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Clinical status and evolution in moyamoya: which angiographic findings correlate?

ABSTRACT: Moyamoya is a progressive steno-occlusive cerebrovascular pathology of unknown aetiology that usually involves the terminal portions of the internal carotid arteries and/or the proximal portions of the anterior and middle cerebral arteries bilaterally. The pre-operative Suzuki staging system and post-operative Matsushima grade are nearly universally used markers of natural history and surgical revascularization results, respectively, but their correlation with clinical and radiographic manifestations of moyamoya has not been systematically evaluated in a large cohort. This study evaluated the strength of correlations between pre- and post-operative angiographic parameters and clinical status among paediatric patients with moyamoya. The participants included 58 patients of mean age 11?years at the time of surgery who underwent bilateral indirect revascularization in the same procedure at Boston Children's Hospital, between January 2010 and December 2015. All included patients had available pre-operative and 1-year post-operative digital subtraction angiography. Clinical data included presenting symptoms, degree of functional incapacity, and peri-operative and long-term complications. Radiographic data included pre-operative Suzuki stage, degree of arterial stenosis, a novel collateral score, the presence of hypovascular territories on digital subtraction angiography, and post-operative Matsushima grade and evolution of stenosis. Chi-squared test and Pearson coefficient were used for correlation studies for categorical variables and Spearman's rho was used for correlation studies for continuous variables. Results showed that Suzuki stage, collateral score and degree of stenosis were insufficient to predict clinical presentation, pre-operative incapacity and radiographic presentation, whereas the presence of hypovascular territories was correlated with all of these. At 1-year follow-up, Matsushima grade was insufficient for predicting peri-operative or long-term complications, nor did it correlate with post-operative incapacity. The presence of hypovascular territories at 1-year follow-up was correlated with the incidence of post-operative ischaemic symptoms.

SUBMITTER: Rosi A

PROVIDER: S-EPMC7425301 | biostudies-literature | 2019

REPOSITORIES: biostudies-literature

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Clinical status and evolution in moyamoya: which angiographic findings correlate?

Rosi Andrea A Riordan Coleman P CP Smith Edward R ER Scott R Michael RM Orbach Darren B DB

Brain communications 20191030 1

Moyamoya is a progressive steno-occlusive cerebrovascular pathology of unknown aetiology that usually involves the terminal portions of the internal carotid arteries and/or the proximal portions of the anterior and middle cerebral arteries bilaterally. The pre-operative Suzuki staging system and post-operative Matsushima grade are nearly universally used markers of natural history and surgical revascularization results, respectively, but their correlation with clinical and radiographic manifesta ...[more]

PMID: 32954269

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Project description:Objective: Extracranial-intracranial bypass is currently recognized as the optimal treatment for hemorrhagic-type moyamoya disease (MMD) which reduces incidence of rebleeding. Recent studies have reported the advantage of combined bypass over direct bypass for the general MMD patients. However, the effect of direct bypass and combined bypass surgery specifically for hemorrhagic-type MMD had not been investigated yet. Methods: Hemorrhagic-type MMD patients who underwent direct and combined bypass surgery with complete clinical and radiological documentation from a multicenter cohort between 2009 and 2017 were retrospectively included. Surgical methods included superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis (direct bypass), combined STA-MCA bypass with encephalodurosynangiosis (EDS), and combined STA-MCA bypass with encephaloduroarteriosynangiosis (EDAS). Matsushima standard on follow-up catheter angiography was used to assess surgical outcome. Modified Rankin Scale, incidence of rebleeding and ischemia during follow-up were recorded. Rebleeding-free survival rates between direct and combined bypass were compared by Kaplan-Meier analysis. Results: Sixty eight hemorrhagic-onset MMD patients were included in this study, among which 71 hemispheres were treated with surgery (direct bypass: 17; bypass+EDS: 24; bypass+EDAS: 30). Forty six (64.8%) hemispheres had satisfactory revascularization (Matsushima level 2-3) and 26 (36.6%) had poor neoangiogenesis. Matsushima level was not significantly different between surgical groups (P = 0.258). Good neoangiogenesis from dural grafts was achieved in 26 (36.6%) hemispheres, and good neoangiogenesis from STA grafts was only seen in 4 (out of 30, 12.5%) hemispheres. Multivariate analysis showed bypass patency [P < 0.001, OR (95%CI): 13.41 (3.28-54.80)] and dural neoangiogenesis [P < 0.001, OR (95%CI): 13.18 (3.26-53.36)] both independently contributed to good angiographic outcome. During follow-up, incidences of rebleeding or ischemic events, and re-bleeding free survival rate were not significantly different between surgical groups (P = 0.433, P = 0.559, and P = 0.997). However, patients who underwent combined bypass surgery had significantly lower mRS at follow-up comparing to patients who underwent direct bypass (P = 0.006). Conclusion: Combined bypass surgery and direct bypass surgery offered similar revascularization for hemorrhagic MMD. Bypass patency and dural angiogenesis both contributed to revascularization independently. The potential of indirect bypass to grow new vessels in hemorrhagic-MMD patients was generally limited, but dural leaflets offered better neoangiogenesis than STA grafts and was therefore recommended for surgical revascularization of hemorrhagic MMD.

Project description:AimPrevious studies have found significant differences in clinical characteristics between pediatric and adult moyamoya disease (MMD) patients, but few studies have focused on the factors underlying these differences. We aimed to investigate the differences in leptomeningeal collateral (LMC) status between pediatric and adult MMD patients and to analyze the effects of LMCs on clinical characteristics and therapeutic prognosis.MethodsWe retrospectively analyzed 214 MMD patients from January 2014 to January 2016. Clinical characteristics and LMC status were compared between the pediatric and adult patients. LMC status was graded as good or poor depending on the retrograde flow from the posterior cerebral artery (PCA) on digital subtraction angiography (DSA).ResultsA total of 83 pediatric and 131 adult (1:1.6) MMD patients were analyzed. Pediatric patients were more likely to experience a transient ischemic attack (81%), whereas adult patients were more likely to experience infarction (51%). Regarding the different MMD stages (the early, medium, and advanced stages corresponded to Suzuki stages 1-2, 3-4, and 5-6, respectively), the prevalence of good LMC status was higher for pediatric patients than for adult patients in the early stage (P = 0.047) and the medium stage (P = 0.001), but there were no differences between these patient groups in the advanced stage (P = 0.547). Worse postoperative angiographic outcomes (P = 0.017) were found in adult patients than in pediatric patients in the medium stage. Poor LMC status had strong correlations with infarction (P < 0.001 and P = 0.017) and poor postoperative outcomes (P = 0.003 and P = 0.043) in both pediatric and adult patients.ConclusionsPediatric MMD patients have greater patency and a greater ability to establish good LMC status than adult patients, and poor LMC status has a strong correlation with severe clinical symptoms and poor postoperative outcomes. LMC status may be an important factor in the differences in clinical characteristics and prognosis between pediatric and adult MMD patients.

Project description:BACKGROUND AND PURPOSE:Moyamoya disease is a chronic neurovascular steno-occlusive disease of the internal carotid artery and its main branches, associated with the development of compensatory vascular collaterals. Literature is lacking about the precise description of these compensatory vascular systems. Usually, the posterior circulation is less affected, and its vascular flow could compensate the hypoperfusion of the ICA territories. The aim of this study was to describe these natural connections between the posterior cerebral artery and the anterior cerebral artery necessary to compensate the lack of perfusion of the anterior cerebral artery territories in the Moyamoya population. MATERIALS AND METHODS:All patients treated for Moyamoya disease from 2004 to 2018 in 4 neurosurgical centers with available cerebral digital subtraction angiography were included. Forty patients (80 hemispheres) with the diagnosis of Moyamoya disease were evaluated. The presence of anastomoses between the posterior cerebral artery and the anterior cerebral artery was found in 31 hemispheres (38.7%). RESULTS:Among these 31 hemispheres presenting with posterior cerebral artery-anterior cerebral artery anastomoses, the most frequently encountered collaterals were branches from the posterior callosal artery (20%) and the posterior choroidal arteries (20%). Another possible connection found was pio-pial anastomosis between cortical branches of the posterior cerebral artery and the anterior cerebral artery (15%). We also proposed a 4-grade classification based on the competence of these anastomoses to supply retrogradely the territories of the anterior cerebral artery. CONCLUSIONS:We found 3 different types of anastomoses between the anterior and posterior circulations, with different abilities to compensate the anterior circulation. Their development depends on the perfusion needs of the territories of the anterior cerebral artery and can provide the retrograde refilling of the anterior cerebral artery branches.

Project description:Background: APOE is the largest genetic risk factor for sporadic Alzheimer's disease (AD), but there is a substantial polygenic component as well. Polygenic risk scores (PRS) can summarize small effects across the genome but may obscure differential risk associated with different molecular processes and pathways. Variability at the genetic level may contribute to the extensive phenotypic heterogeneity of Alzheimer's disease (AD). Here, we examine polygenic risk impacting specific pathways associated with AD and examined its relationship with clinical status and AD biomarkers of amyloid, tau, and neurodegeneration (A/T/N). Methods: A total of 1,411 participants from the Alzheimer's Disease Neuroimaging Initiative (ADNI) with genotyping data were included. Sets of variants identified from a pathway analysis of AD GWAS summary statistics were combined into clusters based on their assigned pathway. We constructed pathway-specific PRSs for each participant and tested their associations with diagnostic status (AD vs cognitively normal), abnormal levels of amyloid and ptau (positive vs negative), and hippocampal volume. The APOE region was excluded from all PRSs, and analyses controlled for APOE -ε4 carrier status. Results: Thirteen pathway clusters were identified relating to categories such as immune response, amyloid precursor processing, protein localization, lipid transport and binding, tyrosine kinase, and endocytosis. Eight pathway-specific PRSs were significantly associated with AD dementia diagnosis. Amyloid-positivity was associated with endocytosis and fibril formation, response misfolded protein, and regulation protein tyrosine PRSs. Ptau positivity and hippocampal volume were both related to protein localization and mitophagy PRS, and ptau positivity was additionally associated with an immune signaling PRS. A global AD PRS showed stronger associations with diagnosis and all biomarkers compared to pathway PRSs, suggesting a strong synergistic effect of all loci contributing to the global AD PRS. Conclusions: Pathway PRS may contribute to understanding separable disease processes, but do not appear to add significant power for predictive purposes. These findings demonstrate that, although genetic risk for AD is widely distributed, AD-phenotypes may be preferentially associated with risk in specific pathways. Defining genetic risk along multiple dimensions at the individual level may help clarify the etiological heterogeneity in AD.

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Clinical status and evolution in moyamoya: which angiographic findings correlate?

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Clinical status and evolution in moyamoya: which angiographic findings correlate?

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