• Peter Karzmark, Penelope D Zeifert, Teresa E Bell-Stephens, Gary K Steinberg, and Leslie J Dorfman.
• Department of Neurology and Neurological Sciences, Stanford University School of Medicine, Stanford, California 94305-5235, USA.
• Neurosurgery. 2012 Mar 1;70(3):634-8.

BackgroundAdults with moyamoya disease (MMD) have been shown to manifest cognitive impairment, but it is unclear whether this is the result of ischemic stroke.ObjectiveTo determine whether adults with MMD but without stroke have cognitive impairment.MethodsWe performed detailed neuropsychological assessments in 30 adults with angiographically confirmed MMD without magnetic resonance imaging (MRI) evidence of stroke.ResultsTwenty patients (67%) exhibited small T2 hyperintensities in the cerebral subcortical white matter on brain MRI but no evidence of gray matter damage. Significant cognitive impairment, defined as half of test scores ≥ 1 SD below the normal mean, was present in 7 patients (23%). Executive functioning, mental efficiency, and word finding were the ability areas most frequently impaired, whereas memory was relatively intact. Clinically significant emotional distress (depression and/or anxiety) was present in 11 patients (37%). Comparable cognitive findings were also observed in the subset of 10 patients (33%) with completely normal static brain MRI.ConclusionCognitive impairment in MMD can occur in the absence of ischemic stroke as manifested on MRI.

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