Lights Off, Lights On: Amaurosis Fugax in Polycythemia

There are many causes of amaurosis fugax, including polycythemia. Polycythemia is associated with elevated hematocrit levels and hyperviscosity, which can lead to ocular manifestations. We report a polycythemia patient with amaurosis fugax, who had resolution of ocular symptoms following venesection. A 29-year-old gentleman presented with a six-month history of episodic bilateral transient loss of vision (amaurosis fugax), followed by slow recovery back to normal after 15-20 minutes. The symptoms worsened with fatigue. He also had an unsteady gait for the preceding one year. Ocular examination was unremarkable. His visual acuity was 20/20 OU. Neurological examination revealed gait ataxia and dysdiadochokinesia. Computed tomography (CT) angiogram showed an old cerebellar infarct. Blood investigations showed persistent elevated hemoglobin and hematocrit with positive JAK-2 V617F mutation. Infective and connective tissue workups were all negative. A diagnosis of polycythemia was made by the haematology team. In addition to oral aspirin given by the neurology team, he underwent venesection with improvement in ocular symptoms following each episode of venesection. The frequency of amaurosis fugax reduced from 2-3 episodes a week to once a month, then resolved completely after five venesections. Systemically, his cerebellar symptoms also resolved and there were no neurological deficits. Polycythemia is a rare disease that can cause amaurosis fugax and thrombotic events in young patients. Better knowledge and accurate diagnosis are important, as early treatment may improve the symptoms and long-term morbidity.