Cerebral Venous Thrombosis in a 35-Year-Old with Undiagnosed Sickled Cell Disease: A Case Report

BACKGROUND: Sickle cell disease (SCD) is characterized by a chronic hypercoagulable state with significant activation of circulating coagulation factors and a subsequent increase in fibrinolysis. This occurs both during sickle cell crises and in the steady state. Central nervous system involvement is a devastating manifestation of SCD, with stroke being the most severe manifestation. Cerebral venous sinus thrombosis (CVST) is uncommon in SCD. Few cases have been reported in the literature. Here, we present a rare case of a young man with CVST presenting with status epilepticus.
CASE REPORT: A 35-year-old native Indian male with no medical history presented with status epilepticus. He was asymptomatic until the age of 35 and was unaware of having SCD. A head CT revealed hyperdensities in the superior and inferior sagittal sinuses, the vein of Galen, and the straight sinuses. A venous contrast study showed a central non-enhancing area signifying a filling defect, suggestive of CVST. The diagnosis of SCD was confirmed by peripheral smear and subsequent hemoglobin electrophoresis.
CONCLUSIONS: This was a unique case of undiagnosed SCD in an adult male presenting as status epilepticus secondary to CVST. Although CVST is a very uncommon complication, it can be the only clinical presentation in patients with undiagnosed SCD. Prompt evaluation with CT and early treatment with anticoagulation can improve the outcome and decrease morbidity.

Keywords: Anemia, Sickle Cell, Status epilepticus, Thrombosis