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Rapid Communication| Volume 6, ISSUE 5, P370-372, July 1997

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

  • J. Maurice Hourihane
    Correspondence
    Address reprint requests to J. Maurice Hourihane, MB, MRCPI, Oregon Stroke Center, L226, Oregon Health Sciences University, 3181 SW Sam Jackson Park Rd, Portland, OR 97201.
    Affiliations
    Oregon Stroke Center Oregon Health Sciences University Portland, OR., USA

    Department of Neurology, Oregon Health Sciences University Portland, OR., USA

    Department of Medicine, Oregon Health Sciences University Portland, OR., USA

    Department of Clinical Pathology, Oregon Health Sciences University Portland, OR., USA
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  • Thomas G. Deloughery
    Affiliations
    Oregon Stroke Center Oregon Health Sciences University Portland, OR., USA

    Department of Neurology, Oregon Health Sciences University Portland, OR., USA

    Department of Medicine, Oregon Health Sciences University Portland, OR., USA

    Department of Clinical Pathology, Oregon Health Sciences University Portland, OR., USA
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  • Wayne M. Clark
    Affiliations
    Oregon Stroke Center Oregon Health Sciences University Portland, OR., USA

    Department of Neurology, Oregon Health Sciences University Portland, OR., USA

    Department of Medicine, Oregon Health Sciences University Portland, OR., USA

    Department of Clinical Pathology, Oregon Health Sciences University Portland, OR., USA
    Search for articles by this author
      This paper is only available as a PDF. To read, Please Download here.
      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

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