Malignant isolated cortical vein thrombosis with type II protein S deficiency: a case report

BACKGROUND: The incidence of cerebral venous thrombosis (CVT) is low, and in particular, isolated cortical vein thrombosis (ICVT) is very rare. The diagnosis of ICVT is difficult by using conventional computed tomography (CT) and magnetic resonance imaging (MRI). However, with appropriate treatment, ICVT has a good prognosis. CASE PRESENTATION: Herein, we present a rare case of a 40-year-old woman with ICVT and type II protein S (PS) deficiency, who experienced a stroke. She initially presented with generalized convulsions. A CT scan showed intracerebral hemorrhage (ICH) in the left temporoparietal region. However, her condition rapidly deteriorated and she went into a coma approximately 20 h after admission. A second CT scan revealed significant ICH expansion and transfalcine herniation. Decompressive hemicraniectomy with duraplasty was performed, and ICVT was confirmed owing to abnormal vascular tone and black appearance of the cortical vein. She underwent anticoagulation therapy and rehabilitation, and gradually recovered. CONCLUSION: We experienced an extremely rare case of isolated cortical vein thrombosis related with type II PS deficiency. CT-digital subtraction angiography is a useful supportive technique in the diagnosis of ICVT. Decompressive hemicraniectomy is effective for hemorrhage extension cases, and ICVT with hemorrhage might require early anticoagulation therapy.