Outcomes of Adult Patients with Ischemic Moyamoya Disease Based on Prospective In-house Cohorts

Abstract

Herein, we present the results of four prospective in-house cohort studies investigating adult patients with ischemic moyamoya disease (aMMD). From the results of this analysis, we concluded the following: Patients not treated with misery perfusion should first receive strict medical management alone, including cilostazol, and should undergo revascularization surgery when ischemic symptoms recur. The incidence of angiographic disease progression was 2.4% per year in patients with aMMD without misery perfusion who underwent medical management alone. Patients with further ischemic events generally exhibited angiographic disease progression with reduced cerebral perfusion. Further, the incidence of an interval increase in cerebral microbleeds was 3.2% per year in patients with aMMD without misery perfusion who underwent medical management alone, and this increase was associated with cognitive decline. Approximately one-third of patients with aMMD with cerebral misery perfusion who underwent direct revascularization surgery developed irreversible cognitive decline due to cerebral hyperperfusion. Cognitive decline is caused by de novo cerebral microbleeding and delayed brain atrophy. In patients with aMMD with misery perfusion, indirect revascularization surgery alone resulted in sufficient collateral circulation, improved cerebral hemodynamics, and the recovery of cognitive function. The latter two beneficial effects were greater than those in aMMD patients treated with direct revascularization surgery. Periventricular anastomosis regressed following indirect revascularization surgery alone for aMMD with misperfusion. Finally, medical management alone was associated with considerably poor outcomes in aMMD patients with misery perfusion.