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The importance of the posterior cerebral artery (PCA) involvement in moyamoya disease has been highlighted in recent years. However, few studies compared the impact of PCA lesions in moyamoya disease between pediatric and adult patients. We conducted this study to summarize the clinical features of moyamoya patients with PCA lesions and describe the difference between pediatric and adult patients. We reviewed the records of 696 consecutive moyamoya vasculopathy patients from 2009 to 2015. The Suzuki and the Miyamoto stages were used to evaluate the steno-occlusive lesions of the anterior and posterior arteries. Clinical and radiographic features were compared between those with and without PCA involvement, also between pediatric and adult patients. A total of 574 angiograms (140 pediatrics and 434 adults) were reviewed. The prevalence of PCA steno-occlusion did not differ significantly between pediatric patients and adult patients (35.0% vs. 30.4%, P = 0.347). Pediatric patients had more advanced PCA stages compared to adult patients (P = 0.045). There was a significant correlation of the PCA angiographic stages with the ipsilateral internal carotid artery (ICA) stages, both in pediatrics and in adults (both P < 0.001). The frequency of ipsilateral cerebral infarction positively correlated with the advancement of PCA stages in adult patients (P < 0.001), but not significant in pediatric patients (P = 0.106). Pediatric patients tend to have more advanced PCA lesions than adult patients. The degree of PCA steno-occlusion positively correlates with the ipsilateral ICA stage, both in pediatrics and in adults.

Background and Purpose: Moyamoya disease (MMD) is a chronic cerebrovascular disorder with little known etiology. We aim to propose a new classification system for MMD from the perspective of embryology., Methods: MMD patients' digital subtraction angiograms were retrospectively analyzed. Every angiogram was analyzed to find the abnormal vessels and from which part of the posterior cerebral artery (PCA) the lesions begin., Results: In 262 MMD cases, 32 pediatric patients had PCA involvement, of which 17 were male and 15 were female; 68 adults had PCA involvement, of which 33 were male and 35 were female. The initially affected part of the PCA was compared between sexes and between pediatric and adult patients, and the findings are not statistically significant (P = 0.233, P = 0.855, P = 0.343, respectively). However, of the 100 cases with PCA involvement, only 4 had the lesions begin from the first part of the PCA, and all of the 4 cases had the basilar artery lesions. All the other 96 cases had the lesions begin from the second part of the PCA or from the posterior communication artery, which is derived from the caudal ramus of the primitive intracarotid artery, leaving the first part of the PCA and basilar artery excluded from affection., Conclusion: MMD should be classified into primitive intracarotid artery system-involved type and primitive vertebral basilar artery system-involved type. The reason that the vertebral basilar artery is so rarely involved in MMD might be because of its late development in the brain., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Moyamoya disease (MMD) is a chronic and progressive cerebrovascular stenosis or occlusive disease that occurs near Willis blood vessels. The aim of this study was to investigate the mutation of DIAPH1 in Asian population, and to compare the angiographic features of MMD patients with and without the mutation of the DIAPH1 gene. Blood samples of 50 patients with MMD were collected, and DIAPH1 gene mutation was detected. The angiographic involvement of the posterior cerebral artery was compared between the mutant group and the non-mutant group. The independent risk factors of posterior cerebral artery involvement were determined by multivariate logistic regression analysis. DIAPH1 gene mutation was detected in 9 (18%) of 50 patients, including 7 synonymous mutations and 2 missense mutations. However, the incidence of posterior cerebral artery involvement in mutation positive group was very higher than that in mutation negative group (77.8% versus 12%; p = 0.001). There is an association between DIAPH1 mutation and PCA involvement (odds ratio 29.483, 95% confidence interval 3.920–221.736; p = 0.001). DIAPH1 gene mutation is not a major genetic risk gene for Asian patients with moyamoya disease but may play an important role in the involvement of posterior cerebral artery. [ABSTRACT FROM AUTHOR]

Background: Ischemic events are the most common postoperative complication in bypass surgery for moyamoya disease (MMD), but the risk factors for pediatric MMD remain unclear. The goal of the study was to investigate the risk factors for postoperative ischemic complications in pediatric MMD patients.Methods: We retrospectively reviewed a consecutive series of pediatric MMD cases at Beijing Tiantan Hospital, Capital Medical University from June 2010 through June 2019. Preoperative clinical variables and radiographic findings were recorded, and logistic regression analysis was carried out to identify the risk factors for postoperative ischemic events.Results: A total of 533 operations in 336 patients were included in this study. Postoperative complications occurred after 51 operations (9.6%), including 40/447 indirect bypass procedures, 9/70 direct bypass procedures, and 2/16 combined bypass procedures. Postoperative ischemic events were the most common complication and occurred in 30 patients after 31 procedures (8.9% per patient; 5.8% per operation), including 26/447 indirect bypass procedures, 4/70 direct bypass procedures, and 1/16 combined bypass procedures, and the incidence of these events did not differ significantly between indirect and non-indirect bypass (5.8% vs 5.8%; p = 0.999). Multivariate logistic regression analyses revealed that older age at operation (OR 1.129, 95% CI 1.011-1.260, p = 0.032) and posterior cerebral artery involvement (OR 2.587, 95% CI 1.030-6.496, p = 0.043) were significantly associated with postoperative ischemic events.Conclusion: We speculate that older age at operation and posterior cerebral artery involvement are risk factors for postoperative ischemic events in pediatric MMD patients. [ABSTRACT FROM AUTHOR]

Objective: To estimate the association between p. R4810K variant and clinical outcomes of patients with moyamoya disease (MMD). Methods: The p.R4810K genetic variant was genotyped among 498 Chinese patients with MMD conducted from June 1, 2012, to June 31, 2017. Data was obtained by retrospective chart review, follow-up information and outcome were obtained through clinical visits and telephone. Results: Among 498 patients, 361 (72.5%) were wild-type patients (G/G), 133 (26.7%) heterozygous patients (G/A), and 4 (0.8%) homozygotes (A/A). Compared with GG group, the patients in the G/A+A/A group were younger at diagnosis and had more familial cases, more transient ischemic attack cases, more posterior cerebral artery involved hemispheres, less unilateral lesions. After the median 53 months follow-up, strokes occurred in 9 patients in the G/A+A/A group and in 52 in the G/G group. Multivariate Cox regression analysis showed that the history of hypertension (HR, 2.294; 95% CI, 1.251–4.206; p = 0.007), the presence of TIA (HR, 0.319; 95% CI, 0.120–0.846; p = 0.022), and the Suzuki stage (HR, 1.510; 95% CI, 1.129–2.018; p = 0.005) were associated with recurrent stroke. The p.R4810K (HR, 0.601; 95% CI, 0.292–1.239; p = 0.168) was not associated with recurrent stroke. Multivariate logistic regression analysis showed that recurrent stroke (OR, 5.997; 95% CI, 2.583–13.924; p = 0.000) was the only factor associated with unfavorable neurological status. And the p.R4810K (OR, 0.885; 95% CI, 0.482–1.627; p = 0.695) was not associated with neurological status. Conclusions: Compared to the patients in G/G group, patients in G/A+A/A group exhibited different clinical features, and had a lower rate of recurrent stroke and better clinical outcome after early medical and surgical interventions. Multivariate COX and logistic regression analysis showed that p.R4810Kvariant was not related to either recurrent stroke or neurological status. The p.R4810Kvariant may not be associated with long-term clinical outcome in Chinese patients with MMD. [ABSTRACT FROM AUTHOR]

Objective To elucidate the clinical features, risk factors for contralateral progression, and long-term outcomes of patients with unilateral moyamoya disease (MMD). Methods We retrospectively reviewed 109 patients with unilateral MMD treated at Beijing Tiantan Hospital. Clinical features, radiologic findings, and outcomes were analyzed. Results The mean age at diagnosis was 30.8 ± 14.2 years with a one-peak distribution in the thirties. The ratio of women to men was 1.1:1. Familial occurrence was 5.5%. The primary symptoms at initial presentation were transient ischemic attack (39.4%), infarction (22%), and hemorrhage (26.6%). The distribution of the initial Suzuki stage was as follows: stage 2, n = 33; stage 3, n = 35; stage 4, n = 27; stage 5, n = 11, and stage 6, n = 3. Contralateral abnormality on initial angiography occurred in 19 adult patients (17.4%). Posterior cerebral artery involvement was observed in 28 (25.7%) patients. During the average follow-up of 43.8 ± 21.3 months, contralateral progression was observed in 18 patients (16.5%). Contralateral abnormalities ( P = 0.033) on initial angiography and infarction at initial presentation ( P = 0.041) were significantly associated with contralateral progression in adults and children, respectively. Approximately 91.7% of patients had a modified Rankin Scale score of 0–2 during follow-up. Conclusions A one-peak pattern in age distribution, lower grade of Suzuki stage, fewer familial cases, and posterior cerebral artery involvement were observed in patients with unilateral MMD. Contralateral abnormalities on initial angiography and infarction at onset were risk factors for contralateral progression in adult and pediatric patients, respectively. Good functional outcomes can be anticipated in the vast majority of surgically treated patients. [ABSTRACT FROM AUTHOR]

Moyamoya disease (MMD) is a cerebrovascular disorder, currently defined as progressive stenosis of intracranial internal carotid artery and its main branches with secondary formation of netlike vessels. Its precise formation mechanism, however, is unknown. We propose that MMD is caused by abnormal embryonic development of the cerebral arteries and also propose a mechanism for MMD formation. The anterior, middle, and posterior cerebral arteries, anterior choroidal arteries (AChoA) and posterior choroidal arteries, and posterior corpus callosum arteries were analyzed separately for each patient with MMD to determine whether the arteries exhibited the following characteristics, which we regarded as remnants of primitive vessels: 1) plexiform arteries rather than normal artery trunks; 2) clustered arteries converging at locations of normal artery trunks; and 3) dilated AChoA. We retrospectively analyzed 39 consecutive patients with MMD, of whom 30 had anterior cerebral arteries, 31 middle cerebral arteries, 10 posterior cerebral arteries, 30 AChoAs, 18 posterior choroidal arteries, and 20 posterior corpus callosum arteries and had the characteristics of primitive vessel remnants. Altogether, 82.05% of the patients had the remnants of primitive vessels. MMD is more likely to be caused by abnormalities of cerebral artery development, which mainly occur in the embryonic period or postnatally. The abnormality developing processes include sprouting angiogenesis, vessel fusion, and pruning, which primarily affect cranial ramus of primitive internal carotid artery, may occasionally affect the caudal ramus of the primitive internal carotid artery, and rarely affect the vertebrobasilar artery system. So-called moyamoya vessels comprise unfused primitive small vessels; to compensate, enlarged AChoAs remain undegenerated but are not dilated. [ABSTRACT FROM AUTHOR]

Recurrent stroke after surgical revascularization is still a big issue for moyamoya disease (MMD). This study aims to identify predictors for postoperative stroke and unfavorable outcome in ischemic-type MMD. We identified a consecutive series of patients with ischemic-type MMD who underwent revascularization between January 2005 and December 2012. Predictors for postoperative stroke and functional outcomes were assessed with logistic and Cox regression analysis. A total of 346 patients underwent 437 revascularization procedures and the mean follow-up period was 4.0 years. The incidence of perioperative stroke was 6.9%. Being adult at onset (odds ratio [OR], 5.033; 95% confidence interval [CI], 1.447–17.506; P = 0.011) and posterior cerebral artery (PCA) stenosis (OR, 3.364; 95% CI, 1.588–7.265; P = 0.002) before surgery were predictors of perioperative stroke. The annual subsequent stroke rate beyond 30 days after surgery was 1.2%. Subsequent stroke events tended to occur throughout the first 5 years after surgery in adults, whereas in children they mainly occurred within the first 2 years after surgery. Age at onset (OR, 1.025; 95% CI, 1.003–1.048; P = 0.023), ischemic stroke or transient ischemic attack at presentation (OR, 2.703; 95% CI, 1.062–6.875; P = 0.037), and PCA involvement (OR, 2.664; 95% CI, 1.462–4.854; P = 0.001) were associated with higher risk of overall postoperative stroke. PCA involvement (OR, 2.62; 95% CI, 1.33–5.15; P = 0.005), internal carotid artery supraclinoid segment occlusion (OR, 2.76; 95% CI, 1.27–6.03; P = 0.011), and older age at onset (OR, 1.03; 95% CI, 1.01–1.05; P = 0.033) were predictive of unfavorable outcome. Patients with ischemic-type MMD at an older age and more severe angiopathy might be at higher risk of recurrent stroke and unfavorable outcome after revascularization. [ABSTRACT FROM AUTHOR]