BACKGROUND: Recent advances are in the genetics, diagnosis, pathophysiology, and management of moyamoya disease (MMD), and moyamoya syndrome (MMS), a term used to describe moyamoya-like vasculopathy associated with various systemic diseases or conditions.
CONCLUSIONS: Ring finger protein (RNF213) has been reported to be a susceptibility gene not only for MMD but also for atherosclerotic intracranial arterial stenosis and ischemic stroke attributable to large artery atherosclerosis. The latest guidelines by the Research Committee on MMD of the Japanese Ministry of Health, Labor, and Welfare, removed limitations of the previous definition that required bilateral involvement of the intracranial carotid artery to make the diagnosis, given the increasing evidence of progression to bilateral involvement in unilateral MMD. 3-dimensional constructive interference in steady-state MRI is useful for the differential diagnosis of MMD from atherosclerosis. Recent advances in the pathophysiology of MMD suggest that genetic and environmental factors play important roles in vascular angiogenesis and remodeling via complex mechanisms. The latest Japanese Guidelines and American Scientific Statement described that antiplatelet therapy can be considered reasonable. Endovascular interventional stent placement fails to prevent ischemic events and does not halt MMD progression. In the Japan Adult Moyamoya trial, a randomized controlled trial for bilateral extracranial-intracranial direct bypass versus conservative therapy in patients with MMD, who had intracranial hemorrhage, recurrent bleeding, completed stroke, or crescendo transient ischemic attack was significantly fewer with direct bypass than with conservative care.
CONCLUSIONS: This review presents updated information on genetics, diagnosis, pathophysiology, and treatment of adult MMD and MMS. Despite recent advances, many mysteries still exist in the etiologies of moyamoya vasculopathy. The diagnostic criteria and treatment guidelines have been updated but not yet been globally established. Ongoing and future studies investigating underlying pathophysiological mechanisms of MMD and MMS may clarify potentially effective medical, surgical, or endovascular treatments.

BACKGROUND: Intracranial arterial narrowing is a significant factor leading to brief episodes of reduced blood flow to the brain, known as transient ischemic attacks, or full-blown strokes. While atherosclerosis is commonly associated with intracranial arterial narrowing, it is frequently of a non-atherosclerotic nature in younger patients.
METHODS: Here, we present the case of a young stroke patient with narrowing of the middle cerebral artery (MCA), characterized as non-atherosclerotic lesions, who experienced an ischemic stroke despite receiving standard drug therapy. The patient underwent digital subtraction angiography (DSA) to assess the entire network of blood vessels in the brain, revealing significant narrowing (approximately 80%) in the M1 segment of the right MCA. Subsequently, the patient underwent Drug-Coated Balloon Angioplasty to treat the stenosis in the right MCA\'s M1 segment. Follow-up DSA confirmed the resolution of stenosis in this segment. Although the remaining branches showed satisfactory blood flow, the vessel wall exhibited irregularities. A review of DSA conducted six months later showed no evident stenosis in the right MCA, with a smooth vessel wall.
CONCLUSIONS: The use of drug-coated balloon angioplasty demonstrated favorable outcomes in repairing and reshaping the blood vessel wall in young patients. Therefore, it may be considered a promising treatment option for similar cases.

UNASSIGNED: To analyze the diagnostic value of HR-VWI in intracranial arterial stenosis and occlusion and compare it with DSA.
UNASSIGNED: A retrospective analysis of clinical data of 59 patients with intracranial arterial stenosis in our hospital was conducted to compare the diagnostic results of the two methods for different degrees of intracranial stenosis and various morphological plaques.
UNASSIGNED: The diagnosis of stenosis and occlusion by both methods showed no significant difference (p > 0.05). Comparison of plaque morphology detected by HR-VWI with pathological examination results showed no significant difference (p > 0.05); however, there was a significant difference between plaque morphology detected by DSA and pathological examination results (p < 0.05). Additionally, there was a significant difference between plaque morphology detected by HR-VWI and DSA (p < 0.05).
UNASSIGNED: HR-VWI technique is comparable to DSA technique in diagnosing intracranial arterial stenosis and occlusion, but it is superior to DSA in plaque morphology diagnosis.

UNASSIGNED: Previous single-center studies have demonstrated that drug-coated balloons (DCBs) may reduce restenosis rates, which is an important factor affecting the prognosis for intracranial interventional therapy. However, currently available cardiac DCBs are not always suitable for the treatment of intracranial atherosclerotic stenosis (ICAS). This study aimed to evaluate the safety and efficacy of a novel DCB catheter designed for patients with severely symptomatic ICAS.
UNASSIGNED: This prospective, multicenter, single-arm, target-value clinical trial was conducted in 9 Chinese stroke centers to evaluate the safety and efficacy of a novel DCB catheter for treating symptomatic severe ICAS. Primary metrics and other indicators were collected and analyzed using SAS version 9.4 (SAS Institute, Cary, NC, USA).
UNASSIGNED: A total of 155 patients were enrolled in this study. The preliminary collection of follow-up data has been completed, while data quality control is ongoing.
UNASSIGNED: Results of this study demonstrated the patency rate, safety, and effectiveness of a novel on-label paclitaxel DCB designed for the treatment of ICAS.
UNASSIGNED: ChiCTR, ChiCTR2100047223. Registered June 11, 2021-Prospective registration, https://www.chictr.org.cn/ChiCTR2100047223.

To directly compare the 90-day outcomes of patients with symptomatic intracranial atherosclerotic disease (ICAD), extracranial carotid atherosclerotic disease (ECAD), and ICAD with concomitant ECAD.
From 2017-2021, patients who had (1) a transient ischemic attack or ischemic stroke within 30 days of admission as evaluated by a stroke neurologist and (2) ipsilateral ICAD and/or ECAD were prospectively enrolled. The cohort was divided into three groups: ICAD, ECAD, and ICAD with concomitant ECAD. The primary outcome assessed was 90-day ischemic stroke recurrence. Secondary outcomes included 90-day myocardial infarction (MI), all-cause mortality, and major adverse cardiovascular events (MACE, including cardiovascular death, nonfatal MI, and/or nonfatal ischemic stroke).
Of 371 patients included in the analysis, 240 (64.7%) patients had ICAD only, 93 (25.0%) patients had ECAD only, and 38 (10.3%) patients had ICAD with concomitant ECAD. On multivariate time-to-event analysis adjusting for potential confounders and with ICAD as the reference comparator, the risk of 90-day clinical outcomes was highest among patients with ICAD and concomitant ECAD, with adjusted hazard ratios of 4.54 (95% CI=1.45, 14.2; p = 0.006), 9.32 (95% CI=1.58, 54.8; p = 0.014), and 8.52 (95% CI=3.54, 20.5; p < 0.001) for 90-day ischemic stroke, MI, and MACE, respectively.
Patients with ICAD and concomitant ECAD have a poorer prognosis and are at significantly higher risk for 90-day ischemic stroke, MI, and MACE. Further research should focus on the evaluation of coronary atherosclerotic disease and more intensive medical therapy in this population.

UNASSIGNED: We sought to investigate whether the prognostic value of intracranial arterial stenosis (ICAS) is consistent across different risk stratifications using the Essen Stroke Risk score (ESRS).
UNASSIGNED: We derived data from the Clopidogrel in High-Risk Patients with Acute Nondisabling Cerebrovascular Events trial. Patients without complete baseline brain imaging data were excluded. Participants were categorized into different risk groups based on ESRS (low risk, 0-2, and high risk ≥ 3). The main outcome was stroke recurrence within 3 and 12 months. Hazard ratios (HRs) and 95% confidence intervals (95%CIs) of ICAS, and other factors associated with stroke recurrence within 3 and 12 months were estimated using the Cox regression method.
UNASSIGNED: During the 3-month follow-up, 54 patients (7.9%) had recurrent stroke in the low-risk group, and 39 patients (9.6%) had recurrent stroke in the high-risk group. ICAS was associated with a higher risk of stroke within 3 months (HR = 2.761; 95%CI = 1.538-4.957; P < 0.001) in the low-risk group, but not in the high-risk group (HR = 1.501; 95%CI = 0.701-3.213; P = 0.296). ICAS was independently associated with higher recurrent risk in the low-risk group (HR = 2.540; 95%CI = 1.472-4.381; P < 0.001), but not in the high-risk group (HR = 1.951; 95%CI = 0.977-3.893; P = 0.058) within 12 months.
UNASSIGNED: ICAS was an independent predictor of both 3- and 12-month stroke recurrence in low-risk but not high-risk patients with minor ischemic stroke or transient ischemic attack according to ESRS stratification.

OBJECTIVE: Inflammation is associated with vascular events. We aimed to investigate the relationship between high-sensitivity C-reactive protein (hsCRP) levels with and without intracranial arterial stenosis (ICAS) and the prognosis of patients with minor stroke or transient ischemic attack.
METHODS: We used data from the Clopidogrel in High-Risk Patients with Acute Nondisabling Cerebrovascular Events trial (derivation cohort) and the Third China National Stroke Registry (validation cohort). Patients were divided into four groups according to the dichotomy of hsCRP level and ICAS status. The primary outcome was new ischemic stroke within 90 days, and the secondary outcome was dependence or death (Modified Rankin Scale score of 3-6) at 90 days. The associations between hsCRP level with and without ICAS and risk of outcomes were analyzed using multivariate Cox regression and logistic regression models.
RESULTS: In the derivation cohort, compared with patients with nonelevated hsCRP levels and no ICAS, those with both elevated hsCRP levels and ICAS had increased risk of recurrent stroke (adjusted hazard ratio [HR], 2.62; 95% confidence interval [CI], 1.28-5.34; p=0.008) and dependence or death (adjusted odds ratio [OR], 7.58; 95% CI, 1.30-44.13; p=0.02). Consistent relationships of elevated hsCRP levels and presence of ICAS with recurrent stroke (adjusted HR, 1.67; 95% CI, 1.13-2.45; p=0.009) and dependence or death (adjusted OR, 1.87; 95% CI, 1.23-2.84; p=0.003) were observed in the validation cohort.
CONCLUSIONS: Concomitant presence of increased hsCRP levels and ICAS was associated with increased risk of stroke recurrence and dependence or death in patients with minor ischemic stroke or transient ischemic attack.

The association between the fibrinogen-to-albumin ratio (FAR) and intracranial arterial stenosis (ICAS) in patients with acute ischemic stroke (AIS) has not yet been reported. In this large-scale investigation, 7894 AIS patients with ICAS-evaluation imaging data from the Third China National Stroke Registry were included. ICAS was defined as >50% stenosis of the intracranial arteries. We dichotomized the degree of ICAS into stenosis and occlusion. The number of ICAS lesions was the total number of intracranial stenotic arteries. Fibrinogen and albumin levels were assessed in the central laboratory of Beijing Tiantan Hospital. Univariate and multivariate analyses with logistic regression were used to determine the association between the FAR quartiles and ICAS. A total of 3900 (49.66%) patients had ICAS. Compared with those of the lowest FAR quartile, the adjusted odds ratios (ORs) and 95% confidence intervals (CIs) of the highest FAR quartile were 1.26 (1.10-1.44), 1.15 (.99-1.33), and 1.19 (1.01-1.39) for ICAS, symptomatic ICAS, and asymptomatic ICAS, respectively. An elevated FAR was also associated with occlusion (adjusted OR: 1.28, 95% CI: 1.10-1.49) and lesion number ≥2 (adjusted OR: 1.25, 95% CI: 1.07-1.45).

Intracranial atherosclerotic stenosis (ICAS) is a common cause of stroke worldwide. However, whether the treatment options for symptomatic ICAS is stent placement or medical therapy alone is still controversial. At present, three multicentre randomised controlled trials (RCTs) have been published, but their research designs are also slightly different and the conclusions are not completely consistent. Therefore, we plan to conduct a systematic review and individual patient data (IPD) meta-analysis of randomised clinical trials to ascertain safety and efficacy of stenting versus medical therapy alone for symptomatic patients with intracranial arterial stenosis.
We will identify RCTs comparing stenting vs medical therapy alone in patients with symptomatic ICAS stenosis (70%-99%) through a systematic search, mainly including PubMed, MEDLINE, EMBASE, the Cochrane Library and ClinicalTrials.gov. Individual-level patient data for a prespecified list of variables will be sought from authors of all eligible studies. The primary outcome was a composite of stroke or death within 30 days, or stroke in territory of qualifying artery beyond 30 days after randomisation. IPD meta-analysis will be conducted with a one-stage approach.
Ethical approval and individual patient consent will not be required in most cases since this IPD meta-analysis will use pseudoanonymised data from RCTs. Results will be disseminated through peer-reviewed journals and international conferences.
CRD42022369922.

UNASSIGNED: Non-invasive and accurate assessment of intracranial arterial stenosis (ICAS) is important for the evaluation of intracranial atherosclerotic disease. This study aimed to evaluate the performance of 3D pointwise encoding time reduction magnetic resonance angiography (PETRA-MRA) and compare its performance with that of 3D time-of-flight (TOF) MRA and computed tomography angiography (CTA), using digital subtraction angiography (DSA) as the reference standard in measuring the degree of stenosis and lesion length.
UNASSIGNED: This single-center, prospective study included a total of 52 patients (mean age 57 ± 11 years, 27 men, 25 women) with 90 intracranial arterial stenoses who underwent PETRA-MRA, TOF-MRA, CTA, and DSA within 1 month. The degree of stenosis and lesion length were measured independently by two radiologists on these four datasets. The degree of stenosis was classified according to DSA measurement. Severe stenosis was defined as a single lesion with >70% diameter stenosis. The smaller artery stenosis referred to the stenosis, which occurred at the anterior cerebral artery, middle cerebral artery, and posterior cerebral artery, except for the first segment of them. The continuous variables were compared using paired t-test or Wilcoxon signed rank test. The intraclass correlation coefficients (ICCs) were used to assess the agreement between MRAs/CTA and DSA as well as inter-reader variabilities. The ICC value >0.80 indicated excellent agreement. The agreement of data was assessed further by Bland-Altman analysis and Spearman\'s correlation coefficients. When the difference between MRAs/CTA and DSA was statistically significant in the degree of stenosis, the measurement of MRAs/CTA was larger than that of DSA, which referred to the overestimation of MRAs/CTA for the degree of stenosis.
UNASSIGNED: The four imaging methods exhibited excellent inter-reader agreement [intraclass correlation coefficients (ICCs) > 0.80]. PETRA-MRA was more consistent with DSA than with TOF-MRA and CTA in measuring the degree of stenosis (ICC = 0.94 vs. 0.79 and 0.89) and lesion length (ICC = 0.99 vs. 0.97 and 0.73). PETRA-MRA obtained the highest specificity and positive predictive value (PPV) than TOF-MRA and CTA for detecting stenosis of >50% and stenosis of >75%. TOF-MRA and CTA overestimated considerably the degree of stenosis compared with DSA (63.0% ± 15.8% and 61.0% ± 18.6% vs. 54.0% ± 18.6%, P < 0.01, respectively), whereas PETRA-MRA did not overestimate (P = 0.13). The degree of stenosis acquired on PETRA-MRA was also more consistent with that on DSA than with that on TOF-MRA and CTA in severe stenosis (ICC = 0.78 vs. 0.30 and 0.57) and smaller artery stenosis (ICC = 0.95 vs. 0.70 and 0.80). In anterior artery circulation stenosis, PETRA-MRA also achieved a little bigger ICC than TOF-MRA and CTA in measuring the degree of stenosis (0.93 vs. 0.78 and 0.88). In posterior artery circulation stenosis, PETRA-MRA had a bigger ICC than TOF-MRA (0.94 vs. 0.71) and a comparable ICC to CTA (0.94 vs. 0.91) in measuring the degree of stenosis.
UNASSIGNED: PETRA-MRA is more accurate than TOF-MRA and CTA for the evaluation of intracranial stenosis and lesion length when using DSA as a reference standard. PETRA-MRA is a promising non-invasive tool for ICAS assessment.