A Case of Cerebral Venous Sinus Thrombosis in a Patient with Sickle Cell Disease

Abstract

We report a 30 year old woman with sickle cell disease (SCD) who presented with a severe right side headache and neck pain of 1 week duration. Results of a peripheral smear showed sickle cells and sickling test was positive. Magnetic resonance venography showed dural venous sinus thrombosis involving right transverse and sigmoid sinuses with venous infarcts involving right temporal & occipital lobes. The patient was treated with low molecular weight heparin, mannitol and her symptoms and signs resolved.

Unusual Distribution of Cerebral Venous Thrombosis in a Patient With Sickle Cell Disease: A Case Study

“…The majority had frequent emergency visits due to painful vaso-occlusive crises. Only two reported a history of acute chest syndrome [7][8][9][10][11][12][13][14]. Our patient's presentation, characterized by generalized body pain, aphasia, ataxia, and confusion, aligns with a diagnosis of CVT and highlights cerebral involvement.…”

“…A literature review uncovered 10 instances of CVT in patients diagnosed with sickle cell disease. Our patient, at 29 years of age, falls within the age range (18 to 37 years) identified in these cases; the average age reported was 26 years [7][8][9][10][11][12][13][14][15]. While the man-to-woman ratio was 7:3, the available data isn't sufficient to form definitive conclusions.…”

“…Our patient's presentation, characterized by generalized body pain, aphasia, ataxia, and confusion, aligns with a diagnosis of CVT and highlights cerebral involvement. Based on our review, seizures, including an incident of status epilepticus, were the predominant presenting symptom, occurring in four out of the 10 cases [8][9][10]. Other common neurological symptoms included photophobia, dysphasia, aphasia, and headache.…”

“…Other common neurological symptoms included photophobia, dysphasia, aphasia, and headache. Additional symptoms included hemiparesis, confusion, drowsiness, neck pain, facial weakness, facial pain, and chest pain [7][8][9][10][11][12][13][14][15].…”

“…For our case in particular, both imaging techniques were employed to reach the final diagnosis. A review of the literature revealed a slight preference for CT over MRI, potentially due to the similarity in presentation with stroke, where rapid hemorrhage exclusion is crucial [7][8][9][10][11][12][13][14]. Our patient's occlusion pattern was distinctive, involving the internal cerebral veins, the vein of Galen, and the straight sinus.…”