title: Effect of moyamoya disease on neuropsychological functioning in adults.
abstract: OBJECTIVE
  Moyamoya disease is a cerebrovascular disorder characterized by progressive occlusion of vessels comprising the circle of Willis, resulting in formation of collaterals that have a cloudy appearance on angiography. Neuropsychological research on the cognitive effects of the disorder in adults has been limited in scope and generalizability; only a few case studies have been published. The current study was intended to more comprehensively document the nature of cognitive impairment in moyamoya disease by assessing a large number of adult cases with a neuropsychological assessment test battery.
  
  
  METHODS
  Thirty-six adult patients with neurodiagnostically confirmed moyamoya disease were given presurgical neuropsychological assessments.
  
  
  RESULTS
  Mean group performances were within normal limits for all measures assessed. The highest rate of impairment was for measures of executive functioning. The lowest rates occurred with memory and perception measures. Cognitive impairment was present in 11 (31%) of the patients; it was judged to be moderate to severe in four patients (11%). Five patients reported a mild level of depression, and two patients reported a moderate level.
  
  
  CONCLUSIONS
  The present findings suggest that moyamoya disease diagnosed in adults can impair cognition but that the effect is not as severe as in pediatric cases. Executive functioning is most affected. Memory and, to a large extent, intellect are spared. The current pattern of results suggests brain region-behavior correlations that deserve further study.
authors.affiliation: Department of Neurology, Stanford University School of Medicine, Stanford, California 94305-5234, USA. pkarzmark@stanfordmed.org
authors.forename: Peter
authors.initials: P
authors.lastname: Karzmark
authors.forename: Penelope D
authors.initials: PD
authors.lastname: Zeifert
authors.forename: Simon
authors.initials: S
authors.lastname: Tan
authors.forename: Leslie J
authors.initials: LJ
authors.lastname: Dorfman
authors.forename: Teresa E
authors.initials: TE
authors.lastname: Bell-Stephens
authors.forename: Gary K
authors.initials: GK
authors.lastname: Steinberg
mesh_terms: D000328:Adult; D003072:Cognition Disorders / Q000209:etiology*; D005260:Female; D006801:Humans; D008297:Male; D009072:Moyamoya Disease / Q000150:complications* / Q000523:psychology*; D009483:Neuropsychological Tests