Progression in Moyamoya Disease: Clinical Features, Neuroimaging
Evaluation, and Treatment

Zhang, Xin; Xiao, Weiping; Zhang, Qing; Xia, Ding; Gao, Peng; Su, Jiabin; Yang, Heng; Gao, Xinjie; Ni, Wei; Yu, Lei; Gu, Yuxiang

doi:10.2174/1570159x19666210716114016

Cited by 30 publications

(27 citation statements)

References 206 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…With the development and application of radiological techniques, more and more MMD patients are being identified (27). The incidence of progression in MMD patients was reported differently in these included literatures, varying from 8.2 to 58.8%.…”

Section: Discussionmentioning

confidence: 99%

Potential predictors for progression of moyamoya disease: A systematic review and meta-analysis

Cao

Xing²,

Dai³

et al. 2023

Front. Neurol.

View full text Add to dashboard Cite

BackgroundThe progress of Moyamoya disease (MMD) is often accompanied by the occurrence of new ischemia or hemorrhagic events, which was difficult to predict. This systematic review and meta-analysis aimed to identify predictors for progression in MMD patients.MethodsWe searched PubMed, Web of Science, Cochrane Library, and Embase databases up to December 10th, 2022 for randomized controlled trials, case-control studies, or cohort studies reporting predictors of disease progression in MMD patients. The results of each predictor were pooled by meta-analysis and further analyzed by subgroup analysis for predictors of unilateral to bilateral progression of MMD.ResultsA total of 842 patients from 12 studies were included. The estimated pooled means indicated lower age (standard mean difference [SMD]: −0.29, 95% confidence interval [CI]: −0.55 to −0.03; P = 0.03), family history (odds ratio [OR] 3.97, 95% CI: 1.96 to 8.03; P < 0.001) and contralateral abnormality (OR 3.95, 95% CI: 1.10 to 14.20; P = 0.04) were associated with progression in MMD patients. Subgroup analyses indicated that the same three factors were associated with the progression of unilateral to bilateral MMD.ConclusionsThis meta-analysis revealed that lower age, family history and contralateral abnormality were associated with progression in MMD patients. The same three factors are associated with the progression of unilateral to bilateral MMD. Further studies are needed to validate our results.

show abstract

Section: Discussionmentioning

confidence: 99%

Potential predictors for progression of moyamoya disease: A systematic review and meta-analysis

Cao

Xing²,

Dai³

et al. 2023

Front. Neurol.

View full text Add to dashboard Cite

show abstract

“…On the other hand, a 2023 paper comparing patients with MMD with patients with atherosclerosis-associated moyamoya vasculopathy (AS-MMV), reports that over a 4-6-year follow-up period, patients with MMD were more likely to present cerebrovascular events than patients with AS-MMV [31]. The pathophysiology of both MMD and MMS is complex and includes various genetic, inflammatory, and other environmental components [32][33][34]. Currie et al in a 2011 paper indicates how specific comorbidities can lead to vascular lumen narrowing in the MMS, e.g., in sickle cell disease, deoxygenated, deformed erythrocytes, containing hemoglobin S, polymerize and show abnormally high adherence to the vessel walls causing vessel lumen narrowing.…”

Section: Discussionmentioning

confidence: 99%

Ischemic stroke and hypertension in a child — a case report of two patients

Koziej,

Skoczyński,

Pac

et al. 2023

Arterial Hypertension

View full text Add to dashboard Cite

Ischemic stroke is a rare condition in children and is typically a complication of systemic diseases, such as congenital or acquired arterial diseases. The article discusses cases of two patients: a 2.5-year-old girl and a 3.5-year-old boy with ischemic stroke, severe hypertension and with multiple medium-caliber arterial stenosis involving the intracranial segments of the carotid arteries and renal arteries stenoses. Medical imaging showed a developed collateral circulation in the central nervous system typical of moyamoya disease. Molecular confirmation of the RNF213 gene variant was obtained in one patient. Complex drug treatment managed to achieve normotension. In further observation, the development of stenosis in medium-caliber arteries, including the pulmonary vascular bed, was observed. Presented cases show the evolution of vascular alterations in a patient with molecularly confirmed moyamoya disease and in a patient with a similar clinical phenotype without molecular confirmation.

show abstract

“…7 However, these methods lack information on parenchymal impairment and are increasingly complemented with parenchymal measures of cerebrovascular reserve capacity and microvascular vasodilatory kinetics. Furthermore, while surgical revascularization is the most effective treatment to stabilize or improve cerebrovascular hemodynamics in MMD and secondary moyamoya syndrome (MMS), 8 treatment response is variable, emphasizing a need for response efficacy biomarkers. Common anatomical imaging methods such as diffusion-weighted imaging (DWI) and FLuid Attenuated Inversion Recovery (FLAIR) MRI demonstrate changes only after irreversible tissue damage, and as such prognostic functional biomarkers are warranted.…”

Section: Introductionmentioning

confidence: 99%

Cerebrovascular reactivity dispersion as a new biomarker of recent stroke symptomatology in moyamoya

Han,

Richerson,

Garza

et al. 2024

Preprint

View full text Add to dashboard Cite

Background: Moyamoya disease (MMD) is a non-atherosclerotic intracranial steno-occlusive condition placing patients at high risk for ischemic stroke. Direct and indirect surgical revascularization can improve blood flow in MMD; however, randomized trials demonstrating efficacy have not been performed and biomarkers of parenchymal hemodynamic impairment are needed to triage patients for interventions and evaluate post-surgical efficacy. We test the hypothesis that hypercapnia-induced maximum cerebrovascular reactivity (CVRMAX) and the more novel indicator cerebrovascular reactivity (CVR) response time (CVRDELAY), both assessed from time-regression analyses of non-invasive hypercapnic imaging, correlate with recent focal ischemic symptoms. Methods: Hypercapnic reactivity medical resonance imaging (blood oxygenation level-dependent; echo time=35ms; spatial resolution=3.5x3.5x3.5mm) and catheter angiography assessments of cortical reserve capacity and vascular patency, respectively, in MMD participants (n=73) were performed in sequence. Time regression analyses were applied to quantify CVRMAX and CVRDELAY. Symptomatology information for each hemisphere (n=109) was categorized into symptomatic (ischemic symptoms within six months) or asymptomatic (no history of ischemic symptoms) and logistic regression analysis assessed the association of CVR metrics with ischemic symptoms after controlling for age and sex. Results: Symptomatic hemispheres displayed lengthened CVRDELAY (p<0.001), which was more discriminatory between hemispheres than CVRMAX (p=0.037). CVRDELAY (p<0.001), but not CVRMAX (p=0.127), was found to be sensitively related to age in asymptomatic tissue (0.33-unit increase/year); age-dependent normative ranges are presented to enable quantitative assessment of patient-specific impairment. Furthermore, the area under the receiver operating characteristic curves shows that CVRDELAY predicts ischemic symptoms (p<0.001), whereas CVRMAX does not (p=0.056). Conclusion: Findings support that CVR metrics are uniquely altered in hemispheres with recent ischemic symptoms, motivating the investigation of CVR as a surrogate of ischemic symptomatology and treatment efficacy.

show abstract

Progression in Moyamoya Disease: Clinical Features, Neuroimaging Evaluation, and Treatment

Cited by 30 publications

References 206 publications

Potential predictors for progression of moyamoya disease: A systematic review and meta-analysis

Potential predictors for progression of moyamoya disease: A systematic review and meta-analysis

Ischemic stroke and hypertension in a child — a case report of two patients

Cerebrovascular reactivity dispersion as a new biomarker of recent stroke symptomatology in moyamoya

Contact Info

Product

Resources

About