A Case of Recurrent Ischemic Stroke Involving Subacute, Progressive Intracranial Cerebral Arterial Sclerosis Prior to Diagnosis with JAK2-mutated Polycythemia Vera.

CASE REPORT: A 58-year-old man presenting with no vascular risk factors visited our hospital with right hemiparesis and total aphasia. Diffusion-weighted magnetic resonance imaging of the brain showed multiple hyperintensities in watershed distributions in the left hemisphere. Magnetic resonance angiography (MRA) revealed stenosis of the middle cerebral artery, despite normal MRA findings 2 months prior. One year after the first stroke, the patient experienced a recurrent ischemic stroke involving the left anterior choroidal artery, pulmonary embolism, and deep venous thrombosis. After the recurrent stroke event, hemoglobin levels increased gradually. Two years after the first stroke, a JAK2V-617F mutation was detected.

CONCLUSION: Our report suggests that progressive intracranial arterial sclerosis and venous thrombosis of undetermined etiologies could be several initial symptoms of polycythemia vera.