Early recanalisation of cerebral venous sinus thrombosis in an unusual case associated with severe protein S deficiency

Abstract

An inherited or acquired deficiency of protein S leads to a prothrombotic state, with predisposition to venous thrombosis. We describe a case of cerebral venous sinus thrombosis (CVST) associated with acquired protein S deficiency and recanalisation within 15 days of anticoagulation. A 38-year-old man presented with recurrent headache, vomiting, altered sensorium and one episode of transient left hemiparesis. Magnetic resonance venography showed poor flow in the deep cerebral venous sinuses with extensive collateral venous channel formation, which resolved after 15 days of anticoagulation, along with clinical improvement. Serum protein S activity was found to be markedly low (16% of biological reference). CVST should be suspected in a patient with acute features of raised intracranial pressure or focal neurological deficit, and a patient without obvious clinical predisposition for a prothrombotic state should be evaluated for underlying thrombophilic states like protein S deficiency.

Bilateral Abducens Nerve Palsy Due to Extensive Cerebral Venous Sinus Thrombosis

“…Elevated D-dimer results, which may indicate thrombosis [5], gradually normalized in our case, along with the process of regeneration of sinus thrombosis. Similarly to our patient, Verma et al described early recanalization of venous sinuses thrombosis with protein S deficiency after 15 days of anticoagulant therapy [4]. According to data available in the literature, a high recanalization grade of CVST independently predicts positive neurological outcome [15], while lack of venous recanalization is associated with worse clinical outcome [16].…”

“…CVST in protein S deficiency, in comparison to other thrombotic incidents, has been described in only a few case reports [4]. Makanjuola et al described the case of an extensive CVST with protein S deficiency in a pregnant woman [12].…”

“…CVST risk factors include severe dehydration, final trimester of pregnancy, puerpera, genetic disorders of the clotting cascade [2], connective tissue disease, malignancy, contraceptive use and infections such as sinusitis, otitis, and mastoiditis [3]. Protein S is a vitamin K-dependent protein that prevents clotting by acting as a cofactor for activated protein C [4]. Inherited or acquired protein S deficiency leads to the prothrombotic state, with plausible thrombosis [4].…”

“…Protein S is a vitamin K-dependent protein that prevents clotting by acting as a cofactor for activated protein C [4]. Inherited or acquired protein S deficiency leads to the prothrombotic state, with plausible thrombosis [4]. Papilloedema refers to optic disc oedema associated with increased intracranial pressure (ICP) [5].…”

Deep Cerebral Venous Sinus Thrombosis (Cvt) with Excellent Recovery in HIV Patient on Antiretroviral Therapy

“…Protease inhibitors based therapy promotes thrombosis by inducing platelets and endothelial dysfunction [13]. Nonetheless it is still unclear whether non-protease inhibitors are prothrombotic or not [11,14]. Our patient is on reverse transcriptase inhibitors.…”