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

Abstract

obJective Dysexecutive syndrome is common in patients with moyamoya disease (MMD), a chronic cerebrovascular disease that is characterized by stenosis of the bilateral internal carotid arteries and progressive collateral revascularization, and MMD can be classified as ischemic or hemorrhagic according to the disease presentation and history. In this study, the authors aimed to determine which aspects of executive function are impaired in patients with MMD, in addition to the specific dysexecutive functions present among its clinical subtypes and the mechanisms underlying dysexecutive function in these patients. [SART]) to 49 patients with MMD and 47 IQ-, age-, education-, and social status-matched healthy controls. The dysexecutive questionnaire (DEX) was also used to assess participants' subjective feelings about their executive function. A total of 39 of the patients were evaluated by CT perfusion (CTP) before the assessments were performed, and the correlations among the performances of the patients on the above tests with the parameters of cerebral blood volume, cerebral blood flow (CBF), mean transit time (MTT), and time-to-peak (TTP) in the frontal lobes of these patients were also analyzed. results Many aspects of executive function in the patients with MMD were significantly poorer than those in the healthy controls, and the patients performed particularly poorer on the VF test, HSCT, N-back test, and SART. The patients with hemorrhagic MMD exhibited worse executive inhibition, executive processing, and semantic inhibition compared with those with ischemic MMD, but the latter group presented a worse working memory and poorer sustained attention. There were no significant differences in the DEX scores between the patients with MMD and healthy controls. The other findings were as follows: CBF was significantly positively correlated with the number correct on part B of the HSCT (r = 0.481, p = 0.01) and accuracy on the 0-back task of the N-back (r = 0.346, p = 0.031); MTT was significantly positively correlated with accuracy on the 2-back task of the N-back (r = 0.349, p = 0.034) and factor 5 of the DEX (r = 0.359, p = 0.032); and TTP was significantly positively correlated with the number correct on part B of the HSCT (r = 0.402, p = 0.034) and the 1-back reaction time of the N-back (r = 0.356, p = 0.026). coNclusioNs The patients with MMD exhibited impairments in semantic inhibition, executive processing, working memory, and sustained attention, but they were not aware of these deficits. Moreover, differences in dysexecutive function existed between the different subtypes of MMD. Hypoperfusion of the frontal lobe may be related to working memory and semantic inhibition impairments in patients with MMD.

Cognitive Performance Profile in Pediatric Moyamoya Disease Patients and Its Relationship With Regional Cerebral Blood Perfusion

“…Anatomical location of advanced cognitive function has undergone intensive investigation. Despite a few studies reporting a correlation between hemodynamic compromise in temporal lobe and cognitive impairment (26,30), more researchers found that regions in the frontal lobe are linked to executive function, both from aspects of hemodynamics and metabolism (4,15,31,32). Using multivariate linear regression to adjust for confounders, we found that the average CBF of the left temporal lobe independently associated with processing speed.…”

“…Correlation between cerebral blood perfusion and cognitive function in adult MMD patients has been investigated, and hypoperfusion in several specific brain regions had been identified as responsible for corresponding cognitive deficits. Both cerebral vascular reserve and CBF in the frontal lobe were proved to correlate to executive functioning in adult MMD patients (4,15). Moreover, the associations between CBF in several brain areas, including left ventral middle cerebral artery territory and bilateral dorsal anterior cerebral artery territory, and multiple aspects of cognitive function had also been reported (27).…”

“…Moyamoya disease (MMD) is a cerebrovascular disease characterized by idiopathic stenosis or occlusion of the end of internal carotid arteries (1,2). Impaired cognitive function is recognized in both adult and pediatric MMD patients (3), and has been hypothetically ascribed to chronic hypoperfusion in adult MMD patients (4). However, the presence of this phenomenon in pediatric patients has been poorly reported.…”

Mapping altered brain connectivity and its clinical associations in adult moyamoya disease: A resting-state functional MRI study

“…Cognitive dysfunction resulting from MMD has begun to garner the attention of researchers studying the disorder [ 14 , 15 , 18 ]. However, despite the increasing use of rs-fMRI to investigate brain function in other cognitive disorders, few published studies have used rs-fMRI to investigate cognitive dysfunction in MMD, [ 17 , 20 , 23 ].…”

“…Moyamoya disease (MMD) is a rare cerebrovascular disease characterized by a fine vascular network (“moyamoya vessels”) at the base of the brain, which results from stenosis or occlusion of the terminal branches of the internal carotid arteries [ 13 ]. In addition to cerebral stroke, patients with this condition may present with cognitive impairments, such as executive dysfunction, impaired working memory, and attention deficit, likely as a result of chronic ischemic injury [ 14 – 16 ]. Early detection of altered brain function due to ischemia could potentially improve the quality of life of such patients, in whom chronic ischemia can lead to suboptimal brain development in children and adolescents, and/or the early onset of cognitive decline in adults [ 14 – 18 ].…”