Prolonged and regionally progressive symptomatic cerebral hyperperfusion syndrome after superficial temporal artery-middle cerebral artery anastomosis in a patient with moyamoya disease

Abstract

The incidence of symptomatic hyperperfusion syndrome after superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis for patients with moyamoya disease (MMD) approaches 30%. In most cases, hyperperfusion occurs in a localized area and disappears within 1-2 weeks. A 59-year-old female diagnosed with asymptomatic MMD for 4 months became rapidly symptomatic with transient ischemic attacks (TIAs). After left STA-MCA anastomosis surgery, she developed symptomatic hyperperfusion, initially (1-2 weeks after surgery) manifesting with severe headache and lesions located in the left basal ganglia. She then developed (2-5 weeks after surgery) aphasia and right hemiparesis caused by new hyperperfusion lesions located in the left frontal area. At discharge (7 weeks after surgery), she recovered fully without any remaining neurologic deficit and no ischemic lesions. This report details a rare case of a patient with MMD who presented with regionally progressive hyperperfusion lesions after STA-MCA anastomosis and symptoms that persisted for 5 weeks following surgery. Results from this case suggest that regional differences exist in the functional recovery of cerebrovascular reactivity (CVR) in a patient with rapidly progressive MMD.