Cerebral Vein Thrombosis due to Hereditary Antithrombin III Deficiency

Abstract

Cerebral vein thrombosis, also called superior sagittal sinus thrombosis, is a well recognized clinical and radiologic entity associated with a variety of medical disorders. We report a patient with fatal cerebral vein thrombosis following myelography, in whom the cause was familial antithrombin III (AT3) deficiency. Unsuspected AT3 deficiency should be considered in cases of unexplained cerebral venous thromboses. RESUME: Thrombose veineuse cerebrale due a une deficience hereditaire en antithrombine III. La thrombose veineuse cerebrale, egalement appelee thrombose du sinus sagittal superieur, est une entite associee a des affections medicales variees et bien reconnue au point de vue clinique et radiologique. Nous rapportons le cas d'un patient qui a presente, suite a une myelographie, une thrombose veineuse cerebrale causee par un deficit familial en antithrombine III (AT3). Un deficit insoupconne en AT3 devrait etre envisage dans les cas de thrombose cerebrale veineuse inexpliquee.Can. J. Neurol. Sci. 1993; 20:158-161 Cerebral vein thrombosis consists of thrombosis of the superior sagittal sinus, other cortical veins, and sometimes deep cerebral veins. The use of the previous term "superior sagittal sinus thrombosis" is to be discouraged, because the involved veins usually extend beyond the superior sagittal sinus. Neurologic symptoms and signs usually include those of raised intracranial pressure, seizures, and focal neurological deficits. Cerebral vein thrombosis was initially described in association with skull trauma and pyogenic infections of the scalp, sinuses and meninges, but since then an extensive list of other causes or associated medical disorders has accumulated (see Table l). 1 5 A variety of coagulation abnormalities occur in some of these disorders. However we are aware of only one case report linking antithrombin III (AT3) deficiency with cerebral vein thrombosis. Ambruso et al. described a 15-year-old boy with prior history of a calf deep venous thrombosis which was initially treated with heparin, then warfarin. 6 Three months after warfarin was discontinued the patient developed cerebral sinus thrombosis and testing revealed AT3 deficiency. AT3 is a heparin cofactor that blocks the coagulation cascade by inhibiting activated coagulation factors (Figure 1). AT3 deficiency can be familial or acquired and these patients are at risk for thrombotic events. 7 CASE REPORTA 24-year-old man was admitted to hospital because of seizures and headache. He had previously been well except for chronic low back pain for which he had been electively admitted to another hospital for investigation three days earlier. A myelogram had been performed (Isovue non-ionic contrast medium, Squibb, Princeton, NJ) and was normal. The following day he complained of headache, neck pain, photophobia, nausea and vomiting. The headache worsened and he then had a right arm focal motor seizure. This recurred five times over the next 24 hours. He was treated with intravenous phenytoin and transferred to our hospita...

Not Just Another Headache: Cerebral Venous Sinus Thrombosis in a Patient With Isolated Antithrombin III Deficiency

“…To our knowledge, the present case is unique because it is only one of a few reports of an adult with CVST due to isolated AT III deficiency [15,16]. The patient was 61 years old and male, both of which are unordinary, as most patients are less than 50 years old and female [1,4,9].…”