Dr. Levine of the SUNY Health Science Center at Brooklyn has received honorariums from Genentech for service on a scientific advisory committee and a research grant from Genentech as a principal investigator.)
This article includes discussion of cerebral venous thrombosis, cerebral vein thrombosis, CVT, venous thrombosis of the brain, cavernous sinus thrombosis, cortical vein thrombosis, lateral sinus thrombosis, and superior sagittal sinus thrombosis. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.
Cerebral venous thrombosis is a rare but important cause of stroke that is often missed or delayed in diagnosis. The author of this article provides literature on the topic. The clinical manifestations are myriad, and a high level of suspicion must be maintained in order to effectively and expeditiously identify this disorder. Infectious and noninfectious processes may cause cerebral venous thrombosis. In adults, about half of cases are associated with pregnancy and the puerperium, but numerous unusual etiologies must be considered in the remainder of patients. Anticoagulation is the mainstay of therapy in the presence of either ischemic or hemorrhagic stroke; in some dire cases, more aggressive approaches such as thrombolysis or mechanical clot disruption may be undertaken.
• Cerebral venous thrombosis is associated with an extensive range of medical disorders.
• Treatment is primarily focused on thrombus resolution, with either anticoagulation or endovascular approaches in cases with rapid neurologic deterioration.
• Intracerebral hemorrhage due to cerebral venous thrombosis is not necessarily a cause to withhold anticoagulation.
Historical note and terminology
Ribes made the first clinical description of cerebral venous thrombosis in 1825. In the preangiography era, the clinical presentation of progressive headache, papilledema, seizures, focal deficits, and coma led to the diagnosis of cerebral venous thrombosis, which was usually confirmed by the pathological findings of thrombosis of the major venous sinuses accompanied by hemorrhagic infarction. Cerebral angiography, CT, and MRI (Boukobza et al 2009) have facilitated the diagnosis of this entity and a wide range of clinical presentations has been depicted. Rigorous study of cerebral venous thrombosis has been limited due to its low incidence, and information has come mostly in the form of relatively small case series. However, in 2001, the International Study of Cerebral Vein Thrombosis completed prospective enrollment of 624 patients with this disorder and has provided a wealth of clinical data (Ferro 2002a; Ferro 2002b). The overall incidence is estimated at 1.32 per 100,000 person-years (95% CI, 1.06-1.61), and among women between the ages of 31 and 50 years, the incidence is 2.78 (95% CI, 1.98-3.82) (Coutinho et al 2012).
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