title: Neurocognitive impairment in adults with moyamoya disease without stroke.
abstract: BACKGROUND
  Adults with moyamoya disease (MMD) have been shown to manifest cognitive impairment, but it is unclear whether this is the result of ischemic stroke.
  
  
  OBJECTIVE
  To determine whether adults with MMD but without stroke have cognitive impairment.
  
  
  METHODS
  We performed detailed neuropsychological assessments in 30 adults with angiographically confirmed MMD without magnetic resonance imaging (MRI) evidence of stroke.
  
  
  RESULTS
  Twenty 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.
  
  
  CONCLUSIONS
  Cognitive impairment in MMD can occur in the absence of ischemic stroke as manifested on MRI.
authors.affiliation: Department of Neurology and Neurological Sciences, Stanford University School of Medicine, Stanford, California 94305-5235, USA.
authors.forename: Peter
authors.initials: P
authors.lastname: Karzmark
authors.forename: Penelope D
authors.initials: PD
authors.lastname: Zeifert
authors.forename: Teresa E
authors.initials: TE
authors.lastname: Bell-Stephens
authors.forename: Gary K
authors.initials: GK
authors.lastname: Steinberg
authors.forename: Leslie J
authors.initials: LJ
authors.lastname: Dorfman
mesh_terms: D000223:Adaptation, Psychological; D000328:Adult; D000342:Affective Symptoms; D002545:Brain Ischemia / Q000473:pathology; D003072:Cognition Disorders / Q000453:epidemiology* / Q000473:pathology* / Q000523:psychology; D056344:Executive Function; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008568:Memory; D008875:Middle Aged; D009072:Moyamoya Disease / Q000453:epidemiology* / Q000473:pathology* / Q000523:psychology; D009483:Neuropsychological Tests; D015995:Prevalence; D020521:Stroke / Q000473:pathology