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Cerebral venous sinus thrombosis and dural arteriovenous fistula associated with protein S deficiency: a case series

Hui Liang1, Congjie Xu2, Jiyi Xu3,4

  • 1Department of Neurology, Hainan General Hospital, Hainan Affiliated Hospital of Hainan Medical University, Supported by Hainan Province Clinical Medical Center, Haikou, China.

BMC Neurology
|May 3, 2022
PubMed

Insights

Protein S deficiency is linked to cerebral venous sinus thrombosis (CVST) and dural arteriovenous fistula (AVF). Physicians should consider PS deficiency in patients with these conditions, particularly when they coexist.

Area of Science:

  • Neurology
  • Vascular Medicine
  • Hematology

Background:

  • Cerebral venous sinus thrombosis (CVST) and dural arteriovenous fistula (AVF) are serious cerebrovascular conditions.
  • Protein S (PS) deficiency is a known risk factor for thrombosis.
  • The association between CVST, dural AVF, and PS deficiency requires further elucidation.

Purpose of the Study:

  • To characterize patients presenting with both CVST and dural AVF in the context of Protein S deficiency.
  • To analyze the clinical presentation, radiological findings, and outcomes of this specific patient cohort.

Main Methods:

  • Retrospective review of medical records from Hainan General Hospital (2000-2020).
  • Comprehensive literature search of PubMed, Embase, and CBM databases for co-occurring CVST, dural AVF, and PS deficiency.
  • Analysis of clinical data, lesion location, disease progression, PS levels, treatment strategies, and patient prognosis.

Main Results:

  • A total of 6 cases (1 from the hospital, 5 from literature) were analyzed.
  • Common symptoms included headache, seizures, and altered consciousness.
  • CVST predominantly affected the internal cerebral veins, while dural AVF involved sinuses like transverse and sigmoid, and parietal regions.
  • Dural AVF developed months to years after CVST in two instances.

Conclusions:

  • Protein S deficiency should be considered in patients diagnosed with CVST, especially when accompanied by dural AVF.
  • This association highlights the importance of thrombophilia screening in specific cerebrovascular presentations.
  • Early recognition and management of PS deficiency may improve outcomes for patients with complex CVST and dural AVF.
Abstract

Keywords:
Cerebral venous sinus thrombosisClinical characteristicsDural arteriovenous fistulaProtein S deficiency

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