Long-Term Neurocognitive Outcomes in Patients with Adult Moyamoya Disease.

Objective: Neurocognitive impairment is a frequent complication of moyamoya disease in adults, but the long-term outcomes of neurocognitive function remain unclear. We evaluated neurocognitive functions in a group of patients with a history of moyamoya disease and assessed the relationship between regional cerebral blood flow (rCBF) and neurocognitive functions.

Methods: We studied 53 patients with a history of adult moyamoya disease. Neurocognitive functions were evaluated with the Neurobehavioral Cognitive Status Examination (Cognistat) and the Frontal Assessment Battery. The relationship between rCBF and neurocognitive functions were assessed in patients for whom rCBF was measured using single-photon emission computed tomography with I-iodoamphetamine.

Results: Patients had a history of moyamoya disease for an average of 10.6 years, with 23 patients managed conservatively and 30 managed using revascularization. Attention, memory, conceptualization, mental flexibility, and inhibitory control were impaired but with no significant difference between conservatively and surgically treated patients. Posterior cerebral artery involvement and frontal lobe infarction were not associated with neurocognitive examination scores in nonrevascularized or revascularized patients. Orientation, comprehension, calculation, conceptualization, and sensitivity to interference correlated with rCBF in the left ventral middle cerebral artery territory (P < 0.01), whereas memory correlated with rCBF in the right dorsal anterior cerebral artery territory (P < 0.01). Conceptualization also correlated with rCBF in the left dorsal anterior cerebral artery territory (P < 0.001).

Conclusions: Our findings indicate that long-standing hypoperfusion in specific brain regions can cause related neurocognitive dysfunction even in mild moyamoya disease managed conservatively.