Neurocognitive dysfunction in adult moyamoya disease

Abstract

We wanted to determine the neurocognitive profile of adult patients with moyamoya disease prior to neurosurgical intervention. The experience of three United States medical centers, Columbia University, University of Illinois at Chicago, and the University of Texas Southwestern Medical Center at Dallas, were combined. Clinical data from adult patients (N = 29) referred for neuropsychological evaluation from 1996 to 2008 were reviewed. Neurocognitive functioning was assessed using standardized neuropsychological tests and all data were converted to z-scores. Memory, attention, processing speed, verbal memory, visuo-spatial, language, and executive functions were examined. Cognitive dysfunction was defined as performance in two or more cognitive domains 1.5 standard deviations below age-corrected normative means OR one or more cognitive domains two standard deviations below age-corrected normative means. Manual strength and dexterity, as well as depressive symptoms, were also assessed. Two-thirds of patients demonstrated neurocognitive dysfunction. A large proportion of patients were found to have pronounced cognitive dysfunction (>2 SD below the mean) on tests of processing speed (29%), verbal memory (31%), verbal fluency (26%) and executive function (25%). Manual strength and dexterity were also affected in many patients, with impairment found in 36-58% of patients. Twenty-eight percent of patients reported moderate to severe depression, but depressive symptoms did not correlate with neurocognitive findings. A large proportion of adults with moyamoya disease demonstrate disruption of neurocognition in a broad range of functions, particularly those mediated by subcortical and frontal regions. The pattern of deficits suggests a mechanism of diffuse small vessel disease possibly caused by chronic hypoperfusion.

Different aspects of dysexecutive syndrome in patients with moyamoya disease and its clinical subtypes

“…6,7,13,26,31 We also found that various aspects of executive function, including semantic inhibition, executive processing, working memory, and sustained attention were significantly impaired in the patients. Moreover, the 2-back RTs in the patients with MMD were greater than that of the healthy controls, indicating that the controls spent more time thinking about how to achieve accuracy.…”

“…2,13,16,21,32 MMD has variable clinical presentations. The disease causes ischemic stroke, intracranial hemorrhage, headache, seizures, and transient ischemic attacks (TIAs).…”

“…11,17,23 Patients with MMD are known to exhibit impairments in some cognitive functions, especially executive functions. 13,16,19,29,30,34 For example, Karzmark et al assessed executive function in 30 patients with MMD by examining the total number of correct responses on the Delis-Kaplan Executive Functioning System Design Fluency Test, Letter and Category Fluency Tests, and part B of the Trail Making Test and found that some of these patients had impaired executive functioning. 19 Notably, executive function is an umbrella term for cognitive processes, including inhibition, working memory, reasoning, task flexibility, problem solving, and planning and execution.…”

“STA-MCA bypass with encephalo-duro-myo-synangiosis combined with bifrontal encephalo-duro-periosteal-synangiosis” as a one-staged revascularization strategy for pediatric moyamoya vasculopathy

“…[2][3][4][5][6][7] In addition to symptoms that can be ascribed to the MCA territory, children may present with lower extremity motor weakness and neuropsychological dysfunctions probably due to involvement of the frontal lobes. [8][9][10][11][12] In pediatric MMV, the cerebral blood flow (CBF) in the bifrontal areas as well as in the anterior watershed territory may continue to worsen despite good collateral formation or successful revascularization of the MCA territory. [9][10][11][12] Therefore, it is important to consider timely revascularization of the frontal areas, to prevent neurocognitive decline in pediatric patients.…”

Combined Bypass Technique for Contemporary Revascularization of Unilateral MCA and Bilateral Frontal Territories in Moyamoya Vasculopathy

“…However, revascularization in the frontal territory is receiving gradually more attention: a considerable part of the ischemic presentation of (pediatric) moyamoya patients consists of neurocognitive disorders as well as lower extremity function, caused by frontal hypoperfusion. [2,5,12,24] Re-establishing cerebral blood flow (CBF) in the frontal territory may prevent, stabilize or improve neurocognitive decline. [6][7]15,[17][18][19][20]24] In this paper, we describe a combined one-staged procedure for revascularization of the MCA territory unilaterally by a direct STA-MCA bypass and the bifrontal territory by an encephalo-duro-periosteal-synangiosis (EDPS).…”