Rinsho Shinkeigaku (Clinical Neurology)

Case Report

A young patient with endometrioid adenocarcinoma who suffered Trousseau's syndrome associated with vasculitis

Yuko Shimizu, M. D. , Shinichiro Uchiyama, M. D. , Goshi Mori, M. D. , Yukiko Tsutsumi, M. D. , Megumi Takeuchi, M. D. and Makoto Iwata, M. D.

Department of Neurology, Neurological Institute, Tokyo Women's Medical University School of Medicine

A 27-year-old woman was admitted to our hospital because of headache, fever and right neck pain. Neurological examination revealed mild meningeal signs, and hyper-reflexia in all extremities. In the laboratory tests, white-cell count was 13,000/mm3, rheumatoid factor (RF) and C-reactive protein (CRP) were positive. The cerebro-spinal fluid showed pleocytosis (56/mm3, neutorophils and lymphocytes were 26 and 28, respectively). Thus, she was diagnosed as aseptic meningitis. A few days later, she had weakness and dysesthesia of the right face and the left extremities. Pulse therapy with intravenous methylprednisolone was started. A magnetic resonance imaging (MRI) of the brain showed a hemorrhagic infarction in the right parietal lobe. In hemostatic markers, thrombin-antithrombin III complex (TAT; 106 ng/dl), D-dimer 1234 ng/dl,prothrombin fragment 1+2 (F1+2; 2.36 nmol/L), β -thromboglobulin (β TG; 4,300 ng/dl) and platelet factor 4 (PF-4; 1,770 ng/dl) were extremely elevated. On duplex ultrasonography, a low echo lucent plaque was observed at the right internal carotid artery and the mean blood flow velocity in the right carotid artery was decreased. She was placed on oral prednisolone and warfarin for suspected stroke due to hypercoagulability associated with vasculitis. Afterwards, she discharged from our hospital.

Two months later, she was readmitted to our hospital because of irregular menses and vaginal bleeding. Endometrial uterus biopsy was conducted, which revealed a grade I endometrioid adenocarcinoma. She was under total uterectomy without tumor recurrence. After the radical operation, white-cell count, RF, CRP, TAT, D-dimer, F1+2, and β TG were normalized, and the mean flow velocity of the right common carotid artery was increased. Thereafter, she did not experience stroke recurrence. Therefore, we speculated that she had stroke due to hypercoagulability in association with malignancy, that is Trousseau's syndrome. We also assumed that aseptic meningitis, brainstem encephalitis associated with vasculitis in this patient are other clinical variants of paraneoplastic syndrome through immunologial mechanisms associated with malignancy.

We emphasize that patients with Trousseau's syndrome can be associated with other paraneoplstic manifestations such as vasculitis as seen in this patient.

(CLINICA NEUROL, 42: 227|232, 2002)
key words: cerebral infarction, Trousseau's syndrome, uterus carcinoma, vasculitis

(Received: 22-Nov-01)