Improvement in cognitive function and cerebral perfusion after bur hole surgery in an adult with moyamoya disease

Abstract

Recent studies have suggested that cognitive impairment may be a common complication in adults with moyamoya disease (MMD). However, the mechanisms of cognitive dysfunction have not been clarified. Whether cognitive impairment may occur as a consequence of cerebral hypoperfusion and may improve after revascularization surgery has not been determined. A 39-year-old West Indian woman with subacute dysexecutive cognitive syndrome and no history of stroke was diagnosed with MMD. Magnetic resonance imaging showed an old, small cerebral infarction in the left frontal white matter and no evidence of recent cerebral ischemia. Perfusion MR imaging with acetazolamide challenge demonstrated a reduced cerebrovascular reserve in both frontal lobes. Revascularization with bur hole surgery was performed, which resulted in complete regression of initial cognitive impairment. Improvement in cognitive function correlated with the development of transdural collaterals on angiography and improvement in cerebral perfusion on MR imaging. This case suggests a relationship between cognitive dysfunction and cerebral hypoperfusion in MMD. Cognitive impairment may be potentially reversible after bur hole surgery and cerebral perfusion improvement.