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Congenital protein C deficiency and superior sagittal sinus thrombosis causing isolated intracranial hypertension
C Confavreux1, P Brunet, P Petiot
1Clinique de Neurologie, Hôpital Neurologique, Lyon, France.
Insights
Congenital protein C deficiency can cause superior sagittal sinus thrombosis leading to intracranial hypertension. Vitamin K antagonists require cautious initiation in these patients due to potential skin necrosis.
Area of Science:
- Neurology
- Hematology
- Genetics
Background:
- Cerebral venous thrombosis (CVT) is a rare condition with diverse etiologies.
- Intracranial hypertension can be an isolated symptom of CVT.
- Congenital protein C deficiency is a known risk factor for thrombotic events.
Observation:
- This report details the first case of superior sagittal sinus thrombosis (SVS) presenting with isolated intracranial hypertension.
- The patient's condition was attributed to an underlying congenital protein C deficiency.
Findings:
- Superior sagittal sinus thrombosis can manifest solely as intracranial hypertension.
- Congenital protein C deficiency is a potential, albeit rare, cause of SVS.
- Anticoagulation is the standard treatment for CVT.
Implications:
- Clinicians should consider congenital protein C deficiency in the differential diagnosis of SVS, especially with isolated intracranial hypertension.
- Initiation of vitamin K antagonists for anticoagulation in patients with congenital protein C deficiency requires careful monitoring to mitigate the risk of skin necrosis.
Abstract:
The first case of a superior sagittal sinus thrombosis causing isolated intracranial hypertension as a result of congenital protein C deficiency is reported. Such a possibility must not be overlooked. Anticoagulation is recommended as a treatment for cerebral venous thrombosis. In the case of congenital protein C deficiency, vitamin K antagonists must be started cautiously due to the risk of skin necrosis.