Cerebral Dural Sinus Thrombosis Associated with Adenomyosis: A Case Report Kenya Nishioka, MD, PhD,* Ryota Tanaka, MD, PhD,* Satoshi Tsutsumi, MD, PhD,† Kazuo Yamashiro, MD, PhD,* Mariko Nakahara, MD,‡ Hideki Shimura, MD, PhD,x Nobutaka Hattori, MD, PhD,* and Takao Urabe, MD, PhDx
We report a case of cerebral venous thrombosis (CVT) associated with a giant adenomyosis. At admission, the patient demonstrated generalized seizures and consciousness disturbance. Brain fluid–attenuated inversion recovery magnetic resonance imaging revealed a localized, high-intensity region in the left frontal lobe. Subsequent brain angiography showed that right internal carotid angiograms display abrupt termination of the anterior half of the superior sagittal sinus and a filling defect in the lateral part of the left transverse sinus. The patient complicated with iron deficiency anemia (IDA) and adenomyosis with higher levels of serum carbohydrate antigen 125 (CA125) and D-dimer. After 1 year from onset, intermittent severe menalgia and headache persisted, and blood examination revealed abnormal values; the patient was receiving oral medications. Finally, adenomyosis resection was performed with a favorable outcome, and no recurrence was observed during the 2-year follow-up period. We conclude that IDA and increased CA125 levels may have promoted hypercoagulability and CVT. This report emphasizes the possible relationship between CVT and adenomyosis. Key Words: Cerebral venous thrombosis—dural sinus—adenomyosis—iron deficiency anemia. Ó 2014 by National Stroke Association
Introduction Cerebral venous thrombosis (CVT) is an infrequent cerebrovascular disease, with an estimated incidence From the *Department of Neurology, Juntendo University School of Medicine, Tokyo; †Department of Neurosurgery, Juntendo University Urayasu Hospital, Chiba; ‡Department of Obstetrics and Gynecology, Juntendo University Urayasu Hospital, Chiba; and xDepartment of Neurology, Juntendo University Urayasu Hospital, Chiba, Japan. Received December 16, 2013; revision received January 25, 2014; accepted January 30, 2014. Conflicts of interest: The authors declare no financial or other conflicts of interest. Address correspondence to Kenya Nishioka, Department of Neurology, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo, Tokyo 113-8421, Japan. E-mail: [email protected] 1052-3057/$ - see front matter Ó 2014 by National Stroke Association http://dx.doi.org/10.1016/j.jstrokecerebrovasdis.2014.01.027
of .5% among all strokes.1,2 Causes of CVT are heterogeneous.1 We report a case of adenomyosisassociated dural sinus thrombosis complicated by iron deficiency anemia (IDA) and increased serum carbohydrate antigen 125 (CA125) levels. We propose adenomyosis as a possible cause of CVT and briefly discuss its development.
Case Presentation A 47-year-old woman experienced a sudden onset of generalized seizures. At presentation, the patient was somnolent and complained of headache. Her blood pressure was 106/70 mm Hg. She had no history of hypertension, diabetes, or dyslipidemia and had not been a habitual smoker. Her medical history was remarkable for adenomyosis. She was not pregnant or taking
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K. NISHIOKA ET AL.
Figure 1. (A-E) Axial fluid-attenuated inversion recovery images at presentation (A) and 3 days later (B), indicating the emergence of a high-intensity region in the anterior left frontal lobe with moderate mass effect. (C) Sagittal T1-weighted magnetic resonance image revealing a giant adenomyosis (12 3 10 cm) occupying the pelvic cavity. (D and E) Right internal carotid angiograms (venophase) display abrupt termination of the anterior half of the superior sagittal sinus (D, arrow) and a filling defect in the lateral part of the left transverse sinus (E, arrow).
contraceptives. Ophthalmologic examination revealed no abnormalities in the optic fundi. However, brain fluid–attenuated inversion recovery magnetic resonance imaging (MRI) revealed a localized, high-intensity region in the anterior portion of the left frontal lobe, which was unclear on images at presentation but became evident 3 days later (Fig 1, A,B). Cerebrospinal fluid examination by lumbar spinal tap revealed increased cell counts of 70 per microliter and protein levels of 98 mg/dl; however, she did not exhibit signs of meningeal irritation or fever. Blood examination revealed anemia (hemoglobin, 7.6 g/dl) and increased D-dimer (6.3 mg/ml, normal levels , 1.0 mg/ml) and CA125 (784.6 U/ml, normal levels , 40 U/ml) levels. Her lower abdomen was considerably distended, suggesting the presence of a large mass. Pelvic MRI revealed a giant adenomyosis (Fig 1, C). Cerebral angiography revealed oc-
clusions in the superior sagittal sinus and left transverse sinus (Fig 1, D,E). She was diagnosed with cerebral dural venous thrombosis. Heparin was infused intravenously for 7 days. Anticoagulant treatment with warfarin was initiated; however, it was switched to antiplatelet treatment because of hepatotoxicity. The high-intensity region in the anterior area of the left frontal lobe in MRI T2-weighted image and fluid-attenuated inversion recovery images became smaller 2 months after onset. The smaller lesion remained for 2 years. This region may have been involved in primary seizures and consciousness disturbances. Iron supplementation was provided for anemia management; however, low hemoglobin levels (minimum, 8.4 g/dl) and high CA125 (maximum, 2382 U/ml) and D-dimer (1.65 mg/ml) levels persisted. She also began experiencing intermittent severe menalgia and headache.
CVT AND ADENOMYOSIS
Gonadotropin-releasing hormone agonist therapy could not be administered because of the risk of adverse effects such as osteoporosis. Subsequently, the patient underwent adenomyosis resection 1 year after onset. Her clinical symptoms completely disappeared after surgery, and there was no recurrence of anemia. The follow-up magnetic resonance venography still showed disturbances in the blood flow in the superior sagittal sinus and left transverse sinus. The increasing blood flow supplied from the collateral vessels may have improved her symptoms. She is undergoing antiplatelet treatment, and sinus thrombosis has not recurred for 2 years. Her serum D-dimer and CA125 levels have also remained within the normal range.
Discussion To our knowledge, ours is the first case of CVT associated with adenomyosis. We previously reported 4 adenomyosis cases complicated by cerebral infarction.3 Adenomyosis rarely causes cerebral embolism with increase in D-dimer and CA125 levels. In addition to ovarian cancer, CA125 is a marker of benign ovarian tumors, myoma uteri, and adenomyosis.4 CA125 is a mucinous protein that can promote blood hypercoagulability.5 Recently, several reports have suggested a strong association between CVT and IDA.6,7 However, the precise mechanism by which IDA leads to CVT remains unknown. It is thought that abnormal adherence of erythrocytes to the endothelium and subsequent endothelial activation may induce hypercoagulability.6,8 Although the relationship between CVT and adenomyosis is speculative, we assume that IDA and
increased serum CA125 levels could have influenced the development and extension of CVT. In our patient, adenomyosis resection achieved a favorable outcome. This case suggests that adenomyosis can be a risk factor for CVT, particularly when concurrent with IDA and increased serum CA125 levels.
References 1. Saposnik G, Barinagarrementeria F, Brown RD Jr, et al. Diagnosis and management of cerebral venous thrombosis: a statement for healthcare professionals from the American Heart Association/American Stroke Association. Stroke 2011;42:1158-1192. 2. Bousser MG, Ferro JM. Cerebral venous thrombosis: an update. Lancet Neurol 2007;6:162-170. 3. Yamashiro K, Tanaka R, Nishioka K, et al. Cerebral infarcts associated with adenomyosis among middle-aged women. J Stroke Cerebrovasc Dis 2012;21:e1-e5. 4. Ghaemmaghami F, Karimi Zarchi M, Hamedi B. High levels of CA125 (over 1,000 IU/ml) in patients with gynecologic disease and no malignant conditions: three cases and literature review. Arch Gynecol Obstet 2007; 276:559-561. 5. Yamashiro K, Furuya T, Noda K, et al. Cerebral infarction developing in a patient without cancer with a markedly elevated level of mucinous tumor marker. J Stroke Cerebrovasc Dis 2012;21:e1-e2. 6. Stolz E, Valdueza JM, Grebe M, et al. Anemia as a risk factor for cerebral venous thrombosis? An old hypothesis revisited. Results of a prospective study. J Neurol 2007; 254:729-734. 7. Ogata T, Kamouchi M, Kitazono T, et al. Cerebral venous thrombosis associated with iron deficiency anemia. J Stroke Cerebrovasc Dis 2008;17:426-428. 8. Shiu YT, McIntire LV. In vitro studies of erythrocytevascular endothelium interactions. Ann Biomed Eng 2003;31:1299-1313.
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