Unraveling the link: Beta-thalassemia intermedia and Moyamoya syndrome-a hidden connection

Beta-thalassemia intermedia (BTI) is a chronic hematologic disorder that may predispose to systemic complications, including rare cerebrovascular events. Moyamoya angiopathy (MMA) is a progressive steno-occlusive cerebrovascular disease, often presenting with recurrent strokes and transient ischemic attacks (TIAs). The association between BTI and MMA remains exceedingly rare.

We report a 25-year-old woman with BTI who developed recurrent ischemic strokes and TIAs. Imaging studies revealed bilateral chronic infarcts and stenosis of distal internal carotid and middle cerebral arteries, consistent with MMA. Laboratory investigations showed chronic anemia with hemoglobin of 8.1 g/dl. She underwent a successful right superficial temporal artery-middle cerebral artery (STA-MCA) bypass without worsening of her neurological deficits.

This case highlights the potential link between chronic anemia and progressive cerebrovascular pathology in BTI patients. Early diagnosis and surgical intervention can improve neurological outcomes in such complex cases.