Thrombose veineuse cérébrale secondaire à une thrombocytémie essentielle

Abstract

Essential thrombocythemia (ET) is a myeloproliferative syndrome; cerebral venous thrombosis (CVT) is a rare complication. We report the case of a 20-year-old woman with an uneventful history who was admitted with intracranial hypertension syndrome which had developed over the last four months in association with bilateral decline of visual acuity. Physical examination at admission revealed stage II papilloedema and absence of any focal neurological signs. The brain MRI and the venous MRA showed a thrombosis involving the superior longitudinal sinus and the lateral sinus. The etiological analysis disclosed essential thrombocythemia (ET). The patient was given an antiedema and anticoagulant treatment. Later, an etiological cytoreductive therapy was initiated. Signs of intracranial hypertension regressed progressively with persistence of acute visual disorders associated with sequelar optical atrophy. Discussion. ET is a rare cause of CVT. Reports in the literature have discussed the mechanisms, the physiology, the therapeutic modalities and the clinical course of these CVTs secondarily to ET. Stroke, especially transient ischemic attack, is the usual thrombotic expression of myeloproliferative syndrome. CVT is much more exceptional.