cm ) arising from the right ovary . over the next few days , the patient remained comatose and showed no improvement in her neurologic examination ; she was noted to have decorticate posture , with extension of her neck and clonus of all 4 extremities . given the patient 's clinical syndrome of acute psychosis with no prior history of psychiatric illness , inflammatory cns findings , and coma , in the presence of a known ovarian mass , ple was considered . the uterus was of normal size with a freely mobile 17-cm to 20-cm midline pelvic mass . tumor markers , including ca125 , cea , afp , and beta - hcg were obtained and were all within normal limits . the patient was taken to the operating room and underwent a unilateral salpingo - oophorectomy via mini - laparotomy as described by rhodes et al . she remained in the intensive care unit briefly , where she was continued on intravenous fluids and tube feedings . antibiotics were discontinued after her csf and blood cultures were found to be negative for infectious etiologies . she was given 5 days of intravenous immunoglobulin to aid in clearing the presumed autoantibodies mediating her encephalopathy . over the next several days , she progressively improved and was discharged home on postoperative day 11 . the final pathologic diagnosis revealed the mass to be an ovarian teratoma with focal immature elements , without capsular penetration . immature teratomas may contain tissue from all 3 embryonic germ cell layers with at least one of them lacking full differentiation . neuroepithelial tissue usually is the origin of this immature tissue . in the rare case that a teratoma is associated with ple , the majority of women are not aware of having gynecological pathology , similar to our patient . thus , awareness of paraneoplastic syndromes is important for various practitioners , including both neurologists and gynecologists . limbic encephalitis is a paraneoplastic syndrome affecting the central nervous system , which has a subacute onset of days up to 12 weeks . the classic symptoms of le include short - term memory impairment , seizures , confusion , irritability , depression , sleep disturbances , and psychiatric disturbances . if left undiagnosed , patients with le may develop progressive unresponsiveness ( catatonia - like stage ) , central hypoventilation , autonomic instability ( fluctuations in blood pressure , temperature , and cardiac rhythm ) , orofacial dyskinesias , and limb choreoathetosis ( involuntary movements of the face and extremities ) and dystonia . further , intensive care support and ventilation may be required for several weeks or months . the following tumors are most commonly associated with ple : bronchial carcinoma ( 50% to 59% ) , with the majority being small cell lung cancer ; testicular tumors ( 6% to 20% ) ; breast cancer ( 3% to 8% ) ; hodgkin 's lymphoma ; ovarian teratoma ( 3% to 4% ) ; and thymoma ( 2% to 5% ) . the presentation of le precedes the diagnosis of malignancy in approximately 60% of cases , with the median interval between diagnosis of le and underlying cancer being 3.5 months . thus , when ple is suspected , a careful history and physical examination along with a thorough evaluation for the above tumors is essential . a patient with symptoms of le , with a malignancy that develops within 5 years of the diagnosis of the neurological disorder , meets diagnostic criteria for the diagnosis of ple . often onconeural antibodies against neural antigens that are expressed by the tumor can be identified . recent literature has found that patients without antibodies have a better response to treatment compared with patients with antibodies . therefore , laboratory assessment for these onconeural antibodies not only serves as a diagnostic modality but also aids in predicting prognosis . gynecologists play a very important role in the management of ple , as prompt tumor resection is the cornerstone of treatment for cases of ple with an associated ovarian teratoma , and appears to have the best effect on neurological outcome . in our case , ultimately , any woman who presents with symptoms of le should be screened for cancer . further , a gynecologic consult is an integral part of the workup of suspected ple when an adnexal mass is identified . female patients who present with symptoms of le should be thoroughly screened for malignancy . further , gynecologic consultation is an important component of the diagnostic workup when an adnexal mass has been identified . paraneoplastic limbic encephalitis is a serious condition , yet it has a good prognosis if treated promptly with surgical resection of the adnexal mass .
background : paraneoplastic syndromes are a heterogeneous collection of disease manifestations caused by underlying neoplasms , which can include adnexal masses.case:we report a case of paraneoplastic limbic encephalitis consisting of confusion , agitation , and aggression , which subsequently progressed to a comatose state , in the presence of an adnexal mass . 
 it was cured with resection of a mature teratoma with focal immature components.conclusion:female patients who present with symptoms of limbic encephalitis should be thoroughly screened for malignancy . 
 gynecologic consultation is an important component of the diagnostic workup when an adnexal mass has been identified . 
 paraneoplastic limbic encephalitis is a serious condition , yet it has a good prognosis if treated promptly with surgical resection of the adnexal mass .