The “Cord Sign” in Cerebral Venous Thrombosis Associated with High Plasma Levels of Factor VIII

Abstract

Cerebral venous thrombosis (CVT) is a potentially life-threatening condition that requires acute recognition and treatment. Diagnosis of CVT is challenging and requires a high index of clinical suspicion. The finding of the "cord sign" in a non-contrast cranial CT is useful for the rapid recognition of CVT in the emergency setting. We describe a patient with CVT in whom the "cord sign" and elevated factor VIII (FVIII) plasma levels were both present. A 45-year-old Caucasian man was brought to the emergency room with headache and a focal seizure. His neurological examination was non-focal. During his evaluation in the ER, a non-contrast cranial CT showed increased density of cortical veins ("cord sign"), vein of Galen, and superior sagittal, transverse and straight sinuses. A brain MRI demonstrated a flow signal void in the superior sagittal sinus, prominent cortical veins, asymmetric flow signal in the right transverse sinus, and subarachnoid hemorrhage adjacent to the superior sagittal sinus. Laboratory work-up was unremarkable and only revealed elevation of FVIII plasma levels. We report the first case of CVT with the "cord sign" and concomitant elevated FVIII. Early recognition of the "cord sign" is warranted for the emergency diagnosis and treatment of CVT.