(Stroke. 1995;26:1193-1195.)
© 1995 American Heart Association, Inc.
Articles |
Cerebral Venous Thrombosis in Adults
A Study of 40 Cases From Saudi Arabia
From the Division of Neurology (A.D., S.Al-R., M.A., A.R. Al T.) and the Department of Radiology (T.M.), King Saud University; and the Division of Neurology, King Fahad National Guard Hospital (A.A., S.Al-R., T.O.), Riyadh, Saudi Arabia.
Correspondence to Dr Abdulkader Daif, Division of Neurology, King Khalid University Hospital, PO Box 7805 (38), Riyadh 11472, Saudi Arabia.
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Abstract |
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Background and Purpose We undertook this study to determine the frequency, clinical patterns, and etiologies of cerebral venous thrombosis in a Middle Eastern country.
Methods Records of all adult patients admitted with an angiographically documented diagnosis of cerebral venous thrombosis from 1985 through 1994 in two major hospitals of Riyadh, Saudi Arabia, were reviewed.
Results Forty patients (20 men, 20 women) aged 16 to 40 years were identified. Hospital frequency was 7 per 100 000 patients, and the relative frequency against arterial strokes was 1:62.5. Nineteen cases (47%) had a clinical picture of pseudotumor cerebri. Behçet's disease was the cause in 10 cases (25%). Other causes included antiphospholipid antibodies in 4, protein S deficiency in 3, intracranial tumors in 3, systemic lupus erythematosus in 3, infections in 3, antithrombin III deficiency in 2, postpartum in 1, and oral contraceptives in 1.
Conclusions Cerebral venous thrombosis in adults is not uncommon in Saudi Arabia. Behçet's disease is the single most common etiology. Infection is no longer an important cause, whereas "new" coagulation disorders are common. Patients with a pseudotumor cerebri syndrome should undergo angiography or brain MRI before being labeled idiopathic.
Key Words: epidemiology • cerebral embolism and thrombosis • Saudi Arabia
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Introduction |
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The symptoms and signs associated with cerebral venous thrombosis (CVT) are relatively nonspecific. These include headache, papilledema, vomiting, seizures, and focal neurological deficits.1 2 CVT may be difficult to diagnose clinically1 2 because of its various and nonspecific manifestations and the multiple associated conditions and etiologies.1 2 3 4 5 6 7 Hypercoagulable states associated with puerperium as well as infectious diseases are believed to be the major causes in the third world,4 7 8 but these are less significant in western countries.1 The standards of health care in Saudi Arabia have improved tremendously in the last two decades. Infectious diseases are now diagnosed and treated promptly. In this article, 40 cases of CVT are reported. These were encountered over a 9-year period in the Kingdom of Saudi Arabia. The features of this condition as they pertain to the Middle East, including frequency, clinical patterns, and etiologies, are described.
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Subjects and Methods |
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The records of all patients with CVT seen in two large tertiary-care hospitals in Riyadh, Saudi Arabia, from 1985 through 1994 were reviewed. These two hospitals are the King Khalid Hospital, which is the main university hospital of the country, and the King Fahad Hospital, which serves mainly the members of the National Guard and their families. Both hospitals have a catchment population of approximately 1 million. The diagnosis of CVT was based on a partial or complete absence of filling of one dural sinus on two projections using intravenous digital subtraction angiography or transfemoral angiography. MR angiography was not available at the time of this study. Only cases occurring in adulthood, ie, above 15 years of age, were included in this study.
The following investigations were performed in all cases: complete blood count, erythrocyte sedimentation rate, basic blood biochemistry, prothrombin time, activated partial thromboplastin time, VDRL test, urinalysis, brain CT scan with contrast enhancement, and cerebral angiography. Cerebrospinal fluid studies were performed in 31 patients. MRI of the brain was performed in 19 patients. Other ancillary investigations, ie, levels of antithrombin III, protein C, protein S, and antiphospholipid antibodies, were performed in the most recently seen patients.
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Results |
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There were 20 men and 20 women with a mean age of 27.8 years (range, 16 to 40 years). Thirty-two patients (80%) presented with symptoms and signs of increased intracranial pressure. This was the sole manifestation in 19 patients (47%) but was associated with other symptoms and signs in the other 13 (hemiparesis, 3; aphasia, 3; alternate hemiparesis, 2; third nerve palsy, 2; coma, 2; seizures, 2; hemianesthesia, 1; and meningeal signs, 1). In the remaining 8 patients without signs of increased intracranial pressure, 1 had headache and seventh nerve palsy, 3 had hemiparesis (1 with stupor), and 1 had alternate hemiparesis. Two patients presented with focal motor seizures and 1 with an amnestic syndrome.9 The clinical features of the 40 patients are reported in Table 1
. The
mode of onset of symptoms was also highly variable. It was acute (<48
hours) in 14 patients (35%), subacute (<1 month) in 16 patients
(40%), and progressive over many months with exacerbation and
remissions in the remaining 10 patients (25%).
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Brain CT was normal in 17 patients. Hemorrhagic infarction was observed in 5 patients, and cortical hypodensities were seen in 7 others. Two patients had scattered hypodense areas in the deep white matter. The delta sign was observed in 12 patients only. Brain MRI was abnormal in 18 of 19 cases. The cerebral lesions observed on brain CT were confirmed. The most common abnormality in the veins and sinus was a hyperintense image seen on proton-density sequences. In 1 patient, brain MRI did not show the cortical venous occlusion, but cerebral angiography did.
Cerebral angiography was abnormal in all patients. Thirty-four had partial or complete occlusions of the superior sagittal sinus. This was isolated in 22 cases and associated with other sinus or venous occlusion in the remaining 12 (7 lateral sinus, 2 lateral and straight sinus, 2 cerebral vein, and 1 transverse sinus). Two of the other patients had cerebral vein occlusions, 1 of them associated with a straight sinus occlusion. The remaining 4 patients had isolated lateral sinus occlusions. Thus 34 (85%) of the patients had an occlusion of the sagittal sinus, 13 (32%) of a lateral sinus, 3 (7%) of the straight sinus, and 1 of the transverse sinus, and 4 patients (10%) had an occlusion of a cerebral vein.
Cerebrospinal fluid studies were performed in 31 patients. Twenty-nine had a high initial pressure (mean, 320 mm H2O; range, 240 to 510 mm H2O); 14 had a high protein or high cell count or both. In the remaining 17 patients, cerebrospinal fluid had a normal cell count and normal protein. One patient had chronic lymphocytic meningitis and was suspected of having either tuberculosis or sarcoidosis.
The etiologies varied also and are shown in Table 2. In
10 patients (25%) no cause was found. Seven of these patients have
been followed up for more than 2 to 5 years and have remained normal.
In 4 of the 10 patients with Behçet's disease, CVT was the
initial manifestation, and the other features of the disease developed
over 3 to 24 months. The low protein S and antithrombin III titers were
confirmed after the acute stage in 4 cases. In 2 of these the deficit
was familial.
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Eleven patients were treated by repeated lumbar puncture. Steroids and/or acetazolamide were used in 8 patients. Four patients were treated with heparin because their condition was worsening. One recovered, 2 improved but had residual deficits, and 1 died. Twenty-nine patients (72%) recovered completely. Four patients died, 2 with coma at onset and the 2 with tumors. Seven patients had neurological sequelae such as blindness (n=3), dementia (n=1), or focal deficit (n=3).
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Discussion |
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TopAbstractIntroductionSubjects and MethodsResultsDiscussionReferences |
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The first description of CVT appeared in the French literature in 1825 as a postmortem report.10 Since then many series have been published, including 795 cases between 1983 and 1990.11 However, the true incidence of CVT is still unknown. Kalbag and Woolf12 indicated that CVT was the principal cause of death in 1 per 2 million persons per year in England and Wales between 1953 and 1961. The more recent publication of large clinical series suggests that the true incidence of CVT is higher than previously thought. Our 40 patients were observed in 9 years in two of Saudi Arabia's main hospitals. The hospital frequency was 7 CVT cases per 100 000 patients. However, in view of the catchment population of the two hospitals, the incidence was probably less than 1 per 100 000 population per year. In one of the hospitals, 500 arterial strokes were observed over a similar period,13 and the overall ratio of CVT to arterial strokes was 1:62.5. However, the ratio was 1:8.5 in those patients aged 15 to 45 years.14
The neurological symptoms and signs encountered in our series were those classically associated with CVT; headache, the most frequent and often the earliest symptom, was encountered in 82% of our cases. It was present in 75% of the 110 cases reported by Ameri and Bousser15 and in 41% to 74% in other recent series.11 In older series, focal abnormalities such as motor or sensory deficits, dysphasia, or focal seizures occurred in 50% to 75% of the cases.12 16 17 The frequency of these abnormalities in our series was only 32%. This could be related to the fact that 35% of our patients were diagnosed rapidly, within 48 hours of the onset, and cases of CVT due to infectious causes were rare. Isolated intracranial hypertension with headache and papilledema mimicking idiopathic intracranial hypertension was encountered in 47% of our cases. It was reported in 20% to 40% of cases in a recent series.1 This may reflect our routine use of angiography and/or MRI in managing the patients with idiopathic intracranial hemorrhage. Unusual clinical manifestations of CVT were encountered in 5 patients, simulating an acute ischemic attack in 3 and presenting as an amnestic syndrome in 1 and as subarachnoid hemorrhage in 1.
Infection was the cause of CVT in 7% of our cases, whereas it was the cause in 16% and 17% of the cases reported by Bousser et al2 and Shell and Rathe,18 respectively. Only 1 of our patients had puerperal CVT compared with 5% and 10% in these two series.2 18 It is believed that infection and puerperium underlie the development of CVT mainly in third-world countries. Puerperal CVT, for example, was reported to be responsible for 25% of maternal deaths in India and to complicate 4.5 of 1000 obstetrical admissions.8 Six of the 38 (16%) patients in the series of Bousser et al2 had Behçet's disease. This proportion was higher than in any other published series but may have been due to the inclusion of many natives of North Africa, as suggested by these authors in a later publication19 : of 25 patients with CVT and Behçet's disease, 13 were North African, 3 African, and only 9 European. Patients with Behçet's disease constituted a quarter of our cases, which may reflect the higher prevalence of this disease in the Middle East.20 In addition, CVT was the first manifestation of this disease in 4 of our 10 patients compared with only 2 of the 25 cases reported by Wechsler et al.19 Routine screening for protein S, protein C, antithrombin III deficiencies, and antiphospholipid antibodies is very important when CVT has no apparent cause. These coagulopathies may have been implicated in many earlier reported cases of idiopathic CVT. However, even in recent reports 20% to 30% of the CVT cases have no clear etiology.1 Similarly, 25% of our cases were idiopathic.
In conclusion, CVT is not uncommon in Saudi Arabia; infectious causes are no more common in adults. Behçet's disease seems to be the single most frequent etiology in this part of the world. Because 47% of our patients were initially diagnosed with pseudotumor cerebri, this study suggests that angiography, or at least brain MRI, should be performed as part of the diagnostic workup in all patients presenting with this condition. Assessment of the "new" coagulation factors such as protein C, protein S, antithrombin III, and antiphospholipid antibodies should also become a routine investigation in every patient who has CVT without apparent cause.
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Acknowledgments |
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The authors would like to thank Dr N. Russell for his valuable review of the manuscript, Drs B. Yaqub and M. Abdulrazak for allowing the inclusion of some of their patients, and Dr V. Palkar for the review of some of the radiological data.
Received January 4, 1995; revision received March 23, 1995; accepted March 28, 1995.
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References |
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