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Postoperative intracerebral hemorrhage (ICH) after direct bypass surgery for Moyamoya disease could contribute to neurologic deterioration. The aim of this study was to evaluate the effectiveness of 5-day bed rest in reducing the occurrence of postoperative ICH. This study included 122 consecutive hemispheres in 87 Japanese adult MMD patients, composed of 80 control hemispheres from historical data and 42 hemispheres after 5-day bed rest. They all underwent direct bypass surgery. The incidence of postoperative ICH and neurologic deterioration assessed via the modified Rankin Scale were investigated and statistically analyzed. Postoperative ICH was observed in 9 out of the 80 (11.3%) control patients, but not in the 42 patients with 5-day bed rest. The incidence of postoperative ICH and neurologic deterioration via the modified Rankin Scale were significantly different between the 2 groups (P = 0.0268 and 0.0078, respectively). Univariate logistic analysis revealed that 5-day bed rest significantly reduced the incidence of postoperative ICH (P = 0.0048). Five-day bed rest after direct bypass surgery dramatically can reduce the incidence of postoperative ICH and neurologic deterioration after direct bypass surgery. [ABSTRACT FROM AUTHOR]

Extracranial carotid artery aneurysms (ECAAs) are rare, with the etiology mainly classified as degeneration or dissection. Pseudoaneurysms in the region are even rarer and are seen following trauma, iatrogenic injury, or infection. We report a case of extracranial carotid artery pseudoaneurysm (pseudo‐ECAA) with a rare clinical course and pathological features. A 58‐year‐old man presented with swelling and purpura on the left side of his neck after sneezing. Radiological examinations suggested a ruptured left common carotid artery aneurysm. The operative findings were consistent with a pseudoaneurysm. Pathological examination revealed disarrangement and degeneration of smooth muscle fibers in the media, in addition to scattered foci of mucoid accumulation and irregular‐shaped cavitation in the medial extracellular matrix, raising the possibility of an intrinsic dysfunction of the vascular wall in the pathological process of pseudoaneurysm formation. [ABSTRACT FROM AUTHOR]

Objective: Moyamoya disease is a chronic but progressive obliterative cerebrovascular disease of bilateral internal carotid arteries (ICAs) causing hemorrhagic or ischemic cerebral strokes. Surgical revascularization has the potential for resolving the capillary vessels, but the effect on the occlusive ICA and the moyamoya vessels after a direct bypass remains unclear. Patient: A 2-year-old girl with a history of repeated transient ischemic attacks and direct bypasses but demonstrating improvement and associated anomaly is reported. A year and a half later, after a bilateral revascularization, an intracerebral capsulized hematoma growth was identified, and it was removed surgically. Neovascularization including many microvessels similar to capillary telangiectasia were identified by pathological investigation despite the reduction of moyamoya vessels on the repeated angiograms after the revascularization surgeries. In the present case, proliferation of capillary vessels was clearly confirmed by direct bypasses. Conclusion: There is no doubt that direct bypasses prevent further ischemic stroke by improving cerebral blood flow. However, they may result in failure in reducing the load of moyamoya vessels, albeit decreasing the potential risk of hemorrhagic strokes. [ABSTRACT FROM AUTHOR]

Background: As subarachnoid hemorrhage due to rupture of an intracranial aneurysm (IA) has quite a poor outcome despite of an intensive medical care, development of a novel treatment targeting unruptured IAs based on the correct understanding of pathogenesis is mandatory for social health.Methods: Using previously obtained gene expression profile data from surgically resected unruptured human IA lesions, we selected G-protein coupled receptor 120 (GPR120) as a gene whose expression is significantly higher in lesions than that in control arterial walls. To corroborate a contribution of GPR120 signaling to the pathophysiology, we used an animal model of IAs and examine the effect of a GPR120 agonist on the progression of the disease. IA lesion was induced in rats through an increase of hemodynamic stress achieved by a one-sided carotid ligation and induced hypervolemia. Eicosapentaenoic acid (EPA) was used as an agonist for GPR120 in this study and its effect on the size of IAs, the thinning of media, and infiltration of macrophages in lesions were examined.Result: EPA administered significantly suppressed the size of IAs and the degenerative changes in the media in rats. EPA treatment also inhibited infiltration of macrophages, a hallmark of inflammatory responses in lesions. In in vitro experiments using RAW264.7 cells, pre-treatment of EPA partially suppressed lipopolysaccharide-induced activation of nuclear factor-kappa B and also the transcriptional induction of monocyte chemoattractant protein 1 (MCP-1), a major chemoattractant for macrophages to accumulate in lesions. As a selective agonist of GPR120, TUG-891, could reproduce the effect of EPA in RAW264.7 cells, EPA presumably acted on this receptor to suppress inflammatory responses. Consistently, EPA remarkably suppressed MCP-1 expression in lesions, suggesting the in vivo relevance of in vitro studies.Conclusions: These results combined together suggest the potential of the medical therapy targeting GPR120 or using EPA to prevent the progression of IAs. [ABSTRACT FROM AUTHOR]

Background: Homozygosity of this p.R4810K founder variant of RNF213moyamoya disease (MMD) susceptibility gene is known to influence the severity of the clinical disease phenotype at disease onset. However, the association between this genotype and long-term clinical manifestations has remained unclear. Objectives: The principal goal of this study was to investigate whether and how the p.R4810K variant of RNF213influences the long-term phenotype in Japanese patients with MMD. Method: This retrospective cohort study included 94 Japanese patients with MMD who underwent direct or combined bypass for revascularization with the p.R4810K genotype determined in our hospital. The following phenotypic parameters were analyzed at disease onset and over a long-term period: age and initial presentation at onset, recurrent stroke after initial revascularization, and final modified Rankin Scale. Results: The p.R4810K genotype was significantly associated with the phenotype at onset, especially in younger patients. Over a median follow-up period of 100 months, recurrent stroke occurred in 6 out of 94 patients: none out of 5 patients with the homozygous variant, 5 out of 64 with the heterozygous variant, and 1 out of 25 in the wild-type group. There were no significant differences among the genotypes. In particular, recurrent cerebral hemorrhage occurred in 5 patients, all possessing the heterozygous variant. The log-rank test showed no difference between the genotypes in the stroke-free survival rate. Furthermore, the p.R4810K genotype was not associated with a poor functional condition. Conclusions: The p.R4810K founder variant of RNF213 affects the phenotype at disease onset. However, the optimal revascularization may be effective, regardless of the genotype, even for the homozygous variant, which has been thought to be the most pathogenic. This genotype may not strongly influence the long-term clinical manifestations or poor prognosis in MMD. [ABSTRACT FROM AUTHOR]

The use of a stent retriever increases the risk of intracranial vasospasm. Here, we report the case of a man who developed severe vasospasm in a long segment of the extracranial internal carotid artery after mechanical irritation by a stent retriever inserted for the treatment of acute cerebral ischemia. A 47-year-old right-handed man presented with sudden-onset right-sided weakness and difficulty speaking. The patient’s National Institutes of Health Stroke Scale score was 6 and he had an Alberta Stroke Program Early Computed Tomography Score of 9. The patient was started on intravenous alteplase therapy, and an acute thrombectomy was performed. Left internal carotid digital subtraction angiography showed narrowing of the left common and internal carotid arteries and occlusion of the proximal left M1 segment of the middle cerebral artery. A stent retriever was retracted into a guiding catheter placed at the left carotid bulb under continuous suction. Recanalization of the middle cerebral artery was not achieved and there was significant narrowing in a long segment of the extracranial internal carotid artery associated with exacerbation of the patient’s aphasia. The cervical vasospasm improved after nicardipine infusion via the catheter. We encountered vasospasm in a long segment of the extracranial internal carotid artery after mechanical irritation by a stent retriever. If a stent retriever is used in a patient with a narrow extracranial internal carotid artery, consideration should be given to using a Penumbra or smaller guiding catheter located in the distal internal carotid artery to prevent irritation to the cervical vessel wall. [ABSTRACT FROM AUTHOR]

Cerebrovascular bypass techniques are the current cornerstone methods to achieve cerebral revascularization for moyamoya disease or syndrome and select cases of vascular pathologies, such as intracranial atherosclerotic occlusive disease and complex aneurysms. Factors influencing bypass efficiency include graft patency, short temporary occlusion time, and precise anastomosis. On the basis of our senior author's vast experience with 1300 bypasses, we recommend performing the anastomosis with the minimal number of stitches as achievable to avoid stenosis of the artery's internal lumen that may occur with unnecessary, additional stitches, preserving patency. After completing the anastomosis, when a leak occurs between the sutures, cottonoid tamponade, hemostatic materials, or adding 1 or 2 sutures to the space is often enough to close the gap. However, additional suture placement can be difficult, which might cause stenosis of the anastomosis and reduce blood flow. In this video, we introduce a bipolar coagulation technique for remodeling the anastomosis orifices, as an alternative manner, when minor leakages occur between the knots (Video 1). We demonstrate this technique in an adult moyamoya disease patient who underwent a superficial temporal artery−to−middle cerebral artery bypass, in this case coagulation of the donor artery wall at the anastomosis made possible to adapt the edges of the donor artery precisely to the recipient artery wall by shrinking its redundancy between the stitches. The most important task is to coagulate the donor side orifice precisely with low-power bipolar coagulation and never coagulate the recipient artery. This coagulation technique is a simple alternative to stop further leakage, and it prevents placing an additional suture and reduces temporary occlusion time. [ABSTRACT FROM AUTHOR]

The article by Zhang et al[1] titled "Clinical Features and Surgical Outcomes of Patients With Moyamoya Disease and the Homozygous I RNF213 i p.R4810 K Variant" reported the long-term outcomes of patients with moyamoya disease harboring the homozygous I RNF213 i p.R4810 K founder variant and treated by an indirect bypass. At almost the same time as their article was published, we also reported the genotype-phenotype correlation in 94 patients with moyamoya disease, including patients with 5 homozygous p.R4810 K variants.[2] In our series, all patients underwent direct or combined bypass, and the median follow-up period was 8 years 4 months. [Extracted from the article]

Table 2 of the original version of this article contained an added data. Correct Table 2 is presented here. [ABSTRACT FROM AUTHOR]

Objective Superficial temporal artery–middle cerebral artery anastomosis is an established treatment for moyamoya disease. However, hemorrhagic cerebral hyperperfusion syndrome (CHS) leads to poor outcomes. This study aimed to identify predictors of hemorrhagic CHS based on regional cerebral blood flow (rCBF) in patients with moyamoya disease. Methods The study included 251 hemispheres in 155 patients with moyamoya disease who underwent preoperative and postoperative rCBF measurements and superficial temporal artery–middle cerebral artery double anastomosis. We used rCBF increase rate for predicting hemorrhagic CHS. rCBF increase rate was calculated by 2 methods. In method 1, the rCBF value on the operated side was compared with the rCBF value on the nonoperated side. In method 2, the postoperative rCBF value on the operated side was compared with the preoperative rCBF value on the operated side. Patients were classified into 4 groups according to rCBF increase rate to predict risk of hemorrhagic CHS. Results Hemorrhagic CHS occurred in 7 (2.8%) hemispheres (no children). Severe hemorrhagic CHS occurred in only 1 (0.4%) hemisphere. Hemorrhagic CHS was observed in patients with ≥30% rCBF increase according to method 1 and ≥50% rCBF increase according to method 2 and was most frequently noted in ≥100% rCBF increase. Conclusions Predictors for hemorrhagic CHS were ≥30% rCBF increase when using method 1 and ≥50% increase when using method 2. Highlights • Hemorrhagic CHS leads to poor outcomes; we identify predictors of hemorrhagic CHS based on rCBF. • We used 2 methods to calculate rCBF increase rate (method 1 and method 2). • Hemorrhagic CHS occurred in 7 of 251 (2.8%) hemispheres. • Predictors for hemorrhagic CHS were ≥30% rCBF increase (method 1) and ≥50% increase (method 2). [ABSTRACT FROM AUTHOR]

Symptomatic cerebral vasospasm after subarachnoid hemorrhage (SAH) is a significant cause of delayed cerebral ischemia that leads to poor outcomes. Selective intra-arterial administration of fasudil hydrochloride (IAF) has been adopted for its vasodilatory effect on spasm arteries to prevent delayed cerebral ischemia. However, its effect on clinical outcomes and predictive factors for good recovery are not fully understood. This study aimed to investigate the outcomes of selective IAF and identify predictive factors for good outcomes in patients with cerebral vasospasm after SAH.A retrospective study of 36 patients with cerebral vasospasm following SAH who underwent selective IAF at our institution between January 2014 and May 2022 was conducted. We evaluated the improvements in neurological findings before and after selective IAF. Statistical analyses were performed to determine factors associated with good outcomes.Selective IAF improved the neurological findings in 26 patients (72.2%). Pre-therapeutic absence of cerebral infarction in more than 1/3 of the spasm artery perfusion area was significantly associated with an improvement in neurological findings (p < 0.0001). Furthermore, there was a tendency for a good outcome when the age was younger (p = 0.093), and the spasm was limited to peripheral vessels (p = 0.065).Our study indicates that selective IAF has a promising effect in improving symptomatic vasospasm, except when a large cerebral infarction exists in the spasm artery perfusion area. Early consideration of selective IAF could be recommended once patients experience symptomatic cerebral vasospasm after SAH. [ABSTRACT FROM AUTHOR]

Intracranial aneurysms (IA) are major causes of devastating subarachnoid hemorrhages. They are characterized by a chronic inflammatory process in the intracranial arterial walls triggered and modified by hemodynamic force loading. Because IA lesion morphology is complex, the blood flow conditions loaded on endothelial cells in each portion of the lesion in situ vary greatly. We created a 3D-casted mold of the human unruptured IA lesion and cultured endothelial cells on this model; it was then perfused with culture media to model physiological flow conditions. Gene expression profiles of endothelial cells in each part of the IA lesion were then analyzed. Comprehensive gene expression profile analysis revealed similar gene expression patterns in endothelial cells from each part of the IA lesion but gene ontology analysis revealed endothelial cell malfunction within the IA lesion. Histopathological examination, electron microscopy, and immunohistochemical analysis indicated that endothelial cells within IA lesions are damaged and dysfunctional. Thus, our findings reveal endothelial cell malfunction in IA lesions and provided new insights into IA pathogenesis., (© The Author(s) 2022. Published by Oxford University Press on behalf of American Association of Neuropathologists, Inc. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Objective: Patients sometimes experience transient neurological events (TNEs) during the early postoperative period after revascularization surgery for childhood moyamoya disease. The clinical background and pathophysiology of TNEs remain unclear. This study aimed to evaluate the incidence rate of TNEs and discuss the factors associated with pediatric moyamoya disease., Methods: The authors retrospectively reviewed 110 cerebral hemispheres of 61 pediatric patients younger than 15 years who were treated from 2011 to 2020. All children underwent either simple double-direct or combined revascularization surgery. Of these 61 patients, 52 underwent bilateral surgery and 9 underwent unilateral surgery. The authors calculated the incidence of TNEs in accordance with a previously reported TNE definition. Cerebral blood flow (CBF) was evaluated in all eligible cases with xenon CT immediately after revascularization surgery., Results: The incidence rate of TNEs in patients with childhood moyamoya disease was 26.4%. TNEs occurred after an average (range) of 6.26 (2-12) days postoperatively without triggers, and all identified TNEs spontaneously resolved within 2 weeks. The most common symptoms were dysarthria, facial palsy, and numbness around the mouth, followed by sensory disturbance of the upper extremities. The presence of focal hyperperfusion on xenon CT performed immediately after revascularization surgery was strongly correlated with the incidence of TNEs (p = 0.0001). Focal hyperperfusion was observed in 43 of 110 operative sides (39.1%). Notably, only 25.6% of patients with focal hyperperfusion showed numerical global hyperperfusion. In addition, a decrease in CBF compared with the thalamic region in the contralateral side was observed in TNE-affected cases (p = 0.0443)., Conclusions: TNEs occurred more frequently in childhood moyamoya disease patients than expected. The clinical background, including symptoms, timing, and duration, was almost identical to TNEs in adults. Focal hyperperfusion, rather than numerical global hyperperfusion, was strongly correlated with the incidence of TNEs. Furthermore, the authors advocate the notion that a clinical course where symptoms occur without triggers may be a unique characteristic of TNEs, especially in childhood moyamoya disease.

Objective: Patients with pediatric moyamoya disease (PMMD) showing recurrent symptoms or decreased cerebral blood flow after initial revascularization therapy may require additional revascularization to improve their clinical condition. The authors evaluated the clinical and hemodynamic benefits of an occipital artery (OA)-middle cerebral artery (MCA) bypass for patients with PMMD who have undergone an initial revascularization procedure., Methods: The authors retrospectively identified 9 patients with PMMD who had undergone OA-MCA bypass between March 2013 and December 2017, and who had received a previous superficial temporal artery-MCA bypass. The following clinical data were collected: initial revascularization procedure, symptoms (presence or recurrence), pre- and postoperative cerebral blood flow and cerebrovascular reactivity (CVR) changes, posterior cerebral artery (PCA) stenosis, PCA-related and nonrelated symptoms, and latest follow-up., Results: Preoperatively, all patients (n = 9) suffered non-PCA-related recurrent symptoms, and 4 had PCA-related symptoms. At 1-year follow-up, all patients with PCA-related symptoms showed complete recovery. Additionally, 8 (89%) patients with non-PCA symptoms experienced improvement. Only 1 (11%) patient showed no improvement after the surgical procedure. The mean pre- and postoperative CVR values of the MCA territory were 14.8% and 31.3%, respectively, whereas the respective mean CVR values of the PCA territory were 22.8% and 40.0%., Conclusions: The OA-MCA bypass is an effective rescue therapy to improve the clinical condition and hemodynamic changes caused by PMMD in patients who experience recurrent symptoms after initial revascularization.

Background: Eloquent location of a brain arteriovenous malformation (BAVM) is known to increase the surgical risk. Surgical removal of such BAVMs is challenging. Useful indicators for the safe removal of eloquent BAVMs are needed. The aim of this study was to determine the surgical risk factors for these challenging entities., Methods: The authors retrospectively reviewed 29 motor and/or sensory BAVM patients who underwent surgeries. The risk factors for surgical morbidity were analyzed. As a new risk factor, maximum nidus depth, was evaluated., Results: Complete obliteration was achieved in 28 patients (96.6%). Postoperative transient and permanent neurological deteriorations were seen in nine patients (31.0%) and five patients (17.2%), respectively. In univariate analysis, maximum nidus depth (p = 0.0204) and asymptomatic onset (p = 0.0229) were significantly correlated with the total morbidity. In multivariate analysis, only maximum nidus depth was significantly correlated with total morbidity (p = 0.0357; odds ratio, 2.78598; 95% confidence interval, 0.8866-8.7535). The cut-off value for the maximum nidus depth was 36 mm for total morbidity (area under the curve [AUC], 0.7428) and 41 mm for permanent morbidity (AUC, 0.8833). The cutoff value of the maximum nidus size was 30 mm for total morbidity (AUC, 0.5785) and 30 mm for permanent morbidity (AUC, 0.7625). AUC was higher for the maximum nidus depth than it was for the maximum nidus size., Conclusions: Maximum nidus depth was significantly associated with surgical morbidity of eloquent BAVMs. The maximum nidus depth is a novel and a simpler indicator of the risk of surgical morbidity.

Objective: Mechanical thrombectomy for acute large vessel occlusion (LVO) is currently widely performed. However, rescue treatment (RT), such as percutaneous transluminal angioplasty (PTA) and stenting, is occasionally required, particularly in the case of atherothrombotic brain infarction (ATBI) or dissection. As RT requires higher levels of therapeutic skills and additional devices, early prediction of its performance and preparation are important. We retrospectively investigated the pre-therapeutic factors for predicting the necessity of RT., Methods: We reviewed 149 consecutive patients who underwent mechanical thrombectomy for acute LVO between April 2014 and December 2019. Eight patients were excluded because of missing clinical data. RT was performed when severe stenosis was observed in occluded vessels or proximal to them during mechanical thrombectomy. We investigated pre-therapeutic neurological, laboratory, and radiological findings in the 141 remaining patients, and compared them between RT and non-RT groups., Results: RT was performed on 23 of the 141 patients. We found four pre-therapeutic factors with significantly different rates between RT/non-RT as follows: (1) Atrial fibrillation 8.7%/71.1% (p <0.001), (2) diabetes mellitus 39.1%/19.5% (p = 0.04), (3) susceptibility vessel sign (SVS) by T2-weighted imaging 17.4%/66.1% (p <0.001), and (4) tapered occlusion by magnetic resonance angiography (MRA) 47.8%/11.9% (p <0.001). The plasma level of brain natriuretic peptide (BNP) was also significantly different between the two groups. When the BNP level was less than 70 pg/mL, the sensitivity for being in the RT group was 86.9% and the specificity was 83.5%., Conclusion: Pre-therapeutic findings, such as diabetes mellitus, tapered occlusion, absence of atrial fibrillation, negative SVS, and BNP level less than 70 pg/mL, are predictors of RT in mechanical thrombectomy., Competing Interests: The authors declare no conflict of interest., (©2021 The Japanese Society for Neuroendovascular Therapy.)

Introduction: Moyamoya disease (MMD) is a rare cerebrovascular disease associated with cerebral infarction or hemorrhage. Hyperperfusion is the most significant complication of direct bypass surgery. Previous research has shown that an increase in cerebral blood flow (CBF) is strongly related to symptomatic hyperperfusion and highlighted the importance of postoperative assessment of CBF., Objective: The principal aims of this study were to quantitatively analyze the relationship between intraoperative graft flow and increase in CBF and to evaluate the effectiveness of intraoperative graft flow measurement during bypass surgery for patients with MMD., Methods: This study included 91 surgeries in 67 consecutive adult patients with MMD who underwent direct revascularization surgery at our institution between November 2013 and September 2018. Intraoperative graft flow of the branches and main trunk was measured in all patients, after anastomosis had been established. Postoperative CBF measurements were performed under sedation, immediately after surgery. Radiological hyperperfusion was defined as focal high uptake, as determined by CBF imaging immediately after surgery. Patients were divided into two groups (radiological hyperperfusion and nonradiological hyperperfusion groups), and the relationship between intraoperative graft flow and radiological hyperperfusion was analyzed., Results: Significant differences were observed between the radiological hyperperfusion and nonradiological hyperperfusion groups in terms of intraoperative graft flow of both the branch (median 72 vs. 42 mL/min, respectively; p < 0.01) and main trunk (median 113 vs. 68 mL/min, respectively; p < 0.01). A receiver-operating characteristic analysis was performed to test the utility of intraoperative flow as a quantitative measure. We set the cutoff values for the intraoperative branch and main trunk flow at 57 mL/min (sensitivity: 0.707, specificity: 0.702; area under the curve [AUC]: 0.773; 95% confidence interval [CI]: 0.675-0.871) and 84 mL/min (sensitivity: 0.667, specificity: 0.771; AUC: 0.78; 95% CI: 0.685-0.875), respectively., Conclusions: Measuring intraoperative graft flow during bypass surgery may be an effective means of predicting hyperperfusion and could serve to facilitate early therapeutic intervention such as strict blood pressure control., (© 2020 The Author(s) Published by S. Karger AG, Basel.)