Stroke and disease progression during long-term follow-up of patients with moyamoya disease older than 50 years.

OBJECTIVES: The long-term prognosis of elderly patients with moyamoya disease (MMD) is not fully understood and needs to be elucidated.

MATERIALS AND METHODS: MMD patients who first visited our institute between 1999 and 2019, were ≥50 years of age, and were followed for ≥ 1 year were retrospectively included. Follow-up data such as stroke and disease progression on magnetic resonance angiography (MRA) were collected from medical records. The surgical outcomes of ischemic patients treated with indirect revascularization were assessed.

RESULTS: Of the 60 patients included (mean (standard deviation) = 57.0 (5.5) years, 38 females), 9 patients initially received indirect revascularization, 3 patients received direct revascularization, and 49 patients were treated conservatively. During the 57.4 (53.7) month follow-up, the symptomatic stroke rate (person-year %) was 2.79%, and MRA progression was 3.14%. Symptomatic patients had a greater rate of symptomatic stroke than asymptomatic patients did (1.46-5.74% vs. 0.94%, P=0.15), while MRA progression was more common in asymptomatic patients (0-3.83% vs. 5.64%, P=0.22). Among the 14 hemispheres of 11 patients who underwent indirect revascularization, 13/14 (92.9%) demonstrated good neovascularization, and no ischemic stroke occurred after surgery.

CONCLUSIONS: In elderly patients with MMD, MRA progression was not uncommon, especially in asymptomatic patients. Symptomatic patients exhibited a high symptomatic stroke rate, and indirect revascularization seems to be effective at reducing future ischemic stroke in ischemic patients with misery perfusion. Because follow-up events were not uncommon, lifelong follow-up seems necessary for elderly MMD patients, as well as for younger patients.