Effect of Moyamoya Disease on Neuropsychological Functioning in Adults

Karzmark, Peter; Zeifert, Penelope; Tan, Simon; Dorfman, Leslie J.; Bell-Stephens, Teresa

doi:10.1227/01.neu.0000312712.55567.e6

Cited by 26 publications

(15 citation statements)

References 17 publications

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“…The cognitive impairment in MMD patients can involve miscellaneous domains such as executive function, orientation, comprehension, calculation, and memory. 21,23 In line with existing reports, in this study, the proportion of mild, moderate, and severe cognitive impairment was 15.1%, 9.1%, and 4.5%, respectively. Further examination of detailed cognitive scales revealed notable correlations between specific cognitive domains and lobular perfusion.…”

Section: Brain Perfusion Cognition and Ad-like Pathological Burdensupporting

confidence: 90%

“…According to our results, CBF in newly diagnosed MMD is significantly lower than normal, both globally and regionally. Previous reports revealed that cognitive decline could occur in 30% to 40% of MMD patients without presentation of cerebral infarction and hemorrhage 21 . Notably, unlike typical vascular dementia (VaD) caused by ischemic or hemorrhagic stroke, 22 subjects in our study are with normal‐appearing MR images 23,24 .…”

Section: Discussionmentioning

confidence: 60%

“…Previous reports revealed that cognitive decline could occur in 30% to 40% of MMD patients without presentation of cerebral infarction and hemorrhage. 21 Notably, unlike typical vascular dementia (VaD) caused by ischemic or hemorrhagic stroke, 22 subjects in our study are with normal-appearing MR images. 23,24 As previously mentioned, MMD patients are mostly young to middle-aged, and have relatively independent etiology for CBH, which highlights MMD as an alternative model for narrowing the gap between CBH and AD pathogenesis, especially for mimicking the preclinical period of AD.…”

Section: Brain Perfusion Cognition and Ad-like Pathological Burdenmentioning

confidence: 80%

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Brain perfusion, cognition, and plasma Alzheimer's biomarkers in moyamoya disease

Zou

Liao

Jiang

et al. 2023

Alzheimer's & Dementia

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INTRODUCTIONBecause growing interest has been focusing on cerebral blood flow (CBF) to predict, prevent, and treat Alzheimer's disease (AD), it is important to clarify the role of CBF in AD pathology and cognitive decline.METHODSIn a moyamoya disease (MMD) cohort, we examined CBF, specific cognitive domains, and plasma AD biomarkers, as well as correlations among these variables.RESULTSCBF was significantly reduced in newly diagnosed MMD patients, while plasma phosphorylated tau181 was elevated and positively correlated with hypoperfusion accumulation. MMD patients scored significantly lower than controls in multiple cognitive tests. Revascularization increased CBF to the recipient brain territories as well as cognitive performance but produced no significant change in AD biomarker levels.DISCUSSIONThese data suggest a link between accumulated reductions in CBF and cognitive decline, as well as a possible role of AD‐like pathological burden. Further studies in MMD will provide opportunities to explore new treatment strategies.

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Section: Brain Perfusion Cognition and Ad-like Pathological Burdensupporting

confidence: 90%

Section: Discussionmentioning

confidence: 60%

Section: Brain Perfusion Cognition and Ad-like Pathological Burdenmentioning

confidence: 80%

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Brain perfusion, cognition, and plasma Alzheimer's biomarkers in moyamoya disease

Zou

Liao

Jiang

et al. 2023

Alzheimer's & Dementia

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“…This is partly attributable to the clinical heterogeneity of paediatric moyamoya. For example, demographic and pre-existing clinical factors such as age of onset, laterality, history of stroke or TIA, moyamoya comorbidity, or surgical treatment are likely to influence the cognitive outcomes [7,10,[13][14][15][16][17]. Children with NF1 and SCD have recognized complex and wide-ranged cognitive profiles that include normal function, visuo-spatial deficits and executive dysfunction [18][19][20] in the absence of a diagnosis of moyamoya.…”

Section: Introductionmentioning

confidence: 99%

Fronto-Parietal and White Matter Haemodynamics Predict Cognitive Outcome in Children with Moyamoya Independent of Stroke

Choi

Westmacott

Kirkham

et al. 2022

Transl. Stroke Res.

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Moyamoya disease is a major arteriopathy characterized by progressive steno-occlusion of the arteries of the circle of Willis. Studies in adults with moyamoya suggest an association between abnormal fronto-parietal and white matter regional haemodynamics and cognitive impairments, even in the absence of focal infarction.However, these associations have not been investigated in children with moyamoya. We examined the relationship between regional haemodynamics and ratings of intellectual ability and executive function, using hypercapnic challenge blood oxygen level-dependent magnetic resonance imaging of cerebrovascular reactivity in a consecutive cohort of children with confirmed moyamoya. Thirty children were included in the final analysis (mean age: 12.55 ± 3.03 years, 17 females, 15 idiopathic moyamoya and 15 syndromic moyamoya).Frontal haemodynamics were abnormal in all regardless of stroke history and comorbidity, but occipital lobe haemodynamics were also abnormal in children with syndromic moyamoya. Executive function deficits were noted in both idiopathic and syndromic moyamoya, whereas intellectual ability was impaired in syndromic moyamoya, even in the absence of stroke. Analysis of the relative effect of regional abnormal haemodynamics on cognitive outcomes demonstrated that executive dysfunction was predominantly explained by right parietal and white matter haemodynamics independent of stroke and comorbidity, while posterior circulation haemodynamics predicted intellectual ability. These results suggest that parietal and posterior haemodynamics play a compensatory role in overcoming frontal vulnerability and cognitive impairment.

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“…Recent studies have demonstrated that patients with MMD may also present with mild neurocognitive dysfunction that can affect patients' quality of life. 2-4 Frontal lobe revascularizations can improve the dysfunction, suggesting local frontal lobe ischemia is involved in the pathophysiology. 5-9 However, the association of MMD-related frontal hypoperfusion with neurocognitive dysfunction has not been well-studied.…”

mentioning

confidence: 99%

Association Between Frontal Lobe Hemodynamics and Neurocognitive Dysfunction in Adults With Moyamoya Disease: Retrospective Cohort Analysis

et al. 2022

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BACKGROUND:Adult patients with moyamoya disease (MMD) may present with mild cognitive dysfunction, even those without evidence of conspicuous brain parenchymal damage. This cognitive dysfunction might be caused by local frontal lobe ischemia.OBJECTIVE:To explore the relationship between frontal lobe hemodynamic insufficiency and cognitive dysfunction in patients with MMD.METHODS:Thirty adult patients with MMD without conspicuous brain parenchymal damage were retrospectively examined. Patients with MMD with frontal lobe intracerebral steal phenomenon on single photon emission computed tomography were defined as group S (n = 13) and those without it were defined as group P (n = 17). A comparative group comprising patients with unruptured intracranial aneurysm was defined as group C (n = 30). The results of various cognitive and intelligence tests and a composite cognitive score were compared between groups.RESULTS:The digit span test forward version (P = .041), frontal assessment battery (P = .022), and composite cognitive score (P = .015) z-scores were significantly lower in group S than group C. Adjusting for sex and age, patients in group S had a significantly lower composite cognitive score compared with those in group C in multiple regression analysis (P = .037). Executive dysfunction and working memory dysfunction may be involved in the cognitive decline observed in group S.CONCLUSION:Mild cognitive dysfunction in MMD was associated with frontal lobe hemodynamic insufficiency. Future studies should examine whether revascularization can improve cerebral hypoperfusion and neurocognitive function in these patients.

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Effect of Moyamoya Disease on Neuropsychological Functioning in Adults

Cited by 26 publications

References 17 publications

Brain perfusion, cognition, and plasma Alzheimer's biomarkers in moyamoya disease

Brain perfusion, cognition, and plasma Alzheimer's biomarkers in moyamoya disease

Fronto-Parietal and White Matter Haemodynamics Predict Cognitive Outcome in Children with Moyamoya Independent of Stroke

Association Between Frontal Lobe Hemodynamics and Neurocognitive Dysfunction in Adults With Moyamoya Disease: Retrospective Cohort Analysis

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