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Dural sinus thrombosis associated with activated protein C resistance: MR imaging findings and proband identification
J M Provenzale1, D P Barboriak, T L Ortel
1Department of Radiology, Duke University Medical Center, Durham, NC 27710-3808, USA.
Insights
Dural sinus thrombosis is frequently linked to activated protein C resistance, a hypercoagulable state. Testing for activated protein C resistance is recommended for patients with dural sinus thrombosis to identify causes and at-risk families.
Area of Science:
- Neurology
- Hematology
- Genetics
Background:
- Dural sinus thrombosis (DST) is a rare but serious condition.
- Hypercoagulable states are known risk factors for thrombosis.
- Activated protein C resistance (APCR) is an inherited thrombophilia.
Purpose of the Study:
- To investigate the association between dural sinus thrombosis and activated protein C resistance.
- To determine the prevalence of APCR in patients with DST.
Main Methods:
- Retrospective analysis of patients diagnosed with DST.
- Screening for activated protein C resistance using laboratory assays.
- Clinical and radiological assessment of thrombosis and hemorrhagic complications.
Main Results:
- Four out of five patients with DST tested positive for APCR.
- Hemorrhagic venous infarction was observed in three out of four patients with DST and APCR.
- APCR was identified as a significant risk factor in this patient cohort.
Conclusions:
- A high prevalence of APCR was found in patients with DST.
- Testing for APCR should be considered in the evaluation of DST.
- Identifying APCR can aid in understanding thrombosis etiology and family screening.
Objective:
The purpose of this study was to report the association of dural sinus thrombosis with a hypercoagulable state associated with activated protein C resistance.
Conclusion:
In our small study population, hemorrhagic venous infarction was common (three of four patients) among patients with dural sinus thrombosis and activated protein C resistance. Four of five patients with dural sinus thrombosis had positive tests for activated protein C resistance. This finding, in conjunction with data from other studies, suggests that patients with dural sinus thrombosis may need to be studied for the presence of activated protein C resistance. A positive finding for activated protein C resistance can be important not only in helping to explain the cause of thrombosis in affected individuals but also in identifying families at risk for thrombosis.