Effect of moyamoya disease on neuropsychological functioning in adults.
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.
Karzmark
Peter
P
Department of Neurology, Stanford University School of Medicine, Stanford, California 94305-5234, USA. pkarzmark@stanfordmed.org
Zeifert
Penelope D
PD
Tan
Simon
S
Dorfman
Leslie J
LJ
Bell-Stephens
Teresa E
TE
Steinberg
Gary K
GK
D000328:Adult; D003072:Cognition Disorders / Q000209:etiology*; D005260:Female; D006801:Humans; D008297:Male; D009072:Moyamoya Disease / Q000150:complications* / Q000523:psychology*; D009483:Neuropsychological Tests