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Background: Evidence on the natural angiographic course of moyamoya disease (MMD) is lacking. It takes about 6 months for waiting for revascularisation surgery. The issue of when to perform subtraction angiography (DSA) for follow-up remains unclear. We investigated the natural course of MMD by DSA and attempted to determine the best interval to perform the follow-up DSA., Methods: This is a single-centre cohort study of Chinese MMD inpatients treated from 1 January 2015 to 31 August 2019. Their angiographic findings were evaluated on Suzuki stage and collateral circulation between two follow-ups of the same hemisphere., Results: A total of 110 patients who met the criteria were enrolled in this study. After a median 6 months follow-up, five patients (4.5%) had progression, four females and one male. Time interval of progression ranged from 4 to 137 months with a mean of 61.4 months. Of five patients with progression, four had unilateral lesion (two ipsilateral and two contralateral) and one had bilateral lesions. Collateral circulation was changed in three of five patients., Conclusions: The angiographic evidence of progression in MMD was rare in the short-term follow-up, and most patients with progression had initial unilateral involvement. DSA re-examination may be not needed in patients with bilateral MMD, but needed in unilateral MMD., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2020. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Quasi-moyamoya disease (quasi-MMD) is a rare cerebrovascular disease and its clinical features and natural history remain unclear. The aim of the study is to describe the clinical characteristics and the natural histories of this disease, with analysis of the risk factors for future cerebrovascular events., Methods: We identified 64 patients with quasi-MMD from 693 moyamoya vasculopathy patients referred to our hospital between 2011 and 2015. Demographic data, associated disorders, clinical manifestation, angiographic findings, natural history, and risk factors for cerebrovascular events were analyzed., Results: Patients included in the study had a mean age of 31.5 years. A unimodal age distribution was noted. Atherosclerosis was the most frequently associated disorder. Forty-five (70.3%) patients had ischemic events as their initial clinical manifestation and 14 (21.9%) patients presented as hemorrhagic stroke. The majority of patients presented with Suzuki grades 3 and 4 (20.3% and 42.2%). The annual risk of cerebrovascular events was 19.4% per patient-year. Prior hemorrhage (HR 2.77, 95% CI 1.20-6.41) and ischemic stroke (HR 2.77, 95% CI 1.26-6.07) were 2 risk factors for future events., Conclusions: Several clinical characteristic differences were observed in our mainland China cohort compared with the Japanese and European cohorts. The annual risk of cerebrovascular events was relatively high in quasi-MMD patients. Patients with prior hemorrhage and ischemic stroke were inclined to have future cerebrovascular events. Close follow-up is needed for these patients., (Copyright © 2017 National Stroke Association. Published by Elsevier Inc. All rights reserved.)

Objective: To elucidate the clinical and angiographic features in patients with moyamoya disease (MMD) and the p.R4810K heterozygous variant and present an angiographic grading system to evaluate disease severity., Methods: We retrospectively reviewed 87 patients with MMD and the p.R4810K variant treated at Beijing Tiantan Hospital. Clinical features, stroke subtype, and angiographic characteristics were analyzed., Results: The median age at diagnosis was 25 years (range, 3-59). The ratio of women to men was 1.2:1. The familial occurrence of MMD was 14.9%. The primary symptom at diagnosis was ischemia, hemorrhage, or other in 67, 16, and 4 patients, respectively. Angiographic features correlating with ischemic stroke or stroke, including Suzuki grade, external carotid artery collaterals, leptomeningeal collaterals, and Mugikura grade, were identified. A binary logistic regression model demonstrated a significant correlation of Suzuki grade (P = 0.008) and posterior cerebral artery grade (P = 0.029) with ischemic stroke (142 hemispheres). A modified Suzuki-Mugikura grading system was developed. The areas under the receiver operating characteristic curves used to predict ischemic stroke based on the Suzuki grading, Mugikura grading, and modified Suzuki-Mugikura grading systems were 0.736, 0.69, and 0.741, respectively. Furthermore, the modified Suzuki-Mugikura grades were significantly correlated with infarction in posterior circulation and the number of infarcted regions., Conclusions: The clinical and angiographic features of a Chinese MMD population with the p.R4810K variant were similar to those of a Japanese MMD population; they might be a distinct cerebrovascular disease entity and represent a separate subgroup. A modified Suzuki-Mugikura grading system was valuable for predicting stroke and evaluating disease severity., (Copyright © 2016 Elsevier Inc. All rights reserved.)

OBJECT The authors compared the image quality and diagnostic sensitivity and specificity of 7.0-T and 3.0-T MRI and time-of-flight (TOF) MR angiography (MRA) in patients with moyamoya disease (MMD). METHODS MR images of 15 patients with ischemic-type MMD (8 males, 7 females; age 13-48 years) and 13 healthy controls (7 males, 6 females; age 19-28 years) who underwent both 7.0-T and 3.0-T MRI and MRA were studied retrospectively. The main intracranial arteries were assessed by using the modified Houkin's grading system (MRA score). Moyamoya vessels (MMVs) were evaluated by 2 grading systems: the MMV quality score and the MMV area score. Two diagnostic criteria for MMD were used: the T2 criteria, which used flow voids in the basal ganglion on T2-weighted images, and the TOF criteria, which used the high-intensity areas in the basal ganglion on source images from TOF MRA. All data were evaluated by 2 independent readers who were blinded to the strength field and presence or absence of MMD. Using conventional angiography as the gold standard, the sensitivity and specificity of 7.0-T and 3.0-T MRI/MRA in the diagnosis of MMD were calculated. The differences between 7.0-T and 3.0-T MRI and MRA were statistically compared. RESULTS No significant differences were observed between 7.0-T and 3.0-T MRA in MRA score (p = 0.317) or MRA grade (p = 0.317). There was a strong correlation between the Suzuki's stage and MRA grade in both 3.0-T (rs = 0.930; p < 0.001) and 7.0-T (rs = 0.966; p < 0.001) MRA. However, MMVs were visualized significantly better on 7.0-T than on 3.0-T MRA, suggested by both the MMV quality score (p = 0.001) and the MMV area score (p = 0.001). The correlation between the Suzuki's stage and the MMV area score was moderate in 3.0-T MRA (rs = 0.738; p = 0.002) and strong in 7.0-T MRA (rs = 0.908; p < 0.001). Moreover, 7.0-T MR images showed a greater capacity for detecting flow voids in the basal ganglion on both T2-weighted MR images (p < 0.001) and TOF source images (p < 0.001); 7.0-T MRA also revealed the subbranches of superficial temporal arteries much better. Receiver operating characteristic curve analysis showed that, according to the T2 criteria, 7.0-T MRI/MRA was more sensitive (sensitivity 1.000; specificity 0.933) than 3.0-T MRI/MRA (sensitivity 0.692; specificity 0.933) in diagnosing MMD; based on the TOF criteria, 7.0-T MRI/MRA was more sensitive (1.000 vs 0.733, respectively) and more specific (1.000 vs 0.923, respectively) than 3.0-T MRI/MRA. CONCLUSIONS Compared with 3.0-T MRI/MRA, 7.0-T MRI/MRA detected and delineated MMVs more clearly and provided higher diagnostic sensitivity and specificity, although it did not show significant improvement in depicting main intracranial arteries. The authors speculate that 7.0-T MRI/MRA is a promising technique in the diagnosis of MMD because it is noninvasive compared with conventional angiography and it is more sensitive than 3.0-T MRI/MRA.

The ring-finger protein 213 (RNF213) gene is a major susceptibility gene for moyamoya disease. The homozygote of the p.R4810K variant on RNF213 exhibits an early onset age and severe form of moyamoya disease. We report 4 unrelated pediatric moyamoya disease cases with the homozygous p.R4810K variant and the long-term surgical outcomes. Published reports on surgical outcome of moyamoya disease case with the homozygous p.R4810K variant were reviewed. Cerebral angiography revealed classic angiographic findings of moyamoya disease in 7 hemispheres of the 4 children. All patients underwent successful indirect revascularization. Abundant collateral blood flow from the external carotid arteries to the internal carotid arteries was observed in all bypass procedures by angiography. Improvements in symptoms and cerebral blood volume were observed in all patients at long-term follow-up. This report is the first case series in the literature on the surgical management of these patients. These cases highlight the effectiveness of indirect revascularization for moyamoya disease patients with the homozygous p.R4810K variant. Early diagnosis and treatment are crucial to avoid irreversible neurologic deficits in these patients. [ABSTRACT FROM AUTHOR]

Background: The aim of this study was to investigate the hemorrhgic sites and collateral vessels in hemorrhagic MMD with the p.R4810K variant.Methods: Hemorrhage sites were classified as either anterior or posterior. Collateral vessels were classified into three subtypes according to origin: lenticulostriate anastomosis, thalamic anastomosis, and choroidal anastomosis. Hemorrhage sites and collateral vessels were compared between patients with wild-type p.R4810K variant (GG) and patients with heterozygous p.R4810K variant (GA) after 1:1 propensity score matching.Results: A total of 130 hemorrhagic MMD patients were included in present study, 21 pairs (42 hemorrhagic hemispheres) were obtained after 1:1 propensity score. In GA group, 16 hemispheres (76.2%) presented anterior hemorrhage, and 5 hemispheres (23.8%) presented with posterior hemorrhage. In GG group, 13 hemispheres (61.9%) presented anterior hemorrhage, and 8 hemispheres (38.1%) presented with posterior hemorrhage. No significant differences were found in hemorrhagic sites between two matched groups (P > 0.05). Of 21 hemispheres in GA group, 10 (47.6%) exhibited lenticulostriate anastomosis, 6 (28.6%) thalamic anastomosis, and 6 (28.6%) choroidal anastomosis. Of 21 hemispheres in GG group, 3 (14.3%) exhibited lenticulostriate anastomosis, 5 (23.8%) thalamic anastomosis, and 9 (42.9%) choroidal anastomosis. There was significant difference in lenticulostriate anastomosis between two matched groups (P = 0.045). After adjustment the age, sex, and PCA involvement, we found that lenticulostriate anastomosis was associated with p.R4810K variant (OR, 5.995; 95% CI, 1.296-27.737; P = 0.022).Conclusion: Lenticulostriate anastomosis might be associated with p.R4810K variant. Whereas hemorrhagic sites, thalamic anastomosis, and choroidal anastomosis might not be associted withp.R4810K variant. [ABSTRACT FROM AUTHOR]