Influence of an improvement in frontal lobe hemodynamics on neurocognitive function in adult patients with moyamoya disease.

BACKGROUND: In adults, moyamoya disease (MMD) often presents with slight neurocognitive impairment, which may result from frontal lobe hemodynamic insufficiency. METHODS: In this study, we performed revascularization surgery by superficial temporal artery-anterior cerebral artery (ACA) direct bypass in 20 adults with MMD with poor anterograde ACA flow (Group M). The pre- and postoperative neurocognitive test results of these patients were retrospectively analyzed. The comparative group (Group C) included 23 patients with unruptured aneurysms or brain tumors who underwent craniotomy, as well as the same neurocognitive tests as Group M. We calculated the compositive frontal lobe function index (CFFI) based on the results of seven neurocognitive tests for each patient, and the difference between the pre- and postoperative CFFI values (CFFI Post - Pre) was compared between the two groups. RESULTS: Frontal perfusion improved postoperatively in all patients in Group M. The CFFI Post - Pre was significantly higher in Group M than in Group C (0.23 ± 0.44 vs. - 0.20 ± 0.32; p < 0.001). After adjusting for postoperative age, sex, preoperative non-verbal intelligence quotient, and preoperative period of stress, Group M had a significantly higher CFFI Post - Pre than Group C in the multiple regression analysis (t value = 4.01; p < 0.001). CONCLUSION: Improving frontal lobe hemodynamics might be the key for improving neurocognitive dysfunction in adults with MMD. The surgical indication and method should be considered from the perspective of both stroke prevention and neurocognitive improvement or protection.

STA-MCA Double-Barrel Bypass: A Systematic Review of Technique and Single-Arm Meta-Analysis of Outcomes.

INTRODUCTION: Conventionally, one branch of the superficial temporal artery (STA) is utilized to revascularize the middle cerebral artery (MCA). However, there is the possibility of utilizing both branches of the STA when performing the bypass, characterizing the double-barrel (DB) STA-MCA bypass. Notably, a lack of studies evaluating this technique led the authors to conduct a systematic review and single-arm meta-analysis. METHODS: PubMed, Embase and Web of Science were searched systematically for publications of DB-STA-MCA bypass on November 1st, 2023. The findings were reported according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Case reports were not included for statistical analysis purposes. RESULTS: The review included 408 patients and 534 bypasses from 34 studies. The main etiology was Moyamoya disease (64.6%), followed by cerebral ischemia (22.2%) and aneurysms (12.5%). The median of the mean follow-ups of each study was 12.8 months (range 1.5-87.9). The postoperative patency was 100%. The follow-up patency was 98% (95% CI: 96%-100%; I2 = 0%). The procedure-related mortality was 0% (95% CI: 0%-1%; I2 = 0%). Aneurysms obtained 87% (95% CI: 72%-100%; I2 = 4%) of good clinical outcomes, while Moyamoya disease yielded a rate of 70% (95% CI: 10%-100%; I2 = 97%). Ischemic complications occurred at a rate of 6% (95% CI: 2%-11%; I2 = 36%), while hemorrhagic occurred at 6% (95% CI: 1%-11%; I2 = 56%). Hyperperfusion syndrome rate was calculated as 18% (7%-30%; I2 = 55%) for Moyamoya disease. CONCLUSIONS: The procedure appears to be safe, with excellent patency rates. The clinical efficacy for ischemic and Moyamoya diseases warrants further standardized robust investigation with a broader number of patients, and aneurysm studies are required to enhance sample sizes. The main complication for the Moyamoya subgroup is hyperperfusion syndrome.

Clinical Course, Therapy, and Long-Term Outcomes of Children With Moyamoya Disease and Posterior Cerebral Artery Involvement.

BACKGROUND AND OBJECTIVES: Posterior cerebral artery involvement (PCAi) has been identified as an important factor related to poor prognosis in moyamoya disease (MMD). This study summarized the characteristics of children with MMD and PCAi, clarified the clinical course, identified prognostic predictors, and investigated the long-term effect of encephaloduroarteriosynangiosis for posterior circulation (EDAS-p). METHODS: We retrospectively reviewed all our pediatric MMD cases with follow-up angiograms from November 2003 to December 2016. PCAi was classified as early-onset at initial diagnosis and delayed-onset after anterior circulation revascularization. Multivariable data including clinical features, radiographic findings, and surgical outcomes were analyzed. RESULTS: Among 570 children with MMD, 246 (43.2%) had PCAi, with 176 (30.9%) classified as early-onset PCAi. During a median follow-up period of 10 years, 17.8% (70/394) of patients without initial PCAi developed delayed-onset PCAi. The median time to detection of a new PCA lesion was 15.5 (range 7-110) months from initial diagnosis, with a median age of 10.5 (3-22). Younger age at onset, familial occurrence, advanced Suzuki stages, and preoperative infarctions were predictors of delayed-onset PCAi. EDAS-p was performed on 294 hemispheres of 195 patients with PCAi. Stroke-free survival was significantly higher in the EDAS-p group than in the non-EDAS-p group (99.0% vs 90.2%; p < 0.001 [Breslow test]; p = 0.001 [log-rank test]; median follow-up: 101 months). DISCUSSION: PCAi is not uncommon in children with MMD, underscoring the need for long-term close clinical monitoring, especially in patients with high-risk factors for PCA progression. EDAS-p may be a safe and effective procedure for preventing subsequent stroke in children with MMD and PCAi.

FOXM1 c.1205 C > A mutation is associated with unilateral Moyamoya disease and inhibits angiogenesis in human brain endothelial cells.

Unilateral moyamoya disease (MMD) represents a distinct subtype characterised by occlusive changes in the circle of Willis and abnormal vascular network formation. However, the aetiology and pathogenesis of unilateral MMD remain unclear. In this study, genetic screening of a family with unilateral MMD using whole-genome sequencing helped identify the c.1205 C > A variant of FOXM1, which encodes the transcription factor FOXM1 and plays a crucial role in angiogenesis and cell proliferation, as a susceptibility gene mutation. We demonstrated that this mutation significantly attenuated the proangiogenic effects of FOXM1 in human brain endothelial cells, leading to reduced proliferation, migration, and tube formation. Furthermore, FOXM1 c.1205 C > A results in increased apoptosis of human brain endothelial cells, mediated by the downregulation of the transcription of the apoptosis-inhibiting protein BCL2. These results suggest a potential role for the FOXM1 c.1205 C > A mutation in the pathogenesis of unilateral MMD and may contribute to the understanding and treatment of this condition.

EFFECT OF DIRECT SURGICAL REVASCULARIZATION ON CEREBRAL HEMODYNAMICS AND STROKE DEVELOPMENT IN PATIENTS WITH MOYAMOYA DISEASE.

Decreased cerebral blood flow (CBF) leads to impaired cerebral hemodynamics, which causes an increased risk of stroke. Revascularization has been shown to improve CBF in patients with moyamoya disease. The study is devoted to the retrospective study of clinical features and cerebral hemodynamic characteristics of 17 patients with moyamoya disease before, during and after surgical treatment using extracranial-intracranial (EC-IC) bypass by STA-MCA type. Patients underwent superficial temporal artery-middle cerebral artery bypass surgeries. All patients were carried out by DSA, MSCT-angiography, and MSCT-perfusion imagine (MSCTPI) before and 6 months after surgery. The hemodynamic parameters during MSCTPI, changes in cerebral vascular pattern, and clinical outcomes were evaluated. Cerebral blood flow and mean transit time (MTT) were measured using MSCT-perfusion imaging to identify areas of hypoperfusion. Intraoperative indocyanine green (ICG) analysis was performed to assess local cerebral hemodynamics before and after the creation of the STA-MCA bypass. Results showed that hemodynamics improved significantly on the surgery side after revascularization. After STA-MCA bypass CBF increased and MTT reduced by almost 2 times compared to the level before the bypass. The modified Rankin Scale scores demonstrated an improvement in the neurological status of patients following surgical revascularization. Thus, STA-MCA-type surgical revascularization significantly improved cerebral perfusion parameters and reduced the risk of stroke in patients with moyamoya disease. MSCTPI can serve as an effective and noninvasive method for monitoring cerebral hemodynamics in these patients. Intraoperative ICG angiography is a safe method that can display hemodynamic characteristics in the surgical area.

Evaluation of deep learning algorithms in detecting moyamoya disease: a systematic review and single-arm meta-analysis.

The diagnosis of Moyamoya disease (MMD) relies heavily on imaging, which could benefit from standardized machine learning tools. This study aims to evaluate the diagnostic efficacy of deep learning (DL) algorithms for MMD by analyzing sensitivity, specificity, and the area under the curve (AUC) compared to expert consensus. We conducted a systematic search of PubMed, Embase, and Web of Science for articles published from inception to February 2024. Eligible studies were required to report diagnostic accuracy metrics such as sensitivity, specificity, and AUC, excluding those not in English or using traditional machine learning methods. Seven studies were included, comprising a sample of 4,416 patients, of whom 1,358 had MMD. The pooled sensitivity for common and random effects models was 0.89 (95% CI: 0.85 to 0.92) and 0.92 (95% CI: 0.85 to 0.96), respectively. The pooled specificity was 0.89 (95% CI: 0.86 to 0.91) in the common effects model and 0.91 (95% CI: 0.75 to 0.97) in the random effects model. Two studies reported the AUC alongside their confidence intervals. A meta-analysis synthesizing these findings aggregated a mean AUC of 0.94 (95% CI: 0.92 to 0.96) for common effects and 0.89 (95% CI: 0.76 to 1.02) for random effects models. Deep learning models significantly enhance the diagnosis of MMD by efficiently extracting and identifying complex image patterns with high sensitivity and specificity. Trial registration: CRD42024524998 https://www.crd.york.ac.uk/prospero/displayrecord.php?RecordID=524998.

Effective Smartphone Application Use for Postoperative Management of Moyamoya Disease.

Continuous and careful management is necessary after revascularization surgery for moyamoya disease (MMD). The postoperative information has been shared in person or by telephone and emails among doctors; however, this is not always efficient. We aimed to describe the feasibility of remote diagnosis and text chats using a smartphone application on postoperative MMD management. Twenty consecutive patients who underwent combined direct and indirect revascularization for MMD were prospectively investigated in this study. In ten patients, the operator viewed postoperative images uploaded on a smartphone screen using the Join application (Allm Inc., Tokyo, Japan). The doctors shared the radiological findings and treatment plans using the group text chat function and performed postoperative management. We evaluated the intermodality agreements of radiological findings between the smartphone screen and conventional viewer. Postoperative courses were compared between the two patient groups that used or did not use the application. All postoperative images were uploaded to the cloud server and the operator viewed them remotely on the smartphone screen without restriction of location. Detected abnormal findings were cerebral hyperperfusion (CHP), CHP-related watershed shift phenomenon, fluid-attenuated inversion recovery cortical hyperintensity, high signal intensity on diffusion-weighted imaging, CHP-related crossed cerebellar diaschisis, and hypoperfusion. Radiological agreement between the modalities was good in all cases, and additional findings were not obtained on the conventional viewer. The postoperative courses of the Join group were as good as those of the control group. Remote radiological diagnosis and text chat using a smartphone application were feasible and useful for efficient and safe postoperative MMD management.

Effects of goal-directed analgesia using the analgesia nociception index in children undergoing surgery for moyamoya disease: A randomised controlled trial.

BACKGROUND: The potential benefits of Analgesia Nociception Index guided intra-operative analgesia on intra-operative opioid consumption remains to be demonstrated in paediatric anaesthesia. OBJECTIVES: This study aimed to explore the effects of Analgesia Nociception Index guided analgesia on sufentanil consumption during anaesthesia and postoperative pain scores in paediatric patients with moyamoya disease. DESIGN: A prospective randomised controlled study. SETTING: Seoul National University Children's Hospital, Seoul, Republic of Korea. PATIENTS: A total of 40 children scheduled for encephaloduroarteriosynangiosis. MAIN OUTCOME MEASURES: The primary outcome was total intra-operative sufentanil consumption, and the secondary outcomes included postoperative pain scores and incidence of opioid-related adverse events. RESULTS: The Analgesia Nociception Index group showed lower intra-operative sufentanil consumption (in µg kg -1  h -1 ) compared with the Standard group (0.30 ±â€Š0.12 and 0.39 ±â€Š0.17, respectively; mean difference, -0.09; 95% confidence interval, -0.19 to 0.00; P  = 0.049). Postoperatively, compared with the Standard group, the Analgesia Nociception Index group reported lower median pain scores at 18 and 24 h and maximum pain within 24 h (1 [0 to 2] vs. 3 [2 to 5]; P  = 0.004, 1 [0 to 2] vs. 3 [1 to 4]; P  = 0.041, and 4 [3 to 5] vs. 5 [4 to 7]; P  = 0.045, respectively), with fewer patients experiencing nausea (3 [15%] vs. 10 [50%], P  = 0.043). CONCLUSION: The Analgesia Nociception Index guided analgesic protocol can reduce intra-operative sufentanil consumption and postoperative pain within 24 h with fewer nausea symptoms in paediatric patients with moyamoya disease who undergo encephaloduroarteriosynangiosis. TRIAL REGISTRATION NUMBER: NCT05672212.

Modified craniotomy procedures and virtual reality technology for accurate localization of the frontal branch of MMA in revascularization surgery.

The frontal branch of middle meningeal artery (MMA) can easily be damaged during revascularization surgery. To precise locate it and minimize its injury, we propose a set of modified craniotomy procedures combined with simple virtual reality (VR) technology based on three-dimensional (3D) Slicer simply, economically, and efficiently. Patients with Moyamoya disease (MMD) and internal carotid artery occlusion (ICAO) who received revascularization from January 2015 to December 2022 were divided into two groups based on the methods used to locate the MMA: traditional methods and precise MMA locating with VR technology. Patient demographics and clinical characteristics were analyzed to compare the preservation rates of MMA. The distances between this artery and bony anatomical landmarks were also measured to better understand its localization. There was no significant difference in baseline characteristics between the two groups. The precise MMA locating group exhibited a significantly higher preservation rate of the frontal branch of MMA (p = 0.037, 91.7% vs. 68.2%). Over 77% of patients had their frontal branch of MMA partially or completely surrounded by bony structures to varying degrees. Therefore, the combination of modified craniotomy procedures, 3D Slicer, and simple VR technology represents an economical, efficient, and operationally simple strategy.

The usefulness of super-selective arterial spin labeling for postoperative evaluation of pediatric moyamoya disease: technical note.

Moyamoya disease is characterized by progressive internal carotid artery (ICA) occlusion. Extracranial-intracranial bypass surgery is effective, particularly in pediatric patients; imaging plays a crucial role in evaluating intracranial perfusion pre- and post-surgery. Arterial spin labeling (ASL) is a magnetic resonance technique employed for noninvasive, whole-brain perfusion assessment by magnetically labeling inflowing blood. However, ASL cannot evaluate the territories and development of each vessel perfusion compared with digital subtraction angiography (DSA). Recently, super-selective ASL (SS-ASL) has been developed, performing pinpoint labeling on a specific artery at a time, and offering a tomographic view that distinctly displays blood supply areas for each vessel. Unlike DSA, SS-ASL is noninvasive and can be repeatedly performed in pediatric patients. In conclusion, SS-ASL is useful for evaluating bypass development over time and understanding the pathophysiology of pediatric moyamoya disease.

Routine screening serum tests to detect underlying thyroid and autoimmune diseases in patients with moyamoya disease.

BACKGROUND: Recent studies have revealed that thyroid and autoimmune diseases may be associated with sporadic moyamoya disease. However, whether routine screening serum tests to detect these underlying diseases are useful or not remains unclear. METHODS: We retrospectively evaluated 459 patients with moyamoya disease but without previous history of thyroid or autoimmune diseases who underwent the screening serum tests targeting thyroid and autoimmune diseases from 2016 to 2023 in our institute. The number of patients who were diagnosed as thyroid or autoimmune diseases after these tests were investigated. RESULTS: Among the patients who were screened, 237 (42.6 %) patients had abnormal results for some factors, such as thyroid hormones or autoantibodies. After consultation with endocrinologists or rheumatologists, 27 (5.9 %) patients were newly diagnosed with thyroid diseases, including six (1.3 %) patients with Graves' disease, 19 (4.1 %) patients with Hashimoto thyroiditis and two (0.4 %) patients with other thyroid diseases; however, none of the patients were diagnosed with nonthyroidal autoimmune diseases, such as Sjogren's syndrome, antiphospholipid syndrome, or rheumatoid arthritis, listed as moyamoya-related diseases and targeted by our screening serum tests. Patients with newly diagnosed underlying diseases were more likely to be female compared to patients without new diagnosis (96.3 % vs. 72.2 %, p = 0.03). CONCLUSION: Routine thyroid-related serum screening may be clinically meaningful in patients with moyamoya disease to detect occult thyroid diseases, especially in female patients. However, routine serum screening tests targeting other autoimmune diseases are not recommended unless the patients have equivalent symptoms.

Outcomes of Bypass Surgery in Adult Moyamoya Disease by Onset Type.

Importance: Moyamoya disease (MMD) is a rare chronic cerebrovascular disease, and the outcomes of bypass management in adult patients remain controversial. Objective: To categorize adult MMD based on asymptomatic, ischemic, and hemorrhagic onset and compare the outcomes (death, hemorrhagic stroke [HS], and ischemic stroke [IS]) of bypass surgery (direct or indirect) with those of conservative management. Design, Setting, and Participants: This retrospective, nationwide, population-based longitudinal cohort study used Korean National Health Insurance Research data to identify adults (aged ≥15 years) with MMD who were diagnosed between January 1, 2008, and December 31, 2020, and followed up until December 31, 2021 (median follow-up, 5.74 [IQR, 2.95-9.42] years). A total of 19 700 participants (3194 with hemorrhagic, 517 with ischemic, and 15 989 with asymptomatic MMD) were included. Data were analyzed from January 2 to April 1, 2023. Exposures: Bypass surgery and conservative management. Main Outcomes and Measures: Death constituted the primary outcome; secondary outcomes consisted of HS or IS. Kaplan-Meier survival curve and Cox proportional hazards regression analysis were applied. The propensity score-matching and stratified analyses were performed to control covariate effects. Results: A total of 19 700 patients (mean [SD] age, 45.43 [14.98] years; 12 766 [64.8%] female) were included. Compared with conservative management, bypass was associated with a reduced risk of death (adjusted hazard ratio [AHR], 0.50 [95% CI, 0.41-0.61]; P < .001) and HS (AHR, 0.36 [0.30-0.40]; P < .001) in hemorrhagic MMD; reduced risk of IS (AHR, 0.55 [95% CI, 0.37-0.81]; P = .002) in ischemic MMD; and reduced risk of death (AHR, 0.74 [95% CI, 0.66-0.84]; P < .001) in asymptomatic MMD. However, bypass was associated with an increased risk of HS (AHR, 1.76 [95% CI, 1.56-2.00]; P < .001) in asymptomatic MMD. Both direct and indirect bypass demonstrated similar effects in hemorrhagic and asymptomatic MMD, except only direct bypass was associated with a reduced risk of IS (AHR, 0.52 [95% CI, 0.33- 0.83]; P = .01) in ischemic MMD. After stratification, bypass was associated with a reduced risk of death in patients younger than 55 years with ischemic (AHR, 0.34 [95% CI, 0.13- 0.88]; P = .03) and asymptomatic (AHR, 0.69 [95% CI, 0.60-0.79]; P < .001) MMD, but an increased risk of HS in patients 55 years or older with ischemic MMD (AHR, 2.13 [95% CI, 1.1-4.16]; P = .03). Conclusions and Relevance: The findings of this cohort study of bypass outcomes for patients with MMD emphasize the importance of tailoring management strategies in adult patients based on onset types.

3D exoscopic versus microscopic superficial temporal artery to middle cerebral artery bypass surgery for moyamoya disease - a comparative series.

PURPOSE: Superficial temporal artery to middle cerebral artery (STA-MCA) direct bypass surgery is the most common surgical procedure to treat moyamoya disease (MMD). Here, we aim to compare the performance of the 3D exoscope in bypass surgery with the gold standard operative microscope. METHODS: All direct STA-MCA bypass procedures performed at a single university hospital for MMD between 2015 and 2023 were considered for inclusion. Data were retrospectively collected from patient files and surgical video material. From 2020 onwards, bypass procedures were exclusively performed using a digital three-dimensional exoscope as visualization device. Results were compared with a microsurgical bypass control group (2015-2019). The primary endpoint was defined as total duration of surgery, duration of completing the vascular anastomosis (ischemia time), bypass patency, number of stiches to perform the anastomosis, added stiches after leakage testing of the anastomosis and the Glasgow outcome scale (GOS) at last follow-up as secondary outcome parameter. RESULTS: A total of 16 consecutive moyamoya patients underwent 21 STA-MCA bypass procedures. Thereof, six patients were operated using a microscope and ten patients using an exoscope (ORBEYE® n = 1; AEOS® n = 9). Total duration of surgery was comparable between devices (microscope: 313 min. ± 116 vs. exoscope: 279 min. ± 42; p = 0.647). Ischemia time also proved similar between groups (microscope: 43 min. ± 19 vs. exoscope: 41 min. ± 7; p = 0.701). No differences were noted in bypass patency rates. The number of stiches per anastomosis was similar between visualization devices (microscope: 17 ± 4 vs. exoscope: 17 ± 2; p = 0.887). In contrast, more additional stiches were needed in microscopic anastomoses after leakage testing the bypass (p = 0.035). CONCLUSION: Taking into account the small sample size, end-to-side bypass surgery for moyamoya disease using a foot switch-operated 3D exoscope was not associated with more complications and led to comparable clinical and radiological results as microscopic bypass surgery.

How I do it: operative nuances of the Encephalo-Duro-Mio-Synangiosis (EDMS) technique for pediatric moyamoya disease and syndrome.

BACKGROUND: Moyamoya disease, a progressive occlusive arteriopathy mainly affecting the supraclinoid internal carotid artery, leads to abnormal "Moyamoya vessels" and ischemic events in children due to decreased cerebral blood flow. Surgery, especially indirect revascularization, is suggested for pediatric Moyamoya cases. METHOD: We present the Encephalo-Duro-Mio-Synangiosis (EDMS) technique, illustrated with figures and videos, based on 14 years' experience performing 71 surgeries by the senior author (SGJ) and the Moyamoya Interdisciplinary Workteam at "Prof. Dr. J. P. Garrahan" Pediatric Hospital. CONCLUSION: EDMS is a simple and effective treatment for Moyamoya disease, enhancing procedure precision and safety, reducing associated risks, complications, and improving clinical outcomes.

Prediction of cerebral infarction after bypass surgery in adult moyamoya disease: using pulsatility index on TCD.

BACKGROUND: At present, the most effective treatment for symptomatic moyamoya disease (MMD) is surgery. However, the high incidence of postoperative complications is a serious problem plaguing the surgical treatment of MMD, especially the acute cerebral infarction. Decreased cerebrovascular reserve is an independent risk factor for ischemic infarction, and the pulsatility index (PI) of transcranial Doppler (TCD) is a common intuitive index for evaluating intracranial vascular compliance. However, the relationship between PI and the occurrence of ischemic stroke after operation is unclear. OBJECTIVE: To explore whether the PI in the middle cerebral artery (MCA) could serve as a potential predictor for the occurrence of ischemic infarction after bypass surgery in MMD. METHODS: We performed a retrospective analysis of data from 71 patients who underwent combined revascularization surgery, including superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis and encephalo-duro-myo-synangiosis (EDMS). The patients were divided into two groups according to the median of ipsilateral MCA-PI before operation, low PI group (MCA-PI < 0.614) and high PI group (MCA-PI ≥ 0.614). Univariate and multivariate regression analysis were used to explore risk factors affecting the occurrence of postoperative cerebral infarction. RESULTS: Among the 71 patients with moyamoya disease, 11 patients had cerebral infarction within one week after revascularization. Among them, 10 patients' ipsilateral MCA-PI were less than 0.614, and another one's MCA- PI is higher than 0.614. Univariate analysis showed that the lower ipsilateral MCA-PI (0.448 ± 0.109 vs. 0.637 ± 0.124; P = 0.001) and higher Suzuki stage (P = 0.025) were linked to postoperative cerebral infarction. Multivariate analysis revealed that lower ipsilateral MCA-PI was an independent risk factor for predicting postoperative cerebral infarction (adjusted OR = 14.063; 95% CI = 6.265 ~ 37.308; P = 0.009). CONCLUSIONS: A lower PI in the ipsilateral MCA may predict the cerebral infarction after combined revascularization surgery with high specificity. And combined revascularization appears to be safer for the moyamoya patients in early stages.

Preliminary outcomes of endovascular treatment of moyamoya disease.

PURPOSE: This study aimed to investigate the effectiveness and safety of endovascular revascularisation of intracranial artery occlusion and stenosis in moyamoya disease using stent angioplasty. MATERIALS AND METHODS: We recruited 12 patients (8 women and 4 men) with occlusion and stenosis of intracranial arteries in the context of moyamoya disease who underwent endovascular stent angioplasty. Clinical data, baseline conditions, lesion location, treatment outcomes, periprocedural complications, and follow-up outcomes were analysed. RESULTS: The occlusion was located at the M1 segment of the middle cerebral artery in 8 patients, at both the M1 and A2 segments in one patient, and at the C7 segment of the internal carotid artery in 3. Thirteen stents were deployed at the occlusion site, including the low-profile visualized intraluminal support (LVIS) device in 8 patients, an LVIS device and a Solitaire AB stent in one, and a Leo stent in 3, with a success rate of 100% and no intraprocedural complications. Plain CT imaging after stenting revealed leakage of contrast agent, which disappeared on the second day, resulting in no clinical symptoms or neurological sequelae. Follow-up angiography studies were performed in all patients for 6-12 months (mean, 8.8). Slight asymptomatic in-stent stenosis was observed in 2 patients (16.7%), and no neurological deficits were observed in the other patients. All preoperative ischaemic symptoms completely disappeared at follow-up. CONCLUSION: Stent angioplasty is a safe and effective treatment for occlusion and stenosis of intracranial arteries in moyamoya disease.

RNF213 Polymorphisms in Intracranial Artery Dissection.

The ring finger protein 213 gene (RNF213) is involved in several vascular diseases, both intracranial and systemic ones. Some variants are common in the Asian population and are reported as a risk factor for moyamoya disease, intracranial stenosis and intracranial aneurysms. Among intracranial vascular diseases, both moyamoya disease and intracranial artery dissection are more prevalent in the Asian population. We performed a systematic review of the literature, aiming to assess the rate of RNF213 variants in patients with spontaneous intracranial dissections. Four papers were identified, providing data on 53 patients with intracranial artery dissection. The rate of RNF213 variants is 10/53 (18.9%) and it increases to 10/29 (34.5%), excluding patients with vertebral artery dissection. All patients had the RNF213 p.Arg4810Lys variant. RNF213 variants seems to be involved in intracranial dissections in Asian cohorts. The small number of patients, the inclusion of only patients of Asian descent and the small but non-negligible coexistence with moyamoya disease familiarity might be limiting factors, requiring further studies to confirm these preliminary findings and the embryological interpretation.

Exploration of the risk factor for infarction after revascularization in moyamoya disease.

RESULTS: Eventually, 108 consecutive patients received 174 surgeries were enrolled, experienced new or expanded infarction occured in 13 (7.47%) surgeries, which showed higher Suzuki stage on the non-operative side, more posterior cerebral artery (PCA) involvement, and more intraoperative hypotension compared to those without infarction(p < .05). The Suzuki stage on the non-operative side had the highest area under the curve (AUC) of 0.737, with a sensitivity of 0.692 and specificity of 0.783. Combination of the three factors showed better efficiency, with an AUC of 0.762, a sensitivity of 0.692, and a specificity of 0.907. CONCLUSIONS: Revascularization was a safe option for patients with MMD, higher Suzuki stage on the non-operative side, PCA involvement, and intraoperative hypotension might be the risk factors for new or expanded infarction after revascularization in patients with MMD.

Incidence, prevalence, and treatment of Moyamoya disease in Japan: A population-based descriptive study.

BACKGROUND: Moyamoya disease (MMD) is characterized by progressive stenosis or occlusion of the terminal portions of the bilateral internal carotid arteries. A Japanese survey in 2003 reported an incidence and prevalence of MMD of 0.54 and 6.03 per 100,000 people, respectively, showing an upward trend over previous surveys. An update to these estimates is therefore warranted. Additionally, evidence is lacking on trends in revascularization and antiplatelet therapy in MMD patients. METHODS: We conducted a population-based descriptive study using a Japanese claims database. From fiscal year (FY) 2015 to 2019, we standardized the incidence and prevalence estimates of MMD to the 2015 Japanese census population by age and sex. We also estimated the 1-year cumulative incidence of revascularization among incident MMD patients and the proportion of prevalent MMD patients receiving antiplatelet therapy in each FY. RESULTS: The age-standardized male-to-female ratio of both incident and prevalent MMD patients was approximately 1:2. Standardized incidence and prevalence of MMD per 100,000 population increased slightly from 1.8 to 2.4 and 14.7 to 17.6, respectively. The 1-year cumulative incidence of revascularization among incident MMD patients varied between 21.9 % and 28.9 %. Among prevalent MMD patients, 36.6 % to 39.0 % received antiplatelet therapy. CONCLUSIONS: The incidence and prevalence of MMD in Japan from FY 2015 to 2019 were higher than those estimated in 2003. The trends in revascularization and antiplatelet therapy identified in this study will be useful in further improving the quality of MMD clinical practice.