もやもや病

Background Superficial temporal artery to middle cerebral artery (STA-MCA) bypass is a standard-of-care treatment for adult patients with moyamoya disease (MMD). Although the classical technique is end-to-side (E-S) bypass, the side-to-side (S-S) construct has emerged as an alternative strategy with potentially reduced risk of postoperative cerebral hyperperfusion syndrome (CHS). Methods A PRISMA-compliant systematic review of MEDLINE and Embase databases was performed from inception to May 2026 identifying 72 studies. After abstract screening, 14 studies underwent full-text review, and five head-to-head studies met inclusion criteria. A meta-analysis and supplemental sensitivity analyses were conducted to assess robustness, excluding a high-variance study with disparate CHS diagnostic thresholds. Results Five studies were included capturing head-to-head outcomes for a total of 495 patients (194 E-S; 301 S-S). Pooled analysis showed no significant difference in CHS incidence with S-S bypass compared with E-S bypass. Sensitivity analysis excluding a study with high CHS incidence revealed a significant reduction of CHS with S-S bypass (OR = 1.63, 95% CI: 1.19-2.25, p = 0.016), possibly due to decreased heterogeneity, or their inclusion of high-sensitivity diagnostic tools including laser speckle contrast imaging (LSCI) and single-photon emission computed tomography (SPECT), which may have identified a higher incidence of subclinical CHS. No significant difference was observed in the reported incidence of stroke in either model. Findings were unchanged when the analysis was limited to the non-overlapping cohorts. Conclusion S-S constructs for STA-MCA in adult MMD may reduce CHS risk, but without an associated difference in risk of postoperative stroke. Future studies with standardized reporting of CHS are required to validate S-S bypass as a preferred strategy.

PURPOSE: The champagne bottle neck sign (CBNS) is regarded as a characteristic finding of the cervical internal carotid artery in patients with moyamoya disease (MMD). However, there is no universal definition, and its clinical significance remains unclear. Here, we aimed to propose a novel CBNS grading system using catheter angiography. METHODS: First, we proposed a novel grading system for CBNS using the lateral view of common carotid artery angiograms. Three grades were defined (grades 0,1, and 2). CBNS grade was defined as a ratio of the internal carotid artery diameter to the common carotid artery diameter of < 0.5 on the lateral view of the common carotid artery angiogram. CBNS grade 2 additionally required plump enlargement of the bulbous portion of the cervical internal carotid artery. We then conducted a retrospective case-control study including 134 patients (247 sides) with MMD and 94 patients (98 sides) with non-MMD intracranial large artery stenosis/occlusion. Finally, we analyzed the association between CBNS grade and clinical features, including Suzuki stage and hemodynamic impairment. RESULTS: CBNS grade 2 demonstrated a strong association with MMD, although grade 2 CBNS was infrequently observed among patients with non-MMD intracranial large-artery stenosis/occlusion (p < 0.001). Additionally, multivariate analysis demonstrated a significant association between CBNS grade 2 and hemodynamic impairment, which was present in 32.5% (13/40) and 55.9% (19/34) of MMD sides at CBNS grades 1 and 2, respectively (p < 0.001). CONCLUSION: We propose a CBNS grading system for MMD patients. CBNS grade 2, characterized by pronounced enlargement of the bulbous portion of the cervical internal carotid artery, may serve as an imaging marker of advanced Suzuki stage and hemodynamic impairment in MMD.

Moyamoya disease is a rare cerebrovascular disease of chronic and progressive nature, characterized by stenosis and occlusion of the Circle of Willis vessels and formation of compensatory collateral circulation. Here we have reported a case of a 10 year old male patient who first developed seizure, hemiparesis 1 year ago, followed by deteriorating hemiparesis, prolonged fever and blindness. Although seizure hemiparesis are common presentations of the disease, blindness and prolonged fever have been rarely reported. The diagnosis in this case was established by Magnetic Resonance Imaging (MRI) and Magnetic Resonance Angiogram (MRA), where vessel occlusion, encephalomalacia and compensatory circulation formation were detected. Due to financial hardships and limitations of the surgical settings in the hospital, conservative treatment was provided. In fact, there has been no report of performing revascularization surgery in a patient with Moyamoya disease in Bangladesh. This case highlights that progressive cortical blindness and posterior circulation involvement may occur in pediatric Moyamoya disease. In resource-limited settings, characteristic MRI and MRA findings can facilitate early diagnosis when Digital Subtraction Angiography is unavailable, although definitive management with revascularization surgery remains the preferred treatment.

BACKGROUND AND OBJECTIVES: Given the natural history of hemorrhagic moyamoya disease (hMMD) is unclear and research on long-term outcomes following indirect revascularization is limited, this study sought to assess its natural course and the efficacy of indirect revascularization. METHODS: We conducted a retrospective analysis of patients with hMMD admitted to the Chinese PLA General Hospital from April 2002 to March 2022. Clinical characteristics, radiologic findings, rebleeding events, and prognostic outcomes were evaluated. Logistic and Cox regression models identified risk factors for poor outcomes and rebleeding in the natural history. After overlap weighting to balance the encephaloduroarteriosynangiosis (EDAS) and conservative groups, weighted regression models were used to compare outcomes. RESULTS: This study enrolled 1,050 patients with hMMD. The mean age at first bleeding was 37.0 ± 12.3 years, and 577 (55.0%) were female. Among them, 123 were in the natural history group and 927 in the EDAS group. The natural history group had a significantly older mean age (43.7 ± 12.1 vs 36.1 ± 12.1 years, p < 0.001), while the proportion of female patients was similar between the 2 groups (55.7% vs 49.6%, p = 0.204). In the natural history course, anterior choroidal artery (AChA) dilation (hazard ratio [HR] 8.64, 95% CI 3.03-24.67, p < 0.001) was associated with rebleeding. Female sex (OR 3.93, 95% CI 1.27-12.13, p = 0.017), AChA dilation (OR 3.41, 95% CI 1.32-8.82, p = 0.012), and bilateral lesions (OR 5.45, 95% CI 1.45-20.52, p = 0.012) were associated with poor outcomes in patients with hMMD. After overlap weighting, treatment with EDAS was associated with a lower incidence of rebleeding events (HR 0.49, 95% CI 0.30-0.81, p = 0.006) and improved outcomes (OR 0.85, 95% CI 0.79-0.93, p < 0.001). DISCUSSION: In conclusion, hMMD occurs mainly in adult patients. In the natural history of hMMD, AChA dilation was associated with rebleeding, while female sex, AChA dilation, and bilateral lesions were associated with poor outcomes. This study suggests that EDAS is associated with a reduced incidence of rebleeding and improved outcomes, indicating that EDAS may be an effective treatment for hMMD. However, given the retrospective design, prospective validation is needed.

Moyamoya Angiopathy (MMA) is a rare, progressive, steno-occlusive cerebrovascular disorder. The disease can be idiopathic (Moyamoya Disease, MMD) or associated with heritable or acquired conditions (i.e., Moyamoya Syndrome, MMS). Among clinical symptoms of MMA, cognitive aspects are acknowledged but not well-characterized, and their proper neuropsychological assessment has never been standardized. We conducted a systematic review to investigate the characteristics of cognitive deficits in non-revascularized adults MMD patients, as well as the main neuropsychological tests used for their evaluation. The literature search was performed by two independent reviewers across three different databases, including PubMed, EMBASE, and Scopus. Quality assessment was independently performed by two reviewers through the Johanna Briggs Institute critical appraisal tools. Data on sample size, patients' demographics, age at diagnosis, clinical and radiological characteristics of MMD, administered neuropsychological tests, prevalence of cognitive impairment (CI), and altered cognitive domains were extracted. The literature search allowed the inclusion of 12 papers, confirming the limited literature on this topic. The prevalence of CI ranged from 23 to 92% across studies. Executive functions, especially processing speed and cognitive flexibility, followed by memory skills appeared to be the most affected cognitive domains. Conversely, other cognitive functions have been less investigated. The most commonly used neuropsychological tests were the Trail Making Test, the Digit Span test, and Verbal Fluency. Despite growing awareness and knowledge of cognitive aspects in patients with MMD, inconsistency remains in identifying specific neuropsychological profiles, and no standardized assessment protocol has yet been established.