C
R
CASE REPORT * ETUDE DE CAS.,
Cerebral venous sinus thrombosis presenting as idiopathic intracranial hypertension Stephen Couban, MD; Charles E. Maxner, MD, FRCPC diopathic intracranial hypertension is a syndrome of elevated intracranial pressure without clinical or radiologic evidence of a mass or ventricular enlargement and with normal cerebrospinal fluid (CSF) composition.' Cerebral venous sinus thrombosis can present as apparently idiopathic intracranial hypertension.2 It is a rare condition and often not diagnosed before death. It is commonly associated with oral contraceptive use,34 pregnancy5 and systemic malignant disease6 and occasionally associated with head trauma.7'8 We describe a woman with intracranial hypertension. Although there were several risk factors for idiopathic intracranial hypertension she was found to have extensive cerebral venous sinus thrombosis. This case report will show that magnetic resonance imaging (MRI) is a specific and sensitive means of initially diagnosing and subsequently following up cerebral venous sinus thrombosis. Although rare this condition should always be considered in the differential diagnosis of apparently idiopathic intracranial hypertension, particularly if there is a history of head trauma. I
Case report A 25-year-old woman presented with a history of headache, nausea and vomiting of 3 to 4 weeks' duration. She reported photophobia and had experienced transient visual obscurations and diplopia several days before admission. Seven weeks earlier the patient had struck her central forehead while riding a motorcycle. There had been no loss of consciousness. She had gained 13 kg in the previous year. She smoked less than one pack of cigarettes per day and had used an oral contraceptive for 8 years. On admission the patient, who was moderately
obese, was irritable and had bilateral optic disc edema and no focal neurologic signs. A plain axial computed tomography (CT) scan of her head appeared normal. The CSF opening pressure was 47 cm H20, and analysis of the fluid gave normal results. Angiography of the bilateral carotid and left vertebral arteries showed poor filling of most of the superior sagittal sinus, no filling of the straight sinus and a filling defect in the right proximal transverse sinus. The extensive thrombosis was then visualized by means of MRI (Fig. 1). A search for an underlying coagulopathy was unsuccessful: the prothrombin time was 10.6 (normally 10.2 to 12.2) seconds, the partial thromboplastin time 24.7 (normally 24.0 to 33.4) seconds and the platelet count 400 (normally 150 to 350) x 109/L. The plasma concentration of protein C was 2.09 (normally 0.60 to 1.40) U/mL, protein S (total) 1.05 (normally 0.75 to 1.43) U/mL, protein S (free) 0.31 (normally 0.25 to 0.55) U/mL and plasminogen 3.8 (normally 2.5 to 4.1) U/mL. No anticardiolipin antibody was found, and the plasma antithrombin III level was 1.04 (normally 0.82 to 1.16) U/mL. There was no clinical evidence to suggest lupus, and the antinuclear antibody titre (1/100, speckled pattern) was only weakly positive. The patient was given acetaminophen-codeine tablets (each containing 300 and 30 mg respectively) as necessary and acetazolamide (500 mg orally twice daily). She became asymptomatic over the ensuing 3 months, and the papilledema resolved. An MRI scan 5 months after presentation demonstrated substantial resolution of the thrombosis and recanalization of the superior sagittal sinus (Fig. 2).
Comments A high degree of clinical suspicion is required to
From the departments of Medicine and Ophthalmology, Dalhousie University, Halifax, NS
Reprint requests to: Dr. Charles E. Maxner, Department of Medicine, Camp Hill Medical Centre, Gerard Hall, 5303 Morris St., Halifax, NS B3J IB6 SEPTEMBER 15,1991
CAN MED ASSOC J 1991; 145 (6)
657
diagnose cerebral venous sinus thrombosis, because tients to thrombosis9 and - in our experience the presentation is often nonspecific. Our patient head injury is not commonly associated with idiohad many features typical of idiopathic intracranial pathic intracranial hypertension, cerebral angioghypertension, including female sex, low age, obesity raphy was performed. The diagnosis of cerebral venous sinus thrombowith recent weight gain, oral contraceptive use and normal cerebrospinal fluid. However, because head sis rests on radiologic imaging of the cerebral venous trauma may alter coagulation and predispose pa- system. A CT scan may exhibit pathognomonic signs
Fig. 1: Magnetic resonance imaging (MRI) scan. Top: Extracellular methemoglobin representative of extensive thrombosis in vein of Galen (white arrow), straight sinus (arrowhead) and superior sagittal sinus (open arrow). Bottom: Obliteration of vein of Galen (black arrow) and superior sagittal sinus (open arrow) by thrombus. 658
CAN MED ASSOC J 1991; 145 (6)
Fig. 2: MRI scan 5 months after presentation. Top: Signal void in vein of Galen (white arrow) and superior sagittal sinus (open arrow) indicative of normal blood flow and hence resolution of thrombosis. Bottom: Signal void in superior sagittal sinus (open arrow) indicative of resolution of thrombosis. LE 15 SEPTEMBRE 1991
of the condition,'0 be nonspecific" or appear normal. Cerebral angiography remains the gold standard, but there is an appreciable risk associated with the procedure. MRI is a safe and noninvasive technique,12-'4 and as this case suggests it may be the diagnostic procedure of choice. In addition, MRI can be used to follow up patients with cerebral venous sinus thrombosis and thus provide a better description of the natural history of the thrombosis. The issue of treatment is controversial, some authors suggesting measures for anticoagulation or thrombolysis and others advocating supportive therapy only.'5 Because our patient was in a stable clinical state she was treated with acetazolamide to reduce her intracranial hypertension. Over time there was marked resolution of the extensive thrombosis. We thank Dr. Nancy Morrison for referring the patient, Dr. Grant Llewellyn for his assistance in interpreting the radiographs and Ms. Carrie Campbell for her secretarial assistance.
References
Conferences continuedfrom page 654 Nov. 24-27, 1991: 4th 'Annual OPTIMA Conference Building Bridges to Well-being Radisson Hotel, Toronto Patti Etkin, conference coordinator, Donwood Institute, 175 Brentcliffe Rd., Toronto, ON M4G 3Z1; (416) 425-3930, fax (416) 425-7896
Nov. 26-27, 1991: Medical Literature Analysis and Retrieval System (MEDLARS) Course - Intro II Ottawa Health Sciences Resource Centre, Canada Institute for Scientific and Technical Information, National Research Council of Canada, Ottawa, ON KlA 0S2; (613) 993-1604, fax (613) 952-8244
Nov. 28, 1991: Medical Literature Analysis and Retrieval System (MEDLARS) Course - Advanced Ottawa Health Sciences Resource Centre, Canada Institute for Scientific and Technical Information, National Research Council of Canada, Ottawa, ON KlA 0S2; (613) 993-1604, fax (613) 952-8244
1. Ahiskog JE, O'Neill BP: Pseudotumor cerebri. Ann Intern
Med 1982; 97: 249-256 2. Gates PC, Barnett HJM: Venous disease: cortical veins and sinuses. In Barnett HJM, Mohr JP, Stein BM et al (eds): Stroke: Pathophysiology, Diagnosis and Management, Churchill, Baltimore, Md, 1986: 731-743 3. Atkinson EA, Fairburn B, Heathfield KW: Intracranial venous thrombosis as a complication of oral contraception. Lancet 1970; 1: 914-918 4. Buchanan DS, Brazinsky JH: Dural sinus and cerebral vein thrombosis: iflcidence in young women receiving oral contraceptives. Arch Neurol 1970; 22: 440-444 5. Cross JN, Castro PO, Jennett WB: Cerebral strokes associated with pregnancy and the puerperium. BMJ 1968; 3: 214-218 6. Sigsbee B, Deck MDF, Posner JB: Non-metastatic superior sagittal sinus thrombosis complicating systemic cancer. Neurology 1979; 29: 139-146 7. Barnett HJM, Hyland HH: Non-infective intracranial venous thrombosis. Brain 1953; 76: 36-49 8. Kinal ME: Traumatic thrombosis of dural venous sinuses in closed head injuries. JNeurosurg 1967; 27: 142-145 9. Olson JD, Kaufman HH, Moake J et al: The incidence and significance of hemostatic abnormalities in patients with head injuries. Neurosurgery 1989; 24: 825-832 10. Nussel F, Huber P: High resolution computed tomography of superior sagittal sinus thrombosis and abnormalities. Neuroradiology 1989; 31: 307-311 11. Hulcelle PJ, Dooms GC, Mathurin P et al: MRI assessment of unsuspected dural sinus thrombosis. Neuroradiology 1989; 31: 217-221 12. Snyder TC, Sachdev HS: MR imaging of cerebral dural sinus thromboses. J Comput Assist Tomogr 1986; 10: 889-891 13. McMurdo SK, Brant-Zawadski M, Bradley WG et al: Dural sinus thrombosis: study using intermediate field strength MR imaging. Radiology 1986; 161: 83-86 14. Macchi PJ, Grossman RI, Gomari JM et al: High field MRI of cerebral venous thrombosis. J Comput Assist Tomogr 1986; 10: 10-15 15. Bousser MG, Chiras J, Sauron B et al: Cerebral venous thrombosis: a review of 38 cases. Stroke 1985; 16: 199-213 SEPTEMBER 15, 1991
Dec. 11-13, 1991: National Institutes of Health Consensus Development Conference on Acoustic Neuroma Warren Grant Magnuson Clinical Center, National Institutes of Health, Bethesda, Md. Conference Registrar, Prospect Associates, 500-1801 Rockville Pike, Rockville, MD 20852; (301) 468-MEET, fax (301) 770-5164 Dec. 11-15, 1991: American Back Society Fall Symposium on Back Pain San Francisco Marriott Hotel Dr. Aubrey A. Swartz, executive director, American Back Society, Ste. 401, 2647 E 14th St., Oakland, CA 94601; (415) 536-9929, fax (415) 536-1812
Feb. 23-26, 1992: Pan-American Doctors' Medical Society for Generalists and Specialists (Canadian section) 47th Annual Meeting
Manzanillo, Mexico Dr. D.P. Hill, Continuing Medical Education, Ottawa General Hospital, 501 Smyth Rd., Ottawa, ON K1H 8L6; (613) 737-8455 Mar. 30-Apr. 3, 1992: 8th World Conference on Tobacco OR Health - Building a Tobacco-Free World (cosponsors include the Canadian Cancer Society and the American Medical Association) Sheraton Buenos Aires Hotel, Buenos Aires, Argentina Secretariat, American Cancer Society, 1599 Clifton Rd. NE, Atlanta, GA 30329-4251; (404) 329-7638, fax (404) 325-2217 CAN MED ASSOC J 1991; 145 (6)
659
R
CASE REPORT * ETUDE DE CAS.,
Cerebral venous sinus thrombosis presenting as idiopathic intracranial hypertension Stephen Couban, MD; Charles E. Maxner, MD, FRCPC diopathic intracranial hypertension is a syndrome of elevated intracranial pressure without clinical or radiologic evidence of a mass or ventricular enlargement and with normal cerebrospinal fluid (CSF) composition.' Cerebral venous sinus thrombosis can present as apparently idiopathic intracranial hypertension.2 It is a rare condition and often not diagnosed before death. It is commonly associated with oral contraceptive use,34 pregnancy5 and systemic malignant disease6 and occasionally associated with head trauma.7'8 We describe a woman with intracranial hypertension. Although there were several risk factors for idiopathic intracranial hypertension she was found to have extensive cerebral venous sinus thrombosis. This case report will show that magnetic resonance imaging (MRI) is a specific and sensitive means of initially diagnosing and subsequently following up cerebral venous sinus thrombosis. Although rare this condition should always be considered in the differential diagnosis of apparently idiopathic intracranial hypertension, particularly if there is a history of head trauma. I
Case report A 25-year-old woman presented with a history of headache, nausea and vomiting of 3 to 4 weeks' duration. She reported photophobia and had experienced transient visual obscurations and diplopia several days before admission. Seven weeks earlier the patient had struck her central forehead while riding a motorcycle. There had been no loss of consciousness. She had gained 13 kg in the previous year. She smoked less than one pack of cigarettes per day and had used an oral contraceptive for 8 years. On admission the patient, who was moderately
obese, was irritable and had bilateral optic disc edema and no focal neurologic signs. A plain axial computed tomography (CT) scan of her head appeared normal. The CSF opening pressure was 47 cm H20, and analysis of the fluid gave normal results. Angiography of the bilateral carotid and left vertebral arteries showed poor filling of most of the superior sagittal sinus, no filling of the straight sinus and a filling defect in the right proximal transverse sinus. The extensive thrombosis was then visualized by means of MRI (Fig. 1). A search for an underlying coagulopathy was unsuccessful: the prothrombin time was 10.6 (normally 10.2 to 12.2) seconds, the partial thromboplastin time 24.7 (normally 24.0 to 33.4) seconds and the platelet count 400 (normally 150 to 350) x 109/L. The plasma concentration of protein C was 2.09 (normally 0.60 to 1.40) U/mL, protein S (total) 1.05 (normally 0.75 to 1.43) U/mL, protein S (free) 0.31 (normally 0.25 to 0.55) U/mL and plasminogen 3.8 (normally 2.5 to 4.1) U/mL. No anticardiolipin antibody was found, and the plasma antithrombin III level was 1.04 (normally 0.82 to 1.16) U/mL. There was no clinical evidence to suggest lupus, and the antinuclear antibody titre (1/100, speckled pattern) was only weakly positive. The patient was given acetaminophen-codeine tablets (each containing 300 and 30 mg respectively) as necessary and acetazolamide (500 mg orally twice daily). She became asymptomatic over the ensuing 3 months, and the papilledema resolved. An MRI scan 5 months after presentation demonstrated substantial resolution of the thrombosis and recanalization of the superior sagittal sinus (Fig. 2).
Comments A high degree of clinical suspicion is required to
From the departments of Medicine and Ophthalmology, Dalhousie University, Halifax, NS
Reprint requests to: Dr. Charles E. Maxner, Department of Medicine, Camp Hill Medical Centre, Gerard Hall, 5303 Morris St., Halifax, NS B3J IB6 SEPTEMBER 15,1991
CAN MED ASSOC J 1991; 145 (6)
657
diagnose cerebral venous sinus thrombosis, because tients to thrombosis9 and - in our experience the presentation is often nonspecific. Our patient head injury is not commonly associated with idiohad many features typical of idiopathic intracranial pathic intracranial hypertension, cerebral angioghypertension, including female sex, low age, obesity raphy was performed. The diagnosis of cerebral venous sinus thrombowith recent weight gain, oral contraceptive use and normal cerebrospinal fluid. However, because head sis rests on radiologic imaging of the cerebral venous trauma may alter coagulation and predispose pa- system. A CT scan may exhibit pathognomonic signs
Fig. 1: Magnetic resonance imaging (MRI) scan. Top: Extracellular methemoglobin representative of extensive thrombosis in vein of Galen (white arrow), straight sinus (arrowhead) and superior sagittal sinus (open arrow). Bottom: Obliteration of vein of Galen (black arrow) and superior sagittal sinus (open arrow) by thrombus. 658
CAN MED ASSOC J 1991; 145 (6)
Fig. 2: MRI scan 5 months after presentation. Top: Signal void in vein of Galen (white arrow) and superior sagittal sinus (open arrow) indicative of normal blood flow and hence resolution of thrombosis. Bottom: Signal void in superior sagittal sinus (open arrow) indicative of resolution of thrombosis. LE 15 SEPTEMBRE 1991
of the condition,'0 be nonspecific" or appear normal. Cerebral angiography remains the gold standard, but there is an appreciable risk associated with the procedure. MRI is a safe and noninvasive technique,12-'4 and as this case suggests it may be the diagnostic procedure of choice. In addition, MRI can be used to follow up patients with cerebral venous sinus thrombosis and thus provide a better description of the natural history of the thrombosis. The issue of treatment is controversial, some authors suggesting measures for anticoagulation or thrombolysis and others advocating supportive therapy only.'5 Because our patient was in a stable clinical state she was treated with acetazolamide to reduce her intracranial hypertension. Over time there was marked resolution of the extensive thrombosis. We thank Dr. Nancy Morrison for referring the patient, Dr. Grant Llewellyn for his assistance in interpreting the radiographs and Ms. Carrie Campbell for her secretarial assistance.
References
Conferences continuedfrom page 654 Nov. 24-27, 1991: 4th 'Annual OPTIMA Conference Building Bridges to Well-being Radisson Hotel, Toronto Patti Etkin, conference coordinator, Donwood Institute, 175 Brentcliffe Rd., Toronto, ON M4G 3Z1; (416) 425-3930, fax (416) 425-7896
Nov. 26-27, 1991: Medical Literature Analysis and Retrieval System (MEDLARS) Course - Intro II Ottawa Health Sciences Resource Centre, Canada Institute for Scientific and Technical Information, National Research Council of Canada, Ottawa, ON KlA 0S2; (613) 993-1604, fax (613) 952-8244
Nov. 28, 1991: Medical Literature Analysis and Retrieval System (MEDLARS) Course - Advanced Ottawa Health Sciences Resource Centre, Canada Institute for Scientific and Technical Information, National Research Council of Canada, Ottawa, ON KlA 0S2; (613) 993-1604, fax (613) 952-8244
1. Ahiskog JE, O'Neill BP: Pseudotumor cerebri. Ann Intern
Med 1982; 97: 249-256 2. Gates PC, Barnett HJM: Venous disease: cortical veins and sinuses. In Barnett HJM, Mohr JP, Stein BM et al (eds): Stroke: Pathophysiology, Diagnosis and Management, Churchill, Baltimore, Md, 1986: 731-743 3. Atkinson EA, Fairburn B, Heathfield KW: Intracranial venous thrombosis as a complication of oral contraception. Lancet 1970; 1: 914-918 4. Buchanan DS, Brazinsky JH: Dural sinus and cerebral vein thrombosis: iflcidence in young women receiving oral contraceptives. Arch Neurol 1970; 22: 440-444 5. Cross JN, Castro PO, Jennett WB: Cerebral strokes associated with pregnancy and the puerperium. BMJ 1968; 3: 214-218 6. Sigsbee B, Deck MDF, Posner JB: Non-metastatic superior sagittal sinus thrombosis complicating systemic cancer. Neurology 1979; 29: 139-146 7. Barnett HJM, Hyland HH: Non-infective intracranial venous thrombosis. Brain 1953; 76: 36-49 8. Kinal ME: Traumatic thrombosis of dural venous sinuses in closed head injuries. JNeurosurg 1967; 27: 142-145 9. Olson JD, Kaufman HH, Moake J et al: The incidence and significance of hemostatic abnormalities in patients with head injuries. Neurosurgery 1989; 24: 825-832 10. Nussel F, Huber P: High resolution computed tomography of superior sagittal sinus thrombosis and abnormalities. Neuroradiology 1989; 31: 307-311 11. Hulcelle PJ, Dooms GC, Mathurin P et al: MRI assessment of unsuspected dural sinus thrombosis. Neuroradiology 1989; 31: 217-221 12. Snyder TC, Sachdev HS: MR imaging of cerebral dural sinus thromboses. J Comput Assist Tomogr 1986; 10: 889-891 13. McMurdo SK, Brant-Zawadski M, Bradley WG et al: Dural sinus thrombosis: study using intermediate field strength MR imaging. Radiology 1986; 161: 83-86 14. Macchi PJ, Grossman RI, Gomari JM et al: High field MRI of cerebral venous thrombosis. J Comput Assist Tomogr 1986; 10: 10-15 15. Bousser MG, Chiras J, Sauron B et al: Cerebral venous thrombosis: a review of 38 cases. Stroke 1985; 16: 199-213 SEPTEMBER 15, 1991
Dec. 11-13, 1991: National Institutes of Health Consensus Development Conference on Acoustic Neuroma Warren Grant Magnuson Clinical Center, National Institutes of Health, Bethesda, Md. Conference Registrar, Prospect Associates, 500-1801 Rockville Pike, Rockville, MD 20852; (301) 468-MEET, fax (301) 770-5164 Dec. 11-15, 1991: American Back Society Fall Symposium on Back Pain San Francisco Marriott Hotel Dr. Aubrey A. Swartz, executive director, American Back Society, Ste. 401, 2647 E 14th St., Oakland, CA 94601; (415) 536-9929, fax (415) 536-1812
Feb. 23-26, 1992: Pan-American Doctors' Medical Society for Generalists and Specialists (Canadian section) 47th Annual Meeting
Manzanillo, Mexico Dr. D.P. Hill, Continuing Medical Education, Ottawa General Hospital, 501 Smyth Rd., Ottawa, ON K1H 8L6; (613) 737-8455 Mar. 30-Apr. 3, 1992: 8th World Conference on Tobacco OR Health - Building a Tobacco-Free World (cosponsors include the Canadian Cancer Society and the American Medical Association) Sheraton Buenos Aires Hotel, Buenos Aires, Argentina Secretariat, American Cancer Society, 1599 Clifton Rd. NE, Atlanta, GA 30329-4251; (404) 329-7638, fax (404) 325-2217 CAN MED ASSOC J 1991; 145 (6)
659
