Functional and Neuropsychological Outcome After Surgical Treatment of Moyamoya Disease.

BACKGROUND: Moyamoya disease (MMD) is a rare cerebrovascular disease characterized by progressive stenosis of the supraclinoid internal carotid artery. As a result of chronically decreased brain perfusion, eloquent areas of the brain become hypoperfused, leading to cognitive changes in patients. Repeated infarcts and bleeds produce clinically apparent neurologic deficits. OBJECTIVES: 1) To study the functional and neuropsychological outcome in MMD after revascularization surgery. 2) To find postrevascularization correlation between functional and neuropsychological improvement and radiologic improvement. METHODS: A single-center prospective and analytic study was carried out including 21 patients with MMD during the study period from March 2021 to December 2022. Patients were evaluated and compared before and after revascularization for functional, neuropsychological, and radiologic status. RESULTS: Postoperative functional outcome in terms of modified Rankin Scale score showed improvement in 33.33% of cases (P = 0.0769). An overall improving trend was observed in different neuropsychological domains in both adult and pediatric age groups. However, the trend of neuropsychological improvement was better in adults compared with pediatric patients. Radiologic outcome in the form of the Angiographic Outcome Score (AOS) significantly improved after revascularization (P = 0.0001). There was a trend toward improvement in magnetic resonance imaging (MRI) perfusion in the middle cerebral artery and anterior cerebral artery territories, 4.7% (P = 0.075) and 9.33% (P = 0.058) respectively, compared with preoperative MRI perfusion. CONCLUSIONS: After revascularization, significant improvement occurred in functional and neuropsychological status. This result was also shown radiologically as evidenced by improvement in MRI perfusion and cerebral angiography.

Long-term study of the cognitive profile of Moyamoya Disease in adults.

Moyamoya Disease (MMD) is a rare cerebrovascular disorder which can have significant cognitive consequences. The aim of the current study was to describe comprehensively the domain-specific cognitive profile of adult patients with MMD and to assess whether this changes in the absence of recurrent stroke over long-term follow-up. Comprehensive neuropsychological assessment covering seven cognitive domains was conducted on 61 adult patients with MMD at baseline and then at up to 3 further time points during follow up (median=2.31, 4.87 and 7.12 years). Although 27 patients had had prior surgical revasculariation, none had surgery between neuropsychological assessments. Cognitive impairment was common. At baseline, impairment in executive functions was most frequent (57%), followed by performance IQ (36%), speed of information processing (31%) and visual memory (30%). We found that the neuropsychological profile remains broadly stable over long-term follow-up with no clear indication of improvement or significant decline. The pattern of impairment also did not differ depending on age of onset or whether there was a history of either prior stroke at presentation or revascularisation surgery at presentation.

Classify patients with Moyamoya disease according to their cognitive performance might be helpful in clinical and practical with support vector machine based on hypergraph.

Moyamoya disease (MMD) patients were now classified according to their cerebrovascular manifestations, with cognition and emotion ignored, which attenuated the therapy. The present study tried to classify them based on their cognitive and emotional performance and explored the neural basis underlying this classification using resting-state fMRI (rs-fMRI). Thirty-nine MMD patients were recruited, assessed mental function and MRI scanned. We adopted hierarchical analysis of their mental performance for new subtypes. Next, a three-step analysis, with each step consisting of 10 random cross validation, was conducted for robust brain regions in classifying the three subtypes of patients in a support vector machine (SVM) model with hypergraph of rs-fMRI. We found three new subtypes including high depression-high anxiety-low cognition (HE-LC, 50%), low depression-low anxiety-high cognition (LE-HC, 14%), and low depression-low anxiety-low cognition (LE-LC, 36%), and no hemorrhagic MMD patients fell into the LE-HC group. The temporal and the bilateral superior frontal cortex, and so forth were included in all 10 randomized SVM modeling. The classification accuracy of the final three-way classification model was 67.5% in average of 10 random cross validation. In addition, the S value between the frontal cortex and the angular cortex was positively correlated with the anxiety score and backward digit span (p < .05). Our results might provide a new perspective for MMD classification concerning patients' mental status, guide timely surgery and suggest angular cortex, and so forth should be protected in surgery for cognitive consideration.

Specific learning disorder in mathematics and moyamoya disease: A case report.

Moyamoya disease (MMD) is a rare neurological condition that causes impaired blood flow to the brain, transient ischemic attacks or strokes, and accompanying cognitive impairments, especially in executive functioning. There is little data on the impact of this rare condition on academic outcomes in late childhood and adolescence. Here, we present the case of Ms. X, a 17-year-old white female diagnosed with MMD, who presented with evidence of a specific learning disorder (SLD) in mathematics. Ms. X was diagnosed with MMD at 6 years old and underwent revascularization surgery. Though she recovered well and progressed adequately in home schooling, she and her mother noticed a decline in memory and academic performance around 16 years old, prompting a neuropsychological evaluation. Cognitive testing revealed low average overall cognitive abilities with impaired planning and organizational skills. While her reading and spelling skills were consistent with her 10th grade academic level, she scored in the 1st percentile on the WRAT-5 Math Computation section, and her mathematical skills were estimated to be at a 2nd grade level. This case adds to the literature by documenting a specific area of academic deficit in an adolescent with MMD. The case highlights that individuals with MMD, especially those with similar executive deficits, may experience selective learning challenges in mathematics. Children with MMD may benefit from specialized academic services and interventions in specific areas of difficulty.

Recovery of Cortical Neurotransmitter Receptor Function and Its Impact on Cognitive Improvement after Indirect Revascularization Surgery Alone for Adult Patients with Ischemic Moyamoya Disease: 123I-Iomazenil Single-Photon Emission Computed Tomography Study.

OBJECTIVE: Brain 123I-iomazenil single-photon emission computed tomography (SPECT) can assess the distribution of the binding potential of central benzodiazepine receptors in the cerebral cortex. This binding potential may reflect neuronal function in viable tissues. The present prospective study using brain 123I-iomazenil SPECT aimed to determine whether improvements in cognitive function after indirect revascularization surgery alone are associated with postoperative recovery in neurotransmitter receptor function in the affected cerebral hemisphere among adult patients with moyamoya disease accompanied by ischemic presentation due to misery perfusion. METHODS: Twenty-two patients who underwent indirect revascularization surgery alone also underwent brain SPECT scanning at 180 minutes after 123I-iomazenil administration and neuropsychological testing before and at 6 months after surgery. The affected-to-contralateral cerebral hemispheric asymmetry of tracer uptake before and after surgery was then calculated. RESULTS: The asymmetry of tracer uptake was significantly increased after surgery (P < 0.0001). A significant difference between the preoperative and postoperative asymmetry of tracer uptake was seen in patients with improved cognition compared with those with unchanged cognition (P = 0.0001). The area under the receiver operating characteristic curve was 0.99 for the difference between the preoperative and postoperative asymmetry of tracer uptake to assess the ability to discriminate patients with improved cognition from those with unchanged cognition. CONCLUSIONS: Improvements in cognitive function after indirect revascularization surgery alone are associated with postoperative recovery in the binding potential of central benzodiazepine receptors in the affected cerebral hemisphere in adult patients with moyamoya disease accompanied by ischemic presentation due to misery perfusion.

Angiographic, Cerebral Hemodynamic, and Cognitive Outcomes of Indirect Revascularization Surgery Alone for Adult Patients With Misery Perfusion due to Ischemic Moyamoya Disease.

BACKGROUND: Revascularization surgery for adult patients with ischemic moyamoya disease (MMD) may improve both cognitive function and cerebral perfusion. OBJECTIVE: To determine angiographic, cerebral hemodynamic, and cognitive outcomes of indirect revascularization surgery alone for adult patients with misery perfusion due to ischemic MMD (IDR group) and to test the superiority of indirect revascularization surgery for cognitive improvement by conducting comparisons with historical control patients who had undergone direct revascularization surgery (DR group) through prospective cohort study with historical controls. METHODS: Twenty adult patients with cerebral misery perfusion underwent encephalo-duro-myo-arterio-pericranial-synangiosis alone. Cerebral angiography through arterial catheterization, brain perfusion single-photon emission computed tomography, and neuropsychological testing were performed preoperatively and at 6 months postoperatively. RESULTS: In 17 patients of the IDR group, collateral flows that were newly formed after surgery on angiograms fed more than one-third of the middle cerebral artery (MCA) cortical territory. In the IDR group, perfusion in the MCA territory was significantly increased after surgery (P < .0001), and the difference in MCA perfusion between before and after surgery was significantly greater (P = .0493) compared with the DR group. Improved cognition was significantly more frequent in the IDR group (65%) than in the DR group (31%, P = .0233). CONCLUSION: Indirect revascularization surgery alone forms sufficient collateral circulation, improves cerebral hemodynamics, and recovers cognitive function in adult patients with misery perfusion due to ischemic MMD. The latter 2 beneficial effects may be higher when compared with patients undergoing direct revascularization surgery.

Application of intraoperative electrocorticography in bypass surgery for adult moyamoya disease: a preliminary study.

OBJECTIVE: Postoperative complications of surgical revascularization in moyamoya disease (MMD) are difficult to predict because of poor knowledge of the underlying pathophysiological process. Since the aim of surgery is to improve brain dynamics by increasing regional blood flow, we hypothesize that postoperative complications are closely related to aberrant electrophysiological changes. Thus, we evaluated the clinical significance of intraoperative electrocorticography (iECoG) in bypass surgery for adult MMD. METHODS: Ninety-one adult patients operated on by the same neurosurgeon in our institute were involved (26 in the iECoG group, 65 in the traditional group). Two 1 × 6 subdural electrode grids were placed parallel to the middle frontal gyrus and superior temporal gyrus to record ECoG data continuously during the procedure in the iECoG group. Selected from several M4 candidate arteries, the recipient artery was determined to be closer to the cortex with lower power spectral density (PSD) in the beta band. The PSD parameter we used was the (delta+theta)/(alpha+beta) (DTAB) ratio (DTABR). Next, the pre- and post-bypass PSD values were evaluated, and correlations between post-/pre-bypass PSD parameter ratios and neurological/neuropsychological performance (in terms of changes in National Institutes of Health Stroke Scale [NIHSS] and Mini-Mental State Examination [MMSE] scores) were analyzed. RESULTS: Postoperative complications (transient neurological events) in the iECoG group were significantly lower than those in the traditional group (p = 0.046). In the iECoG group, the post-/pre-bypass DTABR ratio in the bypass area was significantly correlated with postoperative NIHSS (p = 0.002, r2 = 0.338) and MMSE changes (p = 0.007, r2 = 0.266). In the nonbypass area, neither postoperative NIHSS nor MMSE changes showed a significant correlation with the post-/pre-bypass DTABR ratio (p > 0.05). Additionally, patients with postoperative complications exhibited significantly higher DTABR (1.67 ± 0.33 vs. 0.95 ± 0.08, p = 0.003) and PSD of the theta band (1.54 ± 0.21 vs. 1.13 ± 0.08, p = 0.036). CONCLUSIONS: This study is the first to explain and guide surgical revascularization from the perspective of electrophysiology. Intraoperative ECoG is not only sensitive in reflecting and predicting postoperative neurological and cognitive performance but also usable as a reference for recipient artery selection.

Changes of cerebral cortical structure and cognitive dysfunction in "healthy hemisphere" after stroke: a study about cortical complexity and sulcus patterns in bilateral ischemic adult moyamoya disease.

BACKGROUND: Moyamoya disease (MMD) is an uncommon cerebrovascular disease which leads to progressive stenosis and occlusion of the bilateral internal carotid artery and main intracerebral arteries. Concerns are always on how the hemisphere with infarction affects cognitive function, while little attention is paid to the role that the non-infarcted hemisphere plays. Therefore, we aimed to detect cortical indexes, especially cortical complexity in the left or right hemisphere separately in patients with MMD after stroke. METHODS: 28 patients with MMD (14 males, 14 females) and 14 healthy controls were included in this study. All participants underwent cognitive tests and magnetic resonance imaging (MRI) scan. The preprocessing of three-dimensional T1 weighted images were performed by standard surface-based morphometry. Surface-based morphometry statistical analysis was carried out with a threshold of False Discovery Rate (FDR) P < 0.05 and fractal dimension (FD) was used to provide a quantitative description of cerebral cortical complexity. RESULTS: Widespread cognitive dysfunctions were found in MMD patient with stroke. Extensive FD reduction in the left hemisphere with right-sided infarction, mainly in the superior temporal, inferior frontal, and insula, while the post central gyrus, superior parietal, and inferior parietal gyrus also showed a wide range of significant differences (FDR corrected P < 0.05). Meanwhile, FD changes in the right hemisphere with left-sided infarction are restricted to the precuneus and cingulate isthmus (FDR corrected P < 0.05). CONCLUSIONS: Extensive cognitive impairment was reconfirmed in Moyamoya disease with stroke, while wild and asymmetrical decrease of cortical complexity is observed on both sides. These differences could be relative to unbalanced cognitive dysfunction, and may be the result of a long-term chronic ischemia and compensatory of the contralateral hemisphere to the infarction.

Impairments in brain perfusion, executive control network, topological characteristics, and neurocognition in adult patients with asymptomatic Moyamoya disease.

BACKGROUND: Asymptomatic Moyamoya disease (MMD) impairs hemodynamic and cognitive function. The relationship between these changes, cerebral blood flow (CBF), and network connectivity remains largely unknown. The aim of this study was to increase understanding of the relationship between CBF, functional networks, and neurocognition in adults with asymptomatic MMD. We compared CBF and functional status in 26 patients with MMD and 20 healthy controls using arterial spin labeling and resting state functional magnetic resonance imaging sequences. At the same time, a detailed cognitive test was performed in 15 patients with no cerebral or lumen infarction who were selected by magnetic resonance imaging-T2 FLAIR screening. RESULTS: Compared to the controls, the patients showed varying degrees of decline in their computational ability (simple subtraction, p = 0.009; complex subtraction, p = 0.006) and short-term memory (p = 0.042). The asymptomatic MMD group also showed decreased CBF in the left anterior central and left inferior frontal gyri of the island flap with multiple node abnormalities in the brain network and reduced network connectivity. There was a significant association of these changes with cognitive decline in the MMD group. CONCLUSIONS: In patients with asymptomatic MMD, disturbance of CBF and impaired brain network connections may be important causes of cognitive decline and appear before clinical symptoms. Clinical trial registration-URL: http://www.chictr.org.cn Unique identifier: ChiCTR1900023610.

[Factors Influencing Health Behavior Compliance in Adult Moyamoya Patients].

PURPOSE: The purpose of this study was to identify factors influencing health behavior compliance in adult patients with moyamoya. METHODS: A descriptive correlation study was conducted to investigate the factors influencing health behavior compliance. Participants were 142 adult patients diagnosed with moyamoya disease who were hospitalized or visited an outpatient clinic in the Gyeonggi province. Data were collected from December 16, 2019 to April 14, 2020 using self-report questionnaires and analyzed using the IBM SPSS 26.0 Win software. RESULTS: The hierarchical multiple regression analysis demonstrated that self-efficacy (ß = .60, p < .001), social support (ß = .13, p = .032), and age (ß = .21, p = .005) affected the health behavior of adults with moyamoya disease. These 3 variables explained 62.0% of the variance of health behavior compliance, and the most influential factor was self-efficacy. CONCLUSION: Based on the results of this study, it concludes that nursing interventions should be focused on self-efficacy and social support to improve health behavior compliance with adult patients diagnosed with moyamoya disease. For that, various strategies to enhance self-efficacy and social support should be developed and actively applied in the clinical setting for adult moyamoya patients.

PedsQL Score Post Encephalo-duro-arterio-myo-synangiosis Procedure for Moyamoya Disease: A Single Center Experience.

BACKGROUND: Moyamoya disease is a progressive, occlusive arteriopathy of the intracranial vessels causing an increased risk of stroke. It often results in functional impairment and decrease in quality of life, both in the presence and absence of stroke. Revascularization is the accepted treatment for patients with symptomatic moyamoya disease, preventing further stroke. At Leeds Children's Hospital we use the encephalo-duro-arterio-myo-synangiosis (EDAMS) technique to facilitate revascularization. We aim to assess the quality of life outcomes of patients who have undergone operative intervention at our unit for moyamoya disease. METHODS: Pediatric patients with operated moyamoya disease from Leeds Children Hospital between February 2009 and January 2019 were included. Patients awaiting primary surgery were excluded. Patients were contacted via telephone and the Pediatric Quality of Life Inventory 4.0 (PedsQL) questionnaire administered via parent proxy. Quality of life outcomes were assessed using the PedsQL questionnaire score, which measures physical, emotional social and school functioning domains. This score was then converted to a health-related quality of life score. Scores in each domain and mean scores were compared with normative data. RESULTS: This study included 11 children (5 boys), median age 6.8 years (range: 22 months to 15.5 years), and 9 children underwent bilateral operations. Mean parent proxy PedsQL score was 66 (range: 12.5-98.4), with a mean score of 61.9 in physical function, 88.9 in emotional function, 70.9 in social function, and 58.7 in school function. This was lower than healthy controls overall and in each individual domain except emotional function, which was similar to normative data. CONCLUSIONS: Children with moyamoya disease have a lower quality of life than healthy controls within this series. This suggests that children with moyamoya should be offered additional psychosocial support within the community.

Psychometric Properties of the Korean Version of the PsyMate Scale Using a Smartphone App: Ecological Momentary Assessment Study.

BACKGROUND: Ecological momentary assessment (EMA) is a method for capturing the changes in the variables in daily life with increased accuracy and decreased recall bias. The PsyMate scale assesses momentary moods in daily life and can be used in various settings. OBJECTIVE: The aim of this study was to develop a Korean version of the PsyMate (K-PsyMate) scale and evaluate its psychometric properties by using the EMA method in patients with moyamoya disease (MMD) in South Korea. METHODS: Patients with MMD aged over 18 years were recruited from July 2018 to January 2019 at the inpatient and outpatient departments of a university hospital in South Korea. The K-PsyMate scale comprising 13 items was developed following a translation/back translation approach of the English version and loaded onto a mobile app. Participants were instructed to enter their moods 4 times a day for 7 consecutive days. Content validity index, factor analysis, and Pearson's correlation were performed for validity analysis. For reliability, intraclass correlation coefficients between the first and last measurements were estimated by mean rating, absolute agreement, and a 2-way mixed-effects model. Usability was analyzed through a descriptive analysis, 2-tailed t test, and analysis of variance, and the results were confirmed by Mann-Whitney U test and the Kruskal-Wallis test, as the dependent variable was not normally distributed. RESULTS: In total, 1929 assessments from 93 patients were analyzed. The mean age of the participants was 40.59 (SD 10.06) years, and 66 (71%) of the 93 participants were women. Content validity was excellent as content validity index was 0.99, and 2 factors, negative affect and positive affect, were derived by an exploratory factor analysis. The correlations between the subdomains of the K-PsyMate scale and Hospital Anxiety and Depression Scale were significant (P<.001). The agreement between the first and last measurements was poor to moderate according to the obtained intraclass correlation coefficient values. Usability was evaluated by 67 (72%) out of the 93 participants. The participants rated the accuracy of assessing their momentary moods on the app at 4.13 (SD 0.97), easiness in understanding questions, operating, and inputting answers at 4.12 (SD 0.88), and interruption by the survey alarms at 2.48 (SD 1.02) out of 5. CONCLUSIONS: The K-PsyMate scale has good validity but poor to moderate agreement, which reflects the characteristics of the EMA data collected in real and natural living environments without control. The findings of our study show that the K-PsyMate scale uploaded in a mobile app can be a valid and reliable tool for evaluating the momentary mood of patients with MMD because using a mobile app is convenient and patients are familiar with their own smartphones, which they use in their daily lives.

Stress and mood of adults with moyamoya disease: A cross-sectional study.

Moyamoya disease in adults is a chronic, progressive disorder characterized by fine collateral vessel networks in the brain. The disorder can lead to negative mood and stress, which, left unresolved, may increase adverse health outcomes. We conducted a cross-sectional survey to examine stress and mood of adults with moyamoya disease. Participants were recruited at a university hospital in Seoul, Korea. Data were collected through questionnaires and review of participants' electronic medical records. A total of 109 adults participated. Significant correlations were found between perceived stress, anxiety, and depression. Adults with moyamoya disease experience anxiety, depression, and stress related to the risk of cerebral hemorrhage or ischemia, similar to those with other cerebrovascular disease. If negative mood and stress were uncontrolled, those can cuase adverse health outcomes. Health professionals caring for people with moyamoya disease should carefully observe their stress and mood and develop interventions tailored to stages of disease to help them manage. The study results provide baseline information for understanding the level of, and the factors associated with, stress and mood.

Combined structural and diffusion tensor imaging detection of ischemic injury in moyamoya disease: relation to disease advancement and cerebral hypoperfusion.

OBJECTIVE: The microstructural integrity of gray and white matter is decreased in adult moyamoya disease, suggesting covert ischemic injury as a mechanism of cognitive dysfunction. Establishing a microstructural brain imaging marker is critical for monitoring cognitive outcomes following surgical interventions. The authors of the present study determined the pathophysiological basis of altered microstructural brain injury in relation to advanced arterial occlusion, cerebral hypoperfusion, and cognitive function. METHODS: The authors examined 58 patients without apparent brain lesions and 30 healthy controls by using structural MRI, as well as diffusion tensor imaging (DTI). Arterial occlusion in each hemisphere was classified as early or advanced stage based on MRA and posterior cerebral artery (PCA) involvement. Regional cerebral blood flow (rCBF) was measured with N-isopropyl-p-[123I]-iodoamphetamine SPECT. Furthermore, cognitive performance was examined using the Wechsler Adult Intelligence Scale, Third Edition and the Trail Making Test (TMT). Both voxel- and region of interest-based analyses were performed for groupwise comparisons, as well as correlation analysis, using parameters such as cognitive test scores; gray matter volume; fractional anisotropy (FA) of association fiber tracts, including the inferior frontooccipital fasciculus (IFOF) and superior longitudinal fasciculus (SLF); PCA involvement; and rCBF. RESULTS: Compared to the early stages, advanced stages of arterial occlusion in the left hemisphere were associated with a lower Performance IQ (p = 0.031), decreased anterior cingulate volumes (p = 0.0001, uncorrected), and lower FA in the IFOF, cingulum, and forceps major (all p < 0.01, all uncorrected). There was no significant difference in rCBF between the early and the advanced stage. In patients with an advanced stage, PCA involvement was correlated with a significantly lower Full Scale IQ (p = 0.036), cingulate volume (p < 0.01, uncorrected), and FA of the left SLF (p = 0.0002, uncorrected) compared to those with an intact PCA. The rCBF was positively correlated with FA of the SLF, IFOF, and forceps major (r > 0.34, p < 0.05). Global gray matter volumes were moderately correlated with TMT part A (r = 0.40, p = 0.003). FA values in the left SLF were moderately associated with processing speed (r = 0.40, p = 0.002). CONCLUSIONS: Although hemodynamic compensation may mask cerebral ischemia in advanced stages of adult moyamoya disease, the disease progression is detrimental to gray and white matter microstructure as well as cognition. In particular, additional PCA involvement in advanced disease stages may impair key neural substrates such as the cingulum and SLF. Thus, combined structural MRI and DTI are potentially useful for tracking the neural integrity of key neural substrates associated with cognitive function and detecting subtle anatomical changes associated with persistent ischemia, as well as disease progression.

Lived experiences of adult patients with moyamoya disease: A qualitative case study.

AIM: To understand the daily lived experiences of adult moyamoya disease patients. METHODS: This qualitative study involved a purposive sample of 14 adult moyamoya disease patients diagnosed after 19 years or older at one university hospital in Seoul. Interviews conducted with patients included open-ended questions about the experience of living with moyamoya disease. The data were analyzed using Colaizzi's seven-step method, which derives the theme. RESULTS: Participants' experiences were divided into three themes and eight sub-themes. "Having an unexpected disease that suddenly struck my life" refers to confusion and depression due to the diagnosis of the unexpected illness; "being occasionally anxious about the illness" describes patients' uncertainty about the disease and worrying about passing the disease on to their child; and "living with the disease by going through the disease experience" refers to the process of accepting and adapting to the illness. CONCLUSIONS: The findings provide a better understanding of the life changes and lived experiences of adult patients with moyamoya disease. Nurses should consider various aspects when providing care to adult moyamoya disease patients.

Neuropsychological impairment in adults with moyamoya angiopathy: preoperative assessment and correlation to MRI and H215O PET.

Patients with moyamoya angiopathy (MMA) are known to have an increased risk of impaired executive function (dysexecutive cognitive syndrome (DCS)). Numbers of moyamoya patients with DCS vary strongly in the literature; evidence of a correlation to affected vascular territories is low. This study aims to identify cognitive impairment in adult moyamoya patients and to correlate findings with imaging results. In addition, the predictive value of individual tests for the identification of DCS was analyzed. Neuropsychological test data of 41 adult moyamoya patients was analyzed for a possible correlation with territorial hypoperfusion on H215O PET with acetazolamide (ACZ) challenge (cerebrovascular reserve-CVR) and infarction patterns observed in MRI. Each vascular territory was analyzed separately and correlated to neuropsychological test results and to the presence of DCS. In total, 41.5% of patients presented with DCS. Significant association of DCS and affection of the right middle cerebral artery (MCA) territory was seen for insufficient CVR in PET (p = 0.030) and for patients with infarctions seen in MRI (p = 0.014). Analysis of individual neuropsychological test results confirmed the main association with the right MCA territory, as well as some association with the right anterior cerebral artery (ACA) territory. Analysis of a subgroup of patients with chronic disease on MRI (presence of large post-infarction gliosis and brain atrophy in affected territories) revealed a significantly higher risk for DCS (85% affected) than non-chronic patients (21% affected) (p < 0.001). Analysis of neuropsychological test data in this moyamoya cohort reveals DCS in 41.5% of all patients. Correlation between DCS and an impairment of CVR seen in PET and/or infarctions seen in MRI was significant for the right MCA territory. Patients with chronic disease had a significantly higher risk for DCS than non-chronic patients (p < 0.001).

Neuropsychological profile associated with Moyamoya disease: A case report.

BACKGROUND: Moyamoya disease (MMD) is a rare cardiovascular condition characterized by stenosis and gradual occlusion of the internal carotid arteries near the Circle of Willis. Current research on the disease has primarily been restricted to its medical implications, without adequate appreciation for its neurocognitive and/or neuropsychiatric implications. OBJECTIVES: The current study presents the neurocognitive profile of a 31-year-old woman diagnosed with MMD, further complicated by cerebral vascular accidents (CVAs) and history of bilateral craniotomy aimed at providing maximal revascularization. METHODS: Although speech and motor disturbances experienced by Ms. Doe around the time of her craniotomy and CVA were resolved at the time of current evaluation, she reported experiencing continued difficulties in processing speed, concentration, memory, word-retrieval, and planning. The patient underwent comprehensive neuropsychological evaluation assessing multiple cognitive domains. RESULTS: Neurocognitive evaluation revealed the presence of a lateralized profile as well as impairments in simple auditory attention, processing speed, working memory, verbal learning, verbal fluency, and speeded fine-motor dexterity. CONCLUSIONS: MMD significantly impacts cognition and daily functioning in affected individuals. This is often further exacerbated by additional CVAs requiring surgical intervention. While there is a clear growth of research on MMD, limited information is available on the neurocognitive and neuropsychiatric outcomes of the disease process. Neuropsychological data from the current case study is closely examined to provide a unique example of the lateralized neuropsychological profile and deficit pattern in a historically high functioning individual diagnosed with MMD following a stroke.

Brain Structure, Connectivity, and Cognitive Changes Following Revascularization Surgery in Adult Moyamoya Disease.

BACKGROUND: The effect of the combined direct/indirect revascularization surgery in Moyamoya disease has not been evaluated sufficiently with regard to cognitive function, brain microstructure, and connectivity. OBJECTIVE: To investigate structural and functional changes following revascularization surgery in patients with moyamoya disease (MMD) through a combined analysis of brain morphology, microstructure, connectivity, and neurobehavioral data. METHODS: Neurobehavioral and neuroimaging examinations were performed in 25 adults with MMD prior to and >12 mo after revascularization surgery. Cognitive function was investigated using the Wechsler Adult Intelligence Scale-III, Trail-Making Test, Wisconsin Card Sorting Test, Continuous Performance Test, Stroop test, and Wechsler Memory Scale. We assessed white matter integrity using diffusion tensor imaging, brain morphometry using magnetization-prepared rapid gradient-echo sequences, and brain connectivity using resting-state functional magnetic resonance imaging (MRI). RESULTS: Cognitive examinations revealed significant changes in the full-scale intelligence quotient (IQ), performance IQ (PIQ), perceptual organization (PO), processing speed, and Stroop test scores after surgery (P < .05). Enlargement of the lateral ventricle, volume reductions in the corpus callosum and subcortical nuclei, and cortical thinning in the prefrontal cortex were also observed (P < .05). Fractional anisotropy in the white matter tracts, including the superior longitudinal fasciculus, increased 2 to 4 yr after surgery, relative to that observed in the presurgical state (P < .05). Resting-state brain connectivity was increased predominantly in the fronto-cerebellar circuit and was positively correlated with improvements in PIQ and PO (P < .05). CONCLUSION: Revascularization surgery may improve processing speed and attention in adult patients with MMD. Further, multimodal MRI may be useful for detecting subtle postsurgical brain structural changes, reorganization of white matter tracts, and brain connectivity alterations.