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We evaluated the effect of early surgical intervention on the change in memory performance of patients with low-grade brain tumors associated with epilepsy. Twenty-three adult patients with low-grade brain tumors and epilepsy who underwent surgery at our institution between 2010 and 2019 were included. The Wechsler Memory Scale-Revised (WMS-R) was used to assess cognitive memory performance. Memory performance before and after surgery was retrospectively evaluated. In addition, the relationships among preoperative memory function, postoperative seizure outcome, preoperative seizure control, temporal lobe lesion, and change in memory function were examined. There were statistically significant improvements from median preoperative to postoperative WMS-R subscale scores for verbal memory, general memory, and delayed recall (p0.001, p0.001, and p=0.0055, respectively) regardless of preoperative sores and tumor location. Good postsurgical seizure control was associated with significant improvements in postoperative WMS-R performance. Our results indicated that early surgical intervention might improve postoperative memory function in patients with low-grade brain tumors and epilepsy.

Introduction: Moyamoya disease is characterized by progressive stenotic changes in the terminal segment of the internal carotid artery and the development of abnormal vascular networks called moyamoya vessels. The objective of this review was to provide a holistic view of the epidemiology, etiology, clinical findings, treatment, and pathogenesis of moyamoya disease. A literature search was performed in PubMed using the term “moyamoya disease,” for articles published until 2021. Results: Artificial intelligence (AI) clustering was used to classify the articles into 5 clusters: (1) pathophysiology (23.5%); (2) clinical background (37.3%); (3) imaging (13.2%); (4) treatment (17.3%); and (5) genetics (8.7%). Many articles in the “clinical background” cluster were published from the 1970s. However, in the “treatment” and “genetics” clusters, the articles were published from the 2010s through 2021. In 2011, it was confirmed that a gene called Ringin protein 213 (RNF213) is a susceptibility gene for moyamoya disease. Since then, tremendous progress in genomic, transcriptomic, and epigenetic profiling (e.g., methylation profiling) has resulted in new concepts for classifying moyamoya disease. Our literature survey revealed that the pathogenesis involves aberrations of multiple signaling pathways through genetic mutations and altered gene expression. Conclusion: We analyzed the content vectors in abstracts using AI, and reviewed the pathophysiology, clinical background, radiological features, treatments, and genetic peculiarity of moyamoya disease.

Background The physiological and pathological significance of the arachnoid membrane (AM) is still unknown. In this study, we investigated various characteristics of the AM, focusing on the influence of inflammation and fibrosis. Methods Small pieces of AM sample were obtained during neurosurgical procedures from 74 cases. The clinical and pathological characteristics of the hyperplastic AM group (≥ 50 μm) and the non-hyperplastic AM group ( Results The median age of the patients in hyperplastic AM group was significantly older than that of the non-hyperplastic AM group. Moreover, the number of fibroblasts, CD68+ cells, CD86+ cells, and CD206+ cells in the hyperplastic AM group was significantly higher than that in the non-hyperplastic AM group. The AM thickness was significantly correlated to age and number of fibroblasts, CD68+ cells, CD86+ cells, and CD206+ cells. The thickness of the AM was significantly correlated to the messenger RNA expression levels of VEGFα (ρ = 0.337), and the VEGFα expression levels were significantly correlated with TGFβ and TNFα. Conclusions The AM hyperplasia was influenced by aging and could be a result of inflammation and fibrosis through cytokine secretion from the inflammatory cells and fibroblasts in the AM.

OBJECTIVEIn-stent restenosis after percutaneous transluminal angioplasty and stenting (PTAS) due to neointimal hyperplasia is a potential cause of clinical complications, including repeated revascularization and ischemic events. Neointimal hyperplasia induced by an inflammatory response to the stent strut may be a possible mechanism of in-stent restenosis. Intravenous infusion of bone marrow–derived mesenchymal stem cells (MSCs) has been reported to show therapeutic efficacy for cerebral stroke, presumably by an antiinflammatory effect. This study aimed to determine whether MSCs can reduce or prevent neointimal hyperplasia induced by an endovascular stent.METHODSIn this study, two types of bare metal stents were deployed using a porcine (mini-pig) model. One stent was implanted in the common carotid artery (CCA), which is considered quite similar to the human CCA, and the other was inserted in the superficial cervical artery (SCA), which is similar in size to the human middle cerebral artery. Angiographic images, intravascular ultrasound (IVUS) imaging, and microscopic images were used for analysis.RESULTSAngiographic images and IVUS studies revealed that intravenous infusion of MSCs immediately after deployment of stents prevented in-stent stenosis of the CCA and SCA. Histological analysis also confirmed that inflammatory responses around the stent struts were reduced in both the stented CCA and SCA in the mini-pig.CONCLUSIONSIntravenous infusion of MSCs inhibited the inflammatory reaction to an implanted stent strut, and prevented progressive neointimal hyperplasia in the stented CCA and SCA in a porcine model. Thus, MSC treatment could attenuate the recurrence of cerebral ischemic events after stenting.

Awake craniotomy has been widely performed in patients with glioma in eloquent areas to minimize postoperative brain dysfunction. However, neurological examination in awake craniotomy is sometimes problematic due to communication difficulties during the intraoperative awake period. We evaluated preoperative predictors of these difficulties in awake craniotomy for patients with glioma. In all, 136 patients with glioma who underwent awake craniotomy at our institution between January 2012 and January 2020 were retrospectively evaluated. Patients were divided into two groups (appropriately awake group and inappropriately awake group) depending on their state during the intraoperative awake period, and the relationship between communication difficulties in awake craniotomy and both clinical and radiological characteristics were assessed. The appropriately awake group included 110 patients, and the inappropriately awake group included 26 patients. Reasons for inclusion in the inappropriately awake group were insufficient wakefulness in 15 patients, restless state in 6, and intraoperative seizures in 5. In multivariate analysis, the likelihood of being inappropriately awake was inversely correlated with preoperative seizures (odds ratio [OR], 0.23; 95% confidence interval [CI], 0.06-0.89; p = 0.033) and positively correlated with left-sided lesions (OR, 7.31; 95% CI, 1.54-34.62; p = 0.012). Both lack of preoperative seizures and left-sided lesions were identified as risk factors for intraoperative difficulties in awake craniotomy for patients with glioma. Understanding these risk factors may lead to more appropriate determination of eligibility for awake craniotomy.

Magnetic resonance angiography (MRA) is an important tool in rat models of cerebrovascular disease. Although MRA has long been used in rodents, the image quality is typically not as high as that observed in clinical practice. Moreover, studies on MRA image quality in rats are limited. This study aimed to develop a practical high-spatial-resolution MRA protocol for imaging cerebral arteries in rats.We used the "half position method" regarding coil placement and modified the imaging parameters and image reconstruction method. We applied this new imaging method to measure maturation-related signal changes on rat MRAs.The new practical high-spatial-resolution MRA imaging protocol obtained a signal intensity up to 3.5 times that obtained using a basic coil system, simply by modifying the coil placement method. This method allowed the detection of a gradual decrease in the signal in cerebral vessels with maturation.A high-spatial-resolution MRA for rats was obtained with an imaging time of approximately 100 min. Comparable resolution and image quality were obtained using the new protocol with an imaging time of 30 min CONCLUSIONS: The new practical high-spatial-resolution MRA protocol can be implemented simply and successfully to achieve high image quality with an imaging time of approximately 30 min. This protocol will benefit researchers performing MRA imaging in cerebral artery studies in rats.

Moyamoya disease is a unique cerebrovascular disease that is characterized by progressive bilateral stenotic alteration at the terminal portion of the internal carotid arteries. These changes induce the formation of an abnormal vascular network composed of collateral pathways known as moyamoya vessels. In quasi-moyamoya disease, a similar stenotic vascular abnormality is associated with an underlying disease, which is sometimes an inflammatory disease. Recent advances in moyamoya disease research implicate genetic background and immunological mediators, and postulate an association with inflammatory disease as a cause of, or progressive factor in, quasi-moyamoya disease. Although this disease has well-defined clinical and radiological characteristics, the role of inflammation has not been rigorously explored. Herein, we focused on reviewing two main themes: (1) molecular biology of inflammation in moyamoya disease, and (2) clinical significance of inflammation in quasi-moyamoya disease. We have summarized the findings of the former theme according to the following topics: (1) inflammatory biomarkers, (2) genetic background of inflammatory response, (3) endothelial progenitor cells, and (4) noncoding ribonucleic acids. Under the latter theme, we summarized the findings according to the following topics: (1) influence of inflammatory disease, (2) vascular remodeling, and (3) mechanisms gleaned from clinical cases. This review includes articles published up to February 2019 and provides novel insights for the treatment of the moyamoya disease and quasi-moyamoya disease.

Cerebrovascular event after open-heart surgery is a critical complication and contributes to poor prognosis, including increased mortality. The purpose of this study is to investigate the appropriate preoperative risk assessment and monitoring for reducing the risk of ischemic stroke after open-heart surgery.184 patients who underwent surgery for valvular heart disease were included in this study. Near-infrared oxygen saturation monitoring (INVOS system) was performed during open-heart surgery. For the purpose of perioperative stroke risk assessment, we evaluated the clinical and radiological factors of the group that underwent preoperative consultation and the group that did not, and of the patients with and without postoperative ischemic stroke.Preoperative consultation was performed in 60 cases. Large vessel steno-occlusive disease was found in nine cases, of which three had undergone revascularization surgery. Cerebral infarction developed in four cases, all of which had no large vessel steno-occlusive disease. There was no significant association between the development of postoperative ischemic stroke and presence of large vessel steno-occlusive disease. Preoperative baseline INVOS value was significantly low in the ischemic stroke group (49.5 ± 12.5) compared to the non-ischemic stroke group (66.8 ± 10.0), (P = 0.012).In open-heart surgery for valvular heart disease, low preoperative baseline INVOS values were associated with cerebral ischemic stroke after surgery. The combination of appropriate preoperative screening for large vessel steno-occlusive disease and measurement of INVOS could be used as a simple and useful method in screening for the risk of ischemic stroke after open-heart surgery.

Objective The present study investigated the effects of the stimulus polarity and location of motor evoked potential (MEP) to establish a stimulation protocol. Methods Nineteen patients who intraoperatively underwent MEP in bipolar direct cortical stimulation were enrolled in the present study. Somatosensory evoked potentials (SEP) of the contralateral median nerve stimulation were recorded to determine stimulation sites. MEP was performed under two settings in all patients: 1. Anodal bipolar stimulation: an anode on the precentral gyrus and a cathode on the postcentral gyrus, 2. Cathodal bipolar stimulation: a cathode on the precentral gyrus and an anode on the postcentral gyrus. MEP amplitudes and the coefficient of variation (CV) at a stimulation intensity of 25 mA and the thresholds of induced MEP were compared between the two settings. Results An electrical stimulation at 25 mA induced a significantly higher amplitude in cathodal bipolar stimulation than in anodal bipolar stimulation. Cathodal bipolar stimulation also showed significantly lower thresholds than anodal stimulation. CV did not significantly differ between the two groups. Conclusions These results indicate that cathodal bipolar stimulation is superior to anodal bipolar stimulation for intraoperative MEP monitoring. Significance MEP in cathodal bipolar cortical stimulation may be used in a safe and useful evaluation method of motor fiber damage that combines sensitivity and specificity.

Objectives: Superficial temporal artery (STA)-middle cerebral artery (MCA) bypass operation is an effective treatment for patients with Moyamoya disease, and the hemodynamic parameters are reported to be improved after operation. However, there is no report concerning hemodynamic changes from the viewpoint of the preoperative anatomical structure of grafts. In this study, we evaluated the correlation between the preoperatively estimated blood flow of the graft obtained through image-based computational fluid dynamics (CFD) analysis and the hemodynamic changes in the acute phase after revascularization. Materials and methods: A total of 30 hemispheric sides of 23 patients were examined. The blood flow, that is, flow rate (FR) of the STA branches that were anastomosed to the MCA was evaluated using CFD analysis based on computed tomography (CT) angiography imaging data. The correlations between the FR and the hemodynamic changes in the acute phase after revascularization obtained through CT perfusion were assessed. Results: The preoperatively estimated FR of the graft was moderately correlated with the changes in the mean transit time significantly and weakly correlated with those in the cerebral blood flow and cerebral blood volume. In addition, the FR was strongly correlated with age and the diameter of the STA from the origin to the bifurcation.Conclusion: The preoperatively estimated FR of the graft obtained through image-based CFD analysis contributed to the improvement of the mean transit time after revascularization. Because the FR of the graft was associated with the diameter of the STA, the size of the STA might be an important factor in postoperative hemodynamic changes. This might lead to the risk assessment of acute drastic hemodynamic changes as cerebral hyperperfusion, and consequently, better surgical outcomes might be expected.

Background Despite rapid developments in devices used to treat arteriovenous malformation (AVM), a randomised trial of Unruptured Brain Arteriovenous malformations published in 2014 recommended conservative treatment for nonhemorrhagic AVM. The purpose of the current retrospective study was to confirm how AVM treatment in Japan has changed and to assess the safety of treatment for hemorrhagic and nonhemorrhagic AVMs. Methods We enrolled 242 consecutive patients with AVM; each patient’s treatment was selected and performed at our hospital. The type of onset, Spetzler–Martin (S–M) grade, age, sex, selected treatment, mortality, and morbidity were compared between the first and second periods of our study. Results In patients with grade I–III AVM, the selected treatment changed between the first and second periods; however, in grade IV and V patients, the selected treatment did not change. Overall, interventions by microsurgery alone decreased (p p = 0.005), interventions using stereotactic radiosurgery (SRS) alone increased (p = 0.009), and interventions including SRS increased (p = 0.002). Morbidity associated with intervention was 0.92% in the first period and 0% in the second period, and mortality was 0.92% in the first period and 1.67% in the second. Conclusions With the development of new devices, the selected treatment was changed in patients with S–M grade I–III AVM, but was not changed in patients with grade IV and V. The complication rate was low and did not change throughout the periods. These findings suggest that the safety of treatment depends on a full understanding of device development and the selection of proper treatment, not on hemorrhagic onset. Further treatment innovations are expected to change the treatment for grade IV and V AVMs.

Objective Grade 2 meningioma is more likely to recur than grade 1 meningioma. Recurrence decreases overall survival in patients with grade 2 meningioma. However, the clinical course of grade 2 meningioma with several repeated recurrences is poorly understood. The purpose of this study was to clarify the clinical characteristics of grade 2 meningioma with repeated recurrences. Methods This study included 28 patients with grade 2 meningioma treated at our institution from January 1994 to December 2017. The relationship between survival and factors including age, sex, number of recurrences, malignant transformation, radiation therapy, tumor location, MIB-1 labeling index, Simpson grade, Karnofsky Performance Status, and surgical interval were analyzed. Results The average age at the initial operation was 53.4 years. The number of recurrences was 3.7 times on average during the follow-up of 113.9 months after the initial operation. An increasing number of recurrences resulted in shortening of the surgical interval, increase in the MIB-1 labeling index, and decrease in Karnofsky Performance Status. In fatal cases, the average surgical interval before death was approximately 1 year. Three factors were related to poor prognosis: number of recurrences (odds ratio, 1.620; P = 0.030), malignant transformation (odds ratio, 10.625; P = 0.019), and high MIB-1 labeling index (odds ratio, 1.089; P = 0.044). Conclusions Shortening of the surgical interval within 1 year because of multiple recurrences led to death in patients with grade 2 meningioma. Malignant transformation was the most potent among the poor prognostic factors.

Postoperative visual function is a major concern in transsphenoidal surgery (TSS). Although several reports have demonstrated the importance of visual evoked potential (VEP) monitoring during TSS, the usefulness of VEP monitoring have been controversial because of its reproducibility. Efficacy of VEP was analyzed in 20 consecutive cases of patients who underwent endoscopic endonasal TSS surgery. We adapted a high-power light-emitting diode stimulator with electroretinography using venous anesthesia. In addition, we used black shield patch and braided codes to obtain reproducible VEP amplitudes. Stable and reproducible VEP waveforms were obtained in 38 of 39 eyes (97.4%) before surgery. Fifteen eyes had deteriorated VEP amplitude during operation, and nine eyes had improved VEP amplitude at the end of surgery, and six eyes had not improved VEP amplitude. But no postoperative visual impairment was observed in all cases by temporary halting the surgical manipulation when the VEP was deteriorated. In conclusion, VEP monitoring could be a warning sign to avoid postoperative visual dysfunction. We recommend VEP as a routine monitoring in TSS.

We report a case of moyamoya disease (MMD), which developed after non-herpetic acute limbic encephalitis (NHALE) associated with anti-leucine-rich glioma-inactivated 1 (LGI1) antibody. The patient's mother had a history of MMD. No vascular lesions were identified at the time of the NHALE. Nine years later, the patient visited our hospital due to memory disturbances and repeated transient ischemic attacks affecting the right limb. Diffusion-weighted magnetic resonance imaging revealed scattered areas of signal hyperintensity, and the patient was ultimately diagnosed with MMD based on angiography. Revascularization surgery was performed on the left side, where cerebral blood flow was impaired on 123I-N-isopropyl-p-iodoamphetamine single photon emission computed tomography. Postoperatively, the patient was discharged with a normal neurological examination. NHALE associated with LGI1 antibodies is an autoimmune disease. Although autoimmune disease is the most frequent finding other than atherosclerosis in quasi-MMD, this is the first report of NHALE associated with anti-LGI1 antibodies mimicking quasi-MMD. Inflammation and angiogenesis may contribute to the development of MMD, in addition to genetic background.

Extracranial-to-intracranial (EC-IC) bypass surgery may be necessary in patients with moyamoya disease and other ischemic conditions. However, there is a potential risk of wound-related complications in some cases. In this study, we report our approach to the prevention of wound-related complications in EC-IC bypass. Technical considerations and pitfalls of surgery are also discussed. This study included 89 patients with ischemic-onset moyamoya disease and atherosclerotic disease who underwent 108 superficial temporal artery (STA)-to-middle cerebral artery bypass procedures. Our study emphasized 3 major features. First, 3-dimensional simulation imaging was used to confirm STA anatomy. Second, the STA was meticulously dissected on the epigaleal layer to protect the galeal layer. Third, scalp skin ischemia was taken into consideration at each step until skin closure. There was no neurologic morbidity or mortality in this series. There were 2 cases of major wound-related complications requiring plastic surgical intervention, and 4 cases of minor complications that were treated conservatively. In major complication cases, the scalp defect was treated with pedicle flap reconstruction. In EC-IC bypass surgery, interdisciplinary management involving neurosurgery, plastic surgery, and radiology should reduce wound-related complications and achieve safe surgery.

In specific cases of moyamoya disease (MMD), posterior cerebral artery (PCA) stenosis can develop after treatment of the anterior circulation and require additional revascularization. Here, we report two cases that underwent additional posterior indirect revascularization with multiple burr holes for PCA involvement after bilateral revascularization treatment of the anterior circulation. They presented with transient ischemic attack even after bilateral superficial temporal artery-middle cerebral artery bypass, and magnetic resonance angiography (MRA) showed that PCA stenosis had worsened. Indirect revascularization with multiple burr holes using Benz-marked skin incisions was performed. After surgery, the symptoms improved without perioperative complications, and cerebral angiography showed collateral circulation via the burr hole. Indirect revascularization for MMD is often combined with direct revascularization, and there are only a few reports on the use of multiple burr hole surgery alone. In addition, there are few reports of posterior circulation, despite the emphasis on the importance of PCA involvement in MMD. Indirect revascularization with multiple burr holes alone can be performed in multiple areas and applied to patients who cannot undergo direct revascularization using the occipital artery. The procedure is simple and less invasive than traditional direct revascularization procedures. Therefore, it can be effective, especially in pediatric cases of MMD with PCA involvement.

Background Dilatation of the microvascular diameter is recognized in moyamoya disease and referred to as microvascularization. The purpose of this study was to characterize the cortical microvascularization in moyamoya disease using imaging analysis, and to explore the developmental mechanism of the collateral network around the cortical surface. Methods A total of 20 hemispheric sides of 14 patients with moyamoya disease were included in this study. From the intraoperative images, cortical surface images were extracted, and binary images were subsequently created. Then the ratio of the microvessels of the brain surface (vascular fraction; VF) and the box-counting fractal dimension (Db) values were calculated. The VF and Db values in the moyamoya disease group were then compared with those in atherosclerotic disease and nonischemic disease groups, and assessed in terms of clinical and radiologic factors. Results VF was significantly higher in the moyamoya disease group compared with the atherosclerotic disease group, and Db was significantly higher in the moyamoya disease group compared with the atherosclerotic disease and nonischemic disease groups. In the moyamoya disease group, VF showed a moderate correlation with magnetic resonance angiography (MRA) score. Moreover, Db was significantly higher in the pediatric patients, in the presence of ischemic symptoms, and in the presence of ivy sign, and Db showed a moderate correlation with MRA score and cerebral blood flow in moyamoya disease. Conclusions In the patients with moyamoya disease, the cortical microvascularization exhibited increased Db and dilatation of the pial arteries. In moyamoya disease, cortical microvascularization is associated with clinical and radiologic factors. This microvascularization might be a compensatory mechanism in the ischemic condition in moyamoya disease.

Background One of the important aims of surgery for moyamoya disease is to establish indirect revascularization. The purpose of this study was to assess the progress of the middle meningeal artery (MMA) after our novel preservation method and to evaluate the relation between direct and indirect bypass in the chronic stage. Methods A total of 24 hemispheric sides of 19 patients with moyamoya disease were included in this study. Craniotomy was performed with preservation of the MMA during the procedure, then direct bypass was carried out. The relationship between anatomic variations of the MMA and success rate of preserving the MMA during craniotomy was noted. The alteration of the MMA and superficial temporal artery (STA) diameters was then evaluated using magnetic resonance imaging, and the correlation between the MMA and the STA in the chronic stage was examined. Results In total, the MMA was preserved during craniotomy in 20 hemispheric sides (83.3%). During the 3-year follow-up period, the MMA and STA diameters were significantly increased. At 3 years after surgery, the alteration of the MMA diameter was significantly more marked in pediatric cases than in adult cases, and MMA diameter was moderately but significantly negatively correlated with STA diameter. Conclusions In moyamoya disease, the MMA could be developed as a pathway for indirect revascularization even after simple preservation, especially in pediatric patients. The progress of the MMA and the STA occurs through their synergistic interaction, and the balance might be decided based on their complementary relations in the chronic stage.

Although the use of titanium implants in cranioplasty has recently become common, long-term results of this strategy are sometimes difficult to manage. The aim of this study was to clarify the characteristics of patients developing exposure of titanium implants. Records of eight patients with exposure of titanium implants were reviewed. Patient ages ranged from 41 to 77 (mean, 56.8) years. Two patients were male, and six were female. The periods from implantation to exposure of titanium implants ranged from 5 to 87 (mean, 30.5) months. In seven cases, titanium mesh cranioplasty was performed after postcraniotomy infections. The titanium implants were removed in all cases. Scalp tissue was closed by applying a primary closure technique in five cases, though scalp reconstruction was performed with a rotation flap in the other three cases involving large scalp ulceration. Although the use of titanium implants is convenient, the indications should be carefully considered, especially in cases with postoperative surgical site infections and in patients with thin scalp skin. For these patients, long-term follow up is necessary.

OBJECTIVEMedullary streaks detected on fluid-attenuated inversion recovery (FLAIR) imaging have been considered to be reflected ischemic regions in pediatric moyamoya disease. The purpose of this study was to evaluate these medullary streaks both clinically and radiologically and to discuss associated pathophysiological concerns.METHODSThe authors retrospectively reviewed data from 14 consecutive pediatric patients with moyamoya disease treated between April 2009 and June 2016. Clinical and radiological features and postoperative imaging changes were analyzed. In 4 patients, hyperintense medullary streaks on FLAIR imaging (HMSF) at the level of the centrum semiovale were detected.RESULTSThe HMSF were coincident with hyperintense medullary streaks on a T2-weighted image, though they were not completely coincident with the vasculature on either a T2*-weighted image or contrast-enhanced CT. Analysis revealed significantly higher values in terms of MR angiography scores, number of flow voids of the basal ganglia, and the presence of the medullary artery in the group with HMSF than in those without. In contrast, the presence of white matter damage was significantly less frequent in the HMSF group. All HMSF disappeared after surgery, and the mean apparent diffusion coefficient at the same level was significantly reduced postoperatively.CONCLUSIONSAlthough HMSF should be associated with collateral circulation in moyamoya disease, other factors may be involved, including stagnated cerebrospinal fluid or vasogenic edema that is relevant to the impaired state of the white matter. Findings in this study provide insight into the pathophysiological basis of the perivascular space in moyamoya disease.

Meandering flow void around the splenium, which can be recognized on conventional T2-weighted images, implicates collateral flow in the splenial artery in patients with moyamoya disease. In this report, curves of flow voids around the splenium (SFVs) were evaluated in patients with moyamoya disease, and their diagnostic value and pathophysiology were verified.A total of 65 consecutive patients with moyamoya disease were included in this analysis. The number of SFV curves was counted on each side. The numbers of SFV curves in patients with moyamoya disease was compared with those in the control group. The clinical features of patients with large numbers of flow voids were analyzed.The mean number of SFVs was significantly higher in the moyamoya disease group than in the control group or the atherosclerotic disease group. The cut-off SFV count for a diagnosis of moyamoya disease should be set to 2.25 in consideration of receiver operating characteristic curve analysis. A multivariate logistic analysis of SFV data in moyamoya disease revealed significant differences between the high SFV count group (≥2.25) and the low SFV count group (2.25) in age, flow voids in the basal ganglia, and magnetic resonance angiography score of the internal carotid artery and anterior cerebral artery.Based on this study, number of SFV curves on conventional T2-weighted imaging could be a useful measurement for confirming a diagnosis of moyamoya disease. Increased number of SFV curves was associated with age and progress of disease.

Objectives The acetazolamide challenge test in conjunction with 123I-IMP single-photon emission computed tomography (SPECT) is a known method of assessing cerebrovascular reserve capacity. In this study, we investigated whether CT perfusion in combination with resting state 123I-IMP SPECT could be used instead of the acetazolamide challenge test to evaluate hemodynamic compromise in patients with atherosclerotic occlusive disease. Methods Twenty consecutive patients with unilateral internal carotid artery or middle cerebral artery steno-occlusive disease were enrolled. 123I-IMP SPECT was performed with and without the acetazolamide challenge test, and with CT perfusion. Cerebral blood flow (CBF), cerebral blood volume, and mean transit time (MTT) obtained by CT perfusion were compared with CBF and cerebrovascular reactivity (CVR) obtained by 123I-IMP SPECT. Results The asymmetry ratio of MTT as measured by CT perfusion showed a strong correlation with the CVR to acetazolamide as measured by 123I-IMP SPECT (ρ = −.780, P Conclusion MTT as measured by CT perfusion in combination with CBF as measured by resting state 123I-IMP SPECT may be useful for evaluating hemodynamic compromise as an alternative to the acetazolamide challenge test.

A nearly 80-year-old man with a history of radiotherapy (RT) and total laryngectomy for laryngeal cancer 25 years previously presented with a rapidly growing mass on the right side of his neck. A huge pseudoaneurysm (pseudoAN) was detected at the right carotid bifurcation, though angiography performed four years previously had shown total occlusion of the internal carotid artery. Stent-assisted coil embolization enabled aneurysm sac shrinkage. Clinicians must be aware that the stump of an arterial occlusion associated with RT can change into a pseudoAN over the long term and must provide follow-up in such cases.

Background Glossopharyngeal neuralgia (GPN) and trigeminal neuralgia (TN) can result from mechanical stimulation of the glossopharyngeal nerve (GPNv) and trigeminal nerve (TNv) by blood vessels. TN can cause severe pain in the orofacial region, whereas GPN manifests as pain in the tongue, throat, tonsil, and ear. Although these 2 neuralgias can occur concurrently, concurrence of recurrent TN and GPN that develops postoperatively has not been previously described. Case Description A 68-year-old male complained of right glossalgia and pain in the pharynx radiating to the right auricular area. The patient had previously undergone microvascular decompression (MVD) for right TN. Medication and intraoral xylocaine spray did not relieve the symptoms. An oral surgeon was unable to find any disease related to the glossalgia. The anesthesiologist pointed out that the symptoms could be from partial recurrence of the TN because the patient also complained of pain in the inferior alveolus. Magnetic resonance angiography indicated that the right GPNv seemed to be compressed by the right posterior inferior cerebellar artery (PICA); hence, MVD for both GPN and TN was performed. Intraoperatively, the right PICA was found to be adherent to the GPNv because of the thickened arachnoid membrane and was subsequently detached. The TNv was also examined, but only a Teflon ball was found, which was detached from the TNv. The GPN disappeared postoperatively, although TN persisted after the second operation. Conclusions GPN can result from adhesions between the GPNv and arachnoid membrane following previous MVD.

Segmental arterial mediolysis(SAM)is a rare non-inflammatory and non-atherosclerotic arteriopathy associated with the occurrence of multiple aneurysms such as intracranial and intraperitoneal aneurysms. We report a case of intraperitoneal hemorrhage that occurred during the acute stage of subarachnoid hemorrhage(SAH). An 82-year-old woman presented with a sudden onset of loss of consciousness with a diagnosis of SAH. Digital subtraction angiography demonstrated two consecutive vertebral artery-posterior inferior cerebellar artery aneurysms. The larger aneurysm, which seemed to be ruptured, was successfully treated by coil embolization. On the 9th day after the onset of SAH, she developed aphasia secondary to the cerebral vasospasm. After selective intra-arterial infusion of fasudil hydrochloride, she was observed to maintain elevated systolic blood pressure. Her aphasia improved; however, on the 14th day, she suddenly developed hemorrhagic shock. An abdominal computed tomography scan demonstrated intraperitoneal hemorrhage secondary to a ruptured fusiform aneurysm of the right gastroepiploic artery. The lesion was successfully treated by coil embolization, although she became bedridden. Although a histopathological examination was not performed, her clinical, radiological, and serological presentation met the criteria of the clinical diagnosis of SAM. Elevated systolic blood pressure and excessive release of catecholamines in the acute stage of SAH might have caused the intraperitoneal hemorrhage. Non-saccular ruptured intracranial aneurysms should be considered among the differential diagnoses of SAM. In such cases, identifying and monitoring intraperitoneal aneurysms might be useful for earlier diagnosis and treatment of SAM, especially in the acute stage after SAH.

Background The large number of people infected with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has plunged the world into fear in recent times. In Japan, 18,769 novel coronavirus disease 2019 (COVID-19) cases have been reported as of June 30, 2020. This study aimed to assess whether cluster infection prevention is possible by evaluating the association between viral transmission and meteorological factors. Methods This study included 1263 people who were successively diagnosed with COVID-19 in Hokkaido, Japan between January 24, 2020 and June 30, 2020. After obtaining the values from the Japanese Meteorological Agency, the average scores of air temperature and humidity were calculated and compared with COVID-19 reproduction numbers, and the association between COVID-19 incidence or reproduction number and meteorological factors was assessed. Results The COVID-19 reproduction number in Hokkaido had three peaks that came several days before the surge in COVID-19 cases. The peaks are indicative of cluster infections. There was a strong negative correlation between the kinematic viscosity of atmospheric air and the reproduction number. Discussion and Conclusion Analysis of the reproduction number is important for predicting or suppressing COVID-19 infection clusters. The authors found a strong association between meteorological factors, such as kinematic viscosity of atmospheric air and the incidence of COVID-19 infection. Meteorological forecasts could provide foreknowledge about COVID-19 infection clusters in the future.

Meningioma is a hypervascular tumor of the central nervous system. Angiographic disappearance of tumor blush after preoperative feeder embolization allows qualitative, but not quantitative, assessment of flow reduction. Pseudocontinuous arterial spin labeling (PCASL), which has evolved from magnetic resonance imaging techniques, allows noninvasive measurement of cerebral blood flow (CBF) using water protons in the arterial blood flow.We applied PCASL for assessment of blood flow in meningioma and its reduction on preoperative embolization.Forty-one consecutive patients (11 males, 30 females) with histologically proven meningioma were evaluated by PCASL. Quantitative assessment by an absolute value of tumor blood flow (TBF) and a relative value of tumor vascular index (tVI; calculated as TBF divided by CBF) were calculated. In 8 cases, in which preoperative embolization was achieved, flow reduction rate was evaluated.TBF of meningiomas, 155.8 mL/100 g·min(-1) on average, was 2.6 times higher than CBF, 59.9 mL/100 g·min(-1) (P0.001). Patients who underwent feeder embolization showed statistically greater flow reduction rate, which was calculated as 42.7% (P0.05). Mean tVI before embolization was 4.1, which was reduced to 2.1 after embolization.PCASL could yield quantitative assessment of blood flow in meningioma including flow reduction rate in cases of feeder embolization.

Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis. It is characterized by multiple xanthogranulomatous masses throughout the body, predominantly in the tibia. One of the characteristic radiological findings of the lesions associated with ECD is a "coated artery," which is often observed in the aorta. Although approximately one-fourth of ECD cases involve the central nervous system (CNS), an intracranial-coated artery has only been reported in four cases. We report a case of ECD that involves the CNS and has the unique appearance of a coated vertebral artery (VA). These tumors entirely encase the bilateral VAs without stenosis and are attached to the dura. Cranial magnetic resonance imaging also showed multiple extra-axial tumors in the cavernous sinus, the frontal convexity, and the orbital cavity. Further investigation revealed additional extracranial lesions around the cervical carotid artery, at the bilateral tibia, and at the elbow joint. A biopsy of the cervical and tibial lesions confirmed ECD. Steroid therapy resulted in a month-long improvement of preoperative symptoms. However, the patient's condition gradually progressed and he died of pneumonia 1 year after ECD diagnosis. The encasement of the intracranial artery by the tumor without stenosis and the dural attachment suggest ECD, which requires whole body investigation.

Objective A 3-dimensional temporal bone model for skull base surgical training was reconstructed via the use of a selective laser sintering technique, which is one of the 3-dimensional printing technologies. Methods The temporal bone model was created in 2 pieces to remove powder material in the mastoid air cells and to place dye into the semicircular canal and the Fallopian canal. Results The powder material was minimal, and the decisive structures were identified in color. Conclusions This artificial model will pave the way to a “new era” in surgical training and medical education.

Decreased cortical and subcortical vascular signals in gradient echo T2* weighted imaging have been reported in acute stroke due to major artery occlusion. The purpose of this study was to evaluate this cortical and subcortical vascular hypointensity (CSVH) in patients with moyamoya disease.Subjects were 20 consecutive patients with moyamoya disease. The numbers of CSVH in each hemisphere were counted and the numbers were compared between patients with moyamoya disease and controls. The distribution of CSVH, clinical features of cases exhibiting large numbers of CSVH and post-operative changes were analysed.Patients with moyamoya disease had significantly more CSVH in the middle cerebral artery territory (p 0.001) and the anterior cerebral artery territory (p 0.001) compared with controls. The number of CSVH was correlated significantly with regional cerebral blood flow, as shown through simple regression analysis (R = 0.461, p = 0.006), and the numbers of CSVH were significantly higher in patients with higher magnetic resonance angiography scores (p = 0.017). A given patient's total number of CSVH per hemisphere was significantly decreased after surgery (p = 0.018).CSVH on T2* WI may be a useful tool for diagnosing and evaluating the extent of moyamoya disease. Our small series study shows that revascularization surgery can decrease the number of CSVH.

Objectives: Balloon test occlusion (BTO) is performed to evaluate ischemic tolerance for large and giant cerebral aneurysms and head and neck tumors that may require parent artery occlusion. However, ischemic tolerance for the temporary test occlusion does not always guarantee a tolerance for permanent occlusion. In this study, we evaluated the utility of computed tomography (CT) perfusion during BTO to quantify ischemic tolerance for detecting delayed ischemic stroke. Materials and Methods: Forty-one patients who underwent BTO for the internal carotid artery were included. The correlations between the parameters of CT perfusion and collateral angiographic appearance or stump pressure during BTO were evaluated. The cerebral blood flow (CBF), cerebral blood volume, mean transit time (MTT), and time to peak (TTP) were obtained through CT perfusion, and the asymmetry ratios were determined. Collateral angiographic appearances were categorized into 5 grades (0-4). Results: The collateral angiographic appearance showed moderate correlations with CBF, MTT, and TTP that was significant. Of these, the absolute value of the correlation coefficient was the highest for MTT. MTT also showed a moderate correlation with stump pressure. CBF and MTT were significantly different between the poor collateral group (grades 2 and 3) and the good collateral group (grade 4). Based on the MTT, the good collateral group was identified with high sensitivity (75.0%) and specificity (81.2%). Conclusions: In BTO, the MTT obtained through CT perfusion showed a correlation with collateral angiographic appearance and stump pressure. Thus, the MTT might be useful to quantify ischemic tolerance for detecting delayed ischemic stroke.

Extracranial-to-intracranial (EC-IC) bypass surgery is an effective treatment for patients with moyamoya disease and other conditions. Some patients with moyamoya disease have a risk of acute thrombogenesis at the anastomotic site just after bypass surgery. The purpose of this study was to study risk factors of acute thrombogenesis and determine effective countermeasures. This study included 48 patients (66 EC-IC bypass procedures) with moyamoya disease and 52 controls (54 procedures) without moyamoya disease. The development of acute thrombogenesis was compared between the moyamoya disease and control groups. In the moyamoya disease group, clinical and radiological characteristics were assessed with respect to acute thrombogenesis. In the patients with acute thrombogenesis, causes of technical problems were retrospectively examined. The incidence of acute thrombogenesis was significantly higher in the moyamoya disease group than those in the control group. In the moyamoya disease group, acute thrombogenesis was observed in seven patients. In the moyamoya disease group, the magnetic resonance angiography (MRA) scores were significantly higher in patients with acute thrombogenesis than those in the patients without acute thrombogenesis. In the multivariate analysis, the predictive factor of acute thrombogenesis in moyamoya disease was a high MRA score (odds ratio, 2.336; p = 0.009). During EC-IC bypass surgery for moyamoya disease, acute thrombogenesis should be considered to obtain a high patency rate, particularly in patients with high MRA scores. Acute thrombogenesis will not influence morbidity if proper countermeasures are followed; therefore, the prediction and recognition of white thrombus are important for a successful bypass surgery.

This case report describes the usefulness of delay alternating with nutation for tailored excitation (DANTE)-prepared, contrast-enhanced magnetic resonance imaging (CE-MRI) for detecting the rupture site of an arteriovenous malformation (AVM). A ruptured intranidal aneurysm was confirmed histopathologically. Accurate non-invasive information about the possible rupture site of an AVM is critical for optimal treatment and evaluation. Vessel wall enhancement visualized by DANTE-prepared CE-MRI may be a useful tool for providing information about changes in inflammatory status and vulnerability to further developments.

Background Microvascular anastomosis is an essential procedure in neurosurgery, but the opportunity to perform the surgery has gradually decreased for neurosurgeons. Therefore, training is necessary for obtaining and maintaining the skills required for the procedure. We describe the detailed anatomy of cervical and femoral regions in rats and discuss the advantages for practicing microvascular anastomosis. Methods Cervical regions of Sprague-Dawley rats were dissected under intraperitoneal anesthesia. The step-by-step anatomic description was documented using a high-resolution charge-coupled device image sensor and recording systems. Using this model, temporal occlusion time and patency were measured, and these measures were compared between the trainee and trainer groups. The number of times the training needs to be completed to attain competency in the bypass procedure was estimated. Results After exposing the carotid triangle, a half-ring was created by end-to-side anastomosis. Anastomosis was performed at the common carotid artery using the contralateral side of the carotid artery as a graft. The cutoff value for the temporal occlusion time was 79.3 minutes in the receiver operating characteristic curve based on a target temporal occlusion time for beginners determined during the training. Conclusions Using a living animal model, a trainee has the opportunity to learn not only anastomotic techniques but also hemostatic control as well as overcoming mental strain during surgery. Living animal models are important in training because the fidelity of a living animal model is superior to nonliving models. Applying training using a half-ring model contributes to safe and efficient surgery.

A positive (outward) remodelling pattern in patients with typical atherosclerotic disease was recognised using magnetic resonance imaging (MRI). In this report, the outer diameter of the circle of Willis was evaluated in patients with moyamoya disease, and its remodelling pattern was verified.A total of 18 consecutive patients with moyamoya disease were included in this analysis. The diameter of the terminal portion of the internal carotid artery (ICA) (C1), proximal portion of the middle cerebral artery (MCA) (M1) and proximal portion of the anterior cerebral artery (A1) was evaluated using fast imaging employing steady-state acquisition (FIESTA) imaging. The outer diameter was estimated using these images, and compared with the control group, and the correlation with magnetic resonance angiography (MRA) scores was calculated for patients with moyamoya disease.The mean diameter of the moyamoya patient group on FIESTA was significantly lower than that of the control group in the C1, M1 and A1 (P 0.0001). The M1 diameter on the FIESTA image showed a strong correlation with the moyamoya disease MRA score (y = (0.53x+2.31; R = 0.72), whereas the C1 diameter on the FIESTA image showed only a weak correlation (y = (0.16x+2.47; R = 0.21).We found that the outer diameter of all the vessels around the C1 was generally reduced in patients with moyamoya disease. Diameter of the MCA especially decreases as the disease progresses, similar to the negative (constrictive) remodelling pattern.

Background Meningiomas or solitary fibrous tumors arising from the cavernous sinus (CS) are usually treated with radiosurgery to control growth. Surgical removal of cavernous tumors is indicated only for tumors extending outside the CS. However, even after adequate treatment, the tumor may exhibit recurrence or malignant transformation. We report a treatment option for recurrent meningeal tumors of the CS. Methods In 4 patients with CS tumors that exhibited regrowth after multiple operations and radiosurgery, radical removal in combination with high-flow bypass was performed, and a vascularized muscle flap was used for reconstruction. One patient had a radiation-induced atypical meningioma, 2 patients had transformed atypical meningiomas, and 1 patient had a frequently recurring solitary fibrous tumor. Results No local recurrence was observed in any patients during a follow-up period of 13–41 months. In all patients, a Karnofsky performance scale score of >80 on admission was maintained at >70 at the final follow-up evaluation. Conclusions Radical removal in combination with high-flow bypass provides favorable results and maintains quality of life in patients with recurrent CS meningeal tumors.

Recent advances in computed tomography angiography (CTA) enable repeated imaging follow up for post-clipping surgery. The purpose of this study was to clarify the critical volume and configuration of the aneurysmal clip in the postoperative evaluation using volume rendering (VR) imaging, and present four-dimensional (4D)-CTA for these larger metal artifacts. A total of 44 patients with cerebral aneurysm, treated using clipping surgery, were included in this study. The metal artifact volume was assessed using CTA and the association between the type of clips and its metal artifact volume was analyzed. A VR image and a 4D-CTA were then produced, and the diagnostic accuracy of arteries around the clip or residual aneurysm on these images was evaluated. In the receiver operating characteristic (ROC) curve analysis, the cutoff value for metal artifacts was 2.32 mm3 as determined through a VR image. Patients were divided into two groups. Group 1 included patients with a simple and small clip, and group 2 included patients with multiple, large or fenestrated clips. The metal artifact volume was significantly larger in group 2, and the group incorporated the cutoff value. Post-clipping status on the VR image was significantly superior in group 1 compared with group 2. In group 2, the imaging quality of post-clipping status on 4D-CTA was superior in 92.9% of patients. The metal artifact volume was dependent on the number, size, or configuration of the clip used. In group 2, evaluation using a 4D-CTA eliminated the effect of the metal artifacts.

Background Preoperative evaluation of malignancy in gliomas is important for surgical planning, particularly to determine whether a 1,3-bis-2-chloroethyl-1-nitrosourea wafer should be placed into the tumor cavity. In some cases, the intraoperative pathologic diagnosis of World Health Organization grade differs from the final diagnosis. Supplemental methods in addition to the routine contrast tomography or magnetic resonance imaging sequences may provide a more accurate preoperative diagnosis. Because tumor vascularity has been useful in distinguishing between low- and high-grade gliomas, we evaluated the accuracy of the arterial spin labeling (ASL) method, which could measure the cerebral blood flow (CBF) without using contrast medium, to determine the malignancy of gliomas. Methods This study included 102 patients with glioma (grade II, n = 40; grade III, n = 18; grade IV, n = 44). All patients underwent ASL to determine the tumor blood flow (TBF) and CBF in the middle cerebral region. The relative tumor vascular index (tVI), which is calculated as TBF divided by CBF in the contralateral middle cerebral region, was used to avoid dispersion of the absolute TBF value. Results tVI was significantly greater (1.46 ± 0.751) in high-grade gliomas than in low-grade gliomas (1.05 ± 0.343) (P = 0.003). As for each grade, tVI was statistically higher in grade IV than in grade II (P = 0.03) gliomas. Conclusions The noninvasive ASL method provides additional information to distinguish high-grade glioma from low-grade gliomas without using contrast medium.

Background Enlarged perivascular spaces (EPVS) are often observed with magnetic resonance imaging in patients with small vessel disease. However, the risk factors, radiological features, and clinical relevance of EPVS in patients with moyamoya disease are poorly understood. The purpose of this study was to evaluate EPVS, the risk factors of many EPVS, and the pathophysiology of EPVS in adult patients with moyamoya disease. Methods One hundred cerebral hemispheres of 50 adult patients with moyamoya disease were examined. The control group consisted of 50 age/sex-matched patients without ischemic disease. The numbers of EPVS at the level of the centrum semiovale per hemisphere were compared between the moyamoya disease and control groups. In each hemisphere, the total numbers of EPVS were categorized into five grades (0–4), and the clinical and radiological characteristics of the predictive factors in patients in the high EPVS grade group (EPVS grade = 4) were assessed. Results The EPVS counts and grades were significantly higher in the moyamoya disease group. Analyses of the background characteristics of the patients with moyamoya disease revealed that significantly higher prevalence of high EPVS grades were associated with the female sex, hypertension, high magnetic resonance angiography scores, high numbers of flow voids in the basal ganglia, high brain atrophy scores, ivy signs, and white matter lesions. A logistic multivariate analysis of the patients with high EPVS grades revealed significant associations with the female sex, hypertension, and flow voids in the basal ganglia. Conclusions Increased EPVS were confirmed in adult patients with moyamoya disease, and the associated clinical and radiological factors were identified. The presence of hypertension, the female sex, and flow voids in the basal ganglia were important for predicting high EPVS grades in patients with moyamoya disease. Reductions in arterial pulsations with steno-occlusive changes can inhibit the flow of interstitial fluid, which can increase the number of EPVS in patients with moyamoya disease. Other clinical factors, such as the female sex and hypertension, may promote secondary brain damage in patients with moyamoya disease. Further evaluations of EPVS in patients with moyamoya disease are needed to better understand their pathophysiological importance.

Background SERPINA1 plays an anti-inflammatory role in protecting tissues from proteolytic mechanisms. SERPINA1 is positive in gliomas by immunohistochemical analysis; however, the role of SERPINA1, including the relationship with prognosis, has been uncertain. In recent years, digital polymerase chain reaction (PCR) has provided ultra-sensitive assessment of messenger RNA expression from formalin-fixed paraffin-embedded (FFPE) tissues. Objective In this study, we quantitatively determined the expression of SERPINA1 in high-grade gliomas (HGGs) using digital PCR, and we analyzed its relationship with prognosis. Methods Twenty-nine FFPE surgical samples from patients with HGGs (7 of World Health Organization [WHO] grade III and 22 of WHO grade IV), and human glioblastoma cell lines, U87 and U118, were used for analysis. A qualitative assessment using immunostaining and quantitative assessment using digital PCR were performed to assess the expression of SERPINA1. Results The expression of SERPINA1 was demonstrated in glioma tissues and glioblastoma multiforme cell lines by immunostaining. Digital PCR analysis showed that SERPINA1 was expressed in 14.3% and 63.6% of the tissues from patients with grade III and grade IV HGG, respectively (P = 0.035). The median overall survival of 38.8 months in the low SERPINA1 expression group was longer than that of 15.3 months in the high expression group (P = 0.030). Conclusions The frequency and the amount of SERPINA1 expression were higher in grade IV than in grade III HGGs. The high expression of SERPINA1 indicates a poor prognosis of HGGs.