Homozygous hereditary resistance to activated protein C presenting as cerebral venous thrombosis

J. Maurice Hourihane, Thomas G. Deloughery, Wayne M. Clark

Research output: Contribution to journalArticlepeer-review

3 Scopus citations

Abstract

The factor V Leiden mutation is a specific point mutation in the gene coding for factor V. It renders activated factor V resistant to degradation by activated protein C (APC). This hereditary resistance to APC (HRAPC) is a known risk factor for systemic venous thrombosis. We present a case of homozygous HRAPC presenting as cerebral venous thrombosis (CVT). A 24-year-old woman presented with a dense left hemiplegia and papiledema. A computed tomography scan showed a high ritht parieto-occipital infarct with hemorrhagic conversion. Angiography confirmed the diagnosis of extensive CVT. Treatment included heparin and direct intrathrombus thrombolysis initially as a bolus and then as an infusion for 21 hours. Repeat angiography showed partial recanalization. After 9 days, the patient was discharged on warfarin with minimal residual left weakness but persistent papilledema. Homozygous HRAPC appears to be a risk factor for CVT and should be considered in the evaluation of CVT.

Original languageEnglish (US)
Pages (from-to)370-372
Number of pages3
JournalJournal of Stroke and Cerebrovascular Diseases
Volume6
Issue number5
DOIs
StatePublished - Jul 1997

Keywords

  • APC
  • Cerebral venous thrombosis
  • HRAPC

ASJC Scopus subject areas

  • Surgery
  • Rehabilitation
  • Clinical Neurology
  • Cardiology and Cardiovascular Medicine

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