Two-Year Clinical, Cerebral Hemodynamic, and Cognitive Outcomes of Adult Patients Undergoing Medication Alone for Symptomatically Ischemic Moyamoya Disease Without Cerebral Misery Perfusion: A Prospective Cohort Study.

BACKGROUND: In nonsurgical adult moyamoya disease (MMD) patients with ischemic onset and stable hemodynamics, the cerebral hemodynamic and cognitive course remains unclear.

OBJECTIVE: To clarify, through a prospective cohort study, 2-yr clinical, cerebral hemodynamic, and cognitive outcomes of adult patients receiving medication alone for symptomatically ischemic MMD without cerebral misery perfusion.

METHODS: Seventy patients without cerebral misery perfusion on the first 15O gas positron emission tomography (PET) were clinically followed up for 2 yr. The second PET was performed at the end of the 2-yr follow-up. Neuropsychological tests were also performed at the study entry and the end of the 2-yr follow-up.

RESULTS: During the 2-yr follow-up period, 2 patients (3%) developed further cerebral ischemic symptoms and showed new cerebral misery perfusion on PET performed at recurrence. None of the 68 patients without further ischemic symptoms showed cerebral misery perfusion on second PET. All 66 patients who underwent the first and second neuropsychological tests displayed unchanged interval cognition at the 2-yr follow-up.

CONCLUSION: Among adult patients receiving medication alone for symptomatically ischemic MMD without cerebral misery perfusion, the incidence of recurrent ischemic events was 3% per 2 yr. In patients without recurrent ischemic events, cerebral hemodynamics and cognitive function had not deteriorated by 2 yr after the last event.