On June 5, 2012, a 76-year-old female patient was transported to the Intensive Care Unit of Mehr General Hospital (Tehran, Iran) via ambulance. She had a 3-week history of progressive somnolence, confusion, agitation, symptoms of depersonalization, and visual hallucinations. Her disorder began 12 weeks prior to her current admission, with daily and repeated short, sharp, lancinating, electric shock-like pain involving the left lower division of the fifth cranial nerve. She was taken to a local hospital in another city, where she underwent neurological evaluation by magnetic resonance imaging (MRI) and electroencephalography (EEG), with normal results. The patient was instructed to take 200 mg carbamazepine 3 times a day, which partially relieved her pain episodes. Eight weeks later, and following attacks of nocturnal hallucinations, a neurologist prescribed 5 mg olanzapine to be taken each night. However, her mental and psychiatric state deteriorated, and she was transferred to Mehr General Hospital.
On physical examination, the patient was afebrile. Scars from a coronary artery bypass surgery performed in 2008 were present on her anterior chest wall. Her lungs were clear, and no lymphadenopathy or organomegaly was detected. Neurological evaluation revealed an awake, agitated, confused woman with garbled and incoherent speech. She was easily distracted by irrelevant stimuli and could not sustain attention. No carotid bruit or stiff neck was detected. Cranial nerves were grossly normal, and no sensory or motor abnormalities were detected in the fifth cranial nerve. Corneal, orbicularis oculi, and sneeze reflexes were intact. Her motor power was 5/5 in all 4 extremities, her deep tendon reflexes were 2/4 in the arms and 1/4 in the legs, she had normal flexor plantar responses, and no abnormalities were seen in gait or coordination.
Brain MRI, MR angiography, and MR venography were normal. EEG showed moderately diffuse slowing (fig. 1). A lumbar puncture was performed, and the results of the cerebrospinal fluid analysis are shown in table 1. To avoid any possible treatable diagnosis, intravenous phenytoin and acyclovir were started, but acyclovir was stopped 5 days later when the final diagnosis was achieved. Quetiapine was administered to keep the patient calm.
Blood tests showed an elevated erythrocyte sedimentation rate (40 mm after 1 h; normal: <20 mm) with normal complete blood count, and low hemoglobin (10.6 mg/dl; normal: 12-16 mg/dl). Liver and kidney function tests and blood sugar levels were normal. Immunologic tests showed negative anti-Ro, anti-nuclear, and anti-neutrophil cytoplasmic antibodies. Serum protein electrophoresis and angiotensin-converting enzyme levels were normal. Broad serological screening assays for infectious conditions, including HIV, herpes simplex virus 1 and 2, hepatitis, Lyme disease, syphilis, and tuberculosis using the intermediate-strength purified protein derivative skin test, were negative. Tumor markers, including carbohydrate antigen (CA)-125, CA-19.9, CA-15.3, CA-242, carcinoembryonic antigen, serum alpha-fetoprotein, and anti-Ri and anti-Yo antibodies were not detected, but anti-Hu antibodies were present in the serum, as visualized by dot blot and indirect immunofluorescence techniques.
To localize the suspected malignancy, the patient underwent computed tomography scans of the chest and abdomen, which disclosed a large mass in the left ovary (fig. 2). She underwent surgery, and a 20-cm ovarian intestinal-type mucinous tumor (fig. 3) was removed. After surgery, the patient recovered quickly. After 2 weeks, she was alert and oriented with normal speech. Her second EEG at this stage was normal (fig. 4). Two months after the operation, the patient was pain-free, and her carbamazepine and phenytoin were gradually discontinued. At her last follow-up visit in March 2013, the patient was well and free of symptoms.