Early Diagnosis of a Cerebral Ve n o u s Thrombosis in the Emergency Department Ji Yun Ahn, M.D., Kyoung Soo Lim, M.D., Jae Ho Lee, M.D., Hui Dong Kang, M.D., Bum Jin Oh, M.D., Won Kim, M.D. P u r p o s e: Early diagnosis and heparin therapy have contributed to a decreased mortality in patients with a cerebral venous thrombosis (CVT). The aim of this study is to evaluate the clinical and the radiologic features of a CVT and to develop an algorithm for diagnosis and treatment. M e t h o d s: Twenty (20) patients who were diagnosed at Asan Medical Center from 1997 to June 2004 as having a CVT were included in this study. The data in their medical records, including clinical features, predisposing factors, and diagnostic modalities, were reviewed. The cranial computed tomography findings of a CVT were investigated in 18 patients. R e s u l t s: Headache was the most common symptom, followed by seizure and focal neurologic deficits. Initially, 14 patients were misdiagnosed by the emergency physician as having another neurologic disease, though 5 patients had pathognomic CT signs of a CVT. CT signs, such as cord signs and empty delta signs, were present in 10 cases. Among them, the cord sign was positive in eight (8) cases (44.4%), the empty delta sign was positive in six (6) cases (46.2%), and both signs were positive in four (4) cases. The presence of CT signs correlated with the presence of seizure at onset, and the Modified Rankin Scale (MRS) was 605 1 or 2 at discharge (p<0.05). Parenchymal changes correlated with headache at discharge (p<0.05). C o n c l u s i o n: Emergency physicians should consider CVT and look for pathognomic signs of a CVT when patients complain of sub-acute headaches or when the clinical features do not correlate with the neuroimage findings. Key Words: Cerebral venous thrombosis, Neuroimaging, Cord sign, Empty delta sign Department of Emergency Medicine, University of Ulsan, College of Medicine, Asan Medical Center, Seoul, Korea.
606 / Table 1. Modified Rankin scale (MRS) Score Description 0 No symptoms at all 1 No significant disability despite symptoms; able to carry out all usual duties and activities 2 Slight disability; unable to carry out all previous activities, but able to look after own affairs without assistance 3 Moderate disability; requiring some help, but able to walk without assistance 4 Moderately severe disability; unable to walk without assistance and unable to attend to own bodily needs without assistance 5 Severe disability; bedridden, incontinent and requiring constant nursing care and attention 6 Dead
607 Table 2. Baseline characteristics of the patients number (n) percent (%) Demographic data Age (years) 38.319.6 Length of stay (days) 23.215.2 Door to drug* (hours) 82.378.6 Female 13 65.0 Main symptoms on ER admission Headache 14 70.0 Seizures 10 50.0 Focal neurologic deficit 10 50.0 Visual symptoms 07 35.0 Impaired consciousness(gcs 13) 03 15.0 Cognitive dysfunction 03 15.0 * drug: anticoagulants or thrombolytics, GCS: Glasgow Coma Scale Table 3. The clinical features and predisposing factors Case Age Sex Chief complaints Predisposing factor & underlying disease 1 47 F General weakness Dehydration, Cushing syndrome 2 21 M Seizure Brain tumor(pineal gland germinoma) 3 42 F Seizure Idiopathic 4 85 F Seizure Chronic otitis media, diabetes mellitus 5 5 M Aphasia Valvular heart disease 6 33 M Fever Behcet s disease, pneumonia 7 43 F Seizure Idiopathic 8 36 F Seizure Primary antiphospholipid antibody syndrome, pregnancy 9 51 F Dizziness Anemia, HRTx* (due to adenomyosis) 10 29 M Diplopia Sepsis 11 27 F Headache Oral contraceptive ingestion 12 62 F Memory dysfunction Idiopathic 13 29 F Seizure Protein C and S deficiency, pregnancy 14 31 F Headache Hormone replacement therapy 15 43 M Headache Dehydration 16 27 F Seizure Anti-thrombin deficiency, pregnancy 17 18 M Fever Systemic lupus erythematosus 18 9 F Seizure Optic chiasm germinoma, dehydration 19 36 F Headache Chronic sinusitis 20 64 M Seizure Anemia * HRTx = hormone replacement therapy
608 / Table 4. Initial CT findings Initial CT finding number percent Pathognomic sign on CT* Cord sign, only 4 22.2 Empty delta sign, only 2 11.1 Cord and Empty delta sign 4 22.2 Absent 8 44.4 Parenchymal change on CT Infarction, only 6 33.3 Hemorrhage, only 2 11.1 Hemorrhage and infarction 8 44.4 No parenchymal lesion 2 11.1 * CT = computed tomography Table 5. The CT findings and initial impression Case pathognomic signs parenchymal changes Initial impression cord sign empty delta Hemorrhage Infarction 1 + - + - CVT 2 - N* + + Hemorrhage 3 + + + - SDH, CVT 4 + N - - Encephalitis 5 - - + + Brain tumor 6 - - + + Neurobehcet 7 + + + + SAH 8 - + + + CVT 9 + N - - Wernicke disease 10 + + + - CVT 11 - N + + Hemorrhage 12 + N - - EDH or SDH 13 - - + + CVT 14 - - + - Brain tumor 15 CT scan was not taken initially Pseudotumor cerebri 16 - N - + Brain tumor 17 CT scan was not taken initially SLE flare-up 18 + + + - SAH or CVT 19 - - + + Chronic sinusits 20 - + + + SAH * N = As CT was not enhanced, empty delta sign could not be evaluated, CVT = cerebral venous thrombosis, SDH = subdural hematoma, SAH = subarachnoid hemorrhage, EDH = epidural hematoma
609 A Fig. 1. Pathognomic signs of cerebral venous thrombosis. (A) Cord sign. Pre-enhanced cranial computed tomography(ct) scan demonstrates the cord sign (arrow) as dense thrombus in superior sagittal sinus. (B) Empty delta sign. Contrastenhanced cranial CT scan shows empty delta sign (arrow head) which consists of a triangular area of enhancement or high attenuation with a relatively low-attenuating center on multiple contiguous transverse CT images obtained in the region of the superior sagittal sinus. B
610 / Fig. 3. Transfemoral cerebral angiography shows lack of flow in the right transverse and sigmoid sinus(arrows) compared to left sinus. A B Fig. 2. Magnetic resonance image(mri) with venography(mrv) (A) MRI can direct visualize the thrombus(arrow head). (B) MRV shows abnormal signal of right sigmoid sinus(arrows).
611 01. Ameri A, Bousser MG. Cerebral venous thrombosis. Neurol Clin 1992;10:459-61. 02. Bousser MG. Cerebral venous thrombosis; Diagnosis and management. J Neurol 2000;247:252-8. 03. De Bruijn SFTM, De Haan RJ. Clinical features and prognostic factors of cerebral venous and sinus thrombosis in a prospective series of 59 patients. J Neurol Neurosurg Psychiatry 2001;70:105-8. 04. Masuhr F, Mehraein S, Einhaupl K. Cerebral venous and sinus thrombosis. J Neurol 2004;251:11-23. 05. Martinelli I, Sacchi E, Landi G, Taioli E, Duca F, Mannuccio P. High risk of cerebral vein thrombosis in carriers of a prothrombin-gene mutation and in users of oral Fig. 4. Algorithm for the diagnosis and treatment of cerebral venous thrombosis. * Risk factors = all known causes of deep vein thrombosis and a number of local or regional causes of infective or noninfective disease, such as tumor, meningitis, sepsis or arterial infarct, CT = computed tomography, DDx = differential diagnosis
612 / contraceptives. N Engl J Med 1998;338:1793-7. 06. Martinelli I, Battaglioli T, Pedotti P, Cattaneo M, Mannucci PM. Hyperhomocysteinemia in cerebral vein thrombosis. Blood 2003;102:1363-6. 07. Rigamonti A, Carriero MR, Boncoraglio G, Leone M, Bussone G. Cerebral vein thrombosis and mild hyperhomocysteinemia. Neurol Sci 2002;23:225-7. 08. Cantu C, Varrinagarrimenteria F. Cerebral venous thrombosis associated with pregnancy and puerperium; Review of 67 cases. Stroke 1993;24:1880-4. 09. Ferro JM, Correia M, Rosas MJ, Pinto AN. Seizures in cerebral vein and dural sinus thrombosis. Cerebrovasc Dis 2003;15:78-83. 10. Teasdale E. Cerebral venous thrombosis; Making the most of imaging. J R Soc Med 2000;93:23-37. 11. Provenzale JM, Joseph GJ, Barboriak DP. Dural sinus thrombosis; Finding on CT and MRI imaging and diagnostic pitfall. AJR 1998;170:777-83. 12. Renowden S. Cerebral venous thrombosis. Eur Radiol 2004;14:215-26. 13. Connor SEJ, Jarosz JM. Magnetic Resonance Imaging of Cerebral venous sinus thrombosis. Radiology 2002;57:449-61. 14. Brucker AB, Vollert-Rogenhofer H, Wagner M, Stieglbauer K, Felber S, Trenkler J, et al. Heparin treatment in acute cerebral sinus thrombosis; A retrospective clinical and MR analysis of 42 cases. Cerebrovasc Dis 1998;8:331-7. 15. Casey SO, Alberico RA, Patel M, Jimenez JM, Ozsvath RR, Maguire WM, et al. Cerebral CT venography. Radiology 1996;198:163-70. 16. Breteau G, Mounier-Vehier F, Godefroy O, Gauvrit JY, Mackowiak-Cordoliani MA, Girot M, et al. Cerebral venous thrombosis; 3-year clinical outcome in 55 consecutive patients. J Neurol 2003;250:29-35. 17. Perkin GD. Cerebral venous thrombosis; Medical therapy. J R Soc Med 2000;93:238-40. 18. Mehraein S, Schmidtketke K, Villringer A, Valdueza JM, Masuhr F. Heparin treatment in cerebral sinus and venous thrombosis. Cerebrovasc Dis 2003;15:17-21. 19. Bousser MG. Cerebral venous thrombosis; Nothing, heparin or local thrombolytics? Stroke 1999;30:481-3. 20. Renowden S. Cerebral venous thrombosis; Local thrombosis. J R Soc Med 2000;93:241-3.