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Clinical implications of RNF213 genetic variants, other than p.Arg4810Lys, in moyamoya disease (MMD), remain unclear. This study aimed to investigate the association of RNF213 variants with clinical phenotypes in MMD. This retrospective cohort study collected data regarding the clinical characteristics of 139 patients with MMD and evaluated the angioarchitectures of 253 hemispheres using digital subtraction angiography at diagnosis. All RNF213 exons were sequenced, and the associations of clinical characteristics and angiographical findings with p.Arg4810Lys, p.Ala4399Thr, and other rare variants (RVs) were examined. Among 139 patients, 100 (71.9%) had p.Arg4810Lys heterozygote (GA) and 39 (28.1%) had the wild type (GG). Fourteen RVs were identified and detetcted in 15/139 (10.8%) patients, and p.Ala4399Thr was detected in 17/139 (12.2%) patients. Hemispheres with GG and p.Ala4399Thr presented with significantly less ischemic events and more hemorrhagic events at diagnosis (p = 0.001 and p = 0.028, respectively). In asymptomatic hemispheres, those with GG were more susceptible to de novo hemorrhage than those with GA (adjusted hazard ratio [aHR] 5.36) with an increased risk when accompanied by p.Ala4399Thr or RVs (aHR 15.22 and 16.60, respectively). Within the choroidal anastomosis–positive hemispheres, GG exhibited a higher incidence of de novo hemorrhage than GA (p = 0.004). The GG of p. Arg4810Lys was a risk factor for de novo hemorrhage in asymptomatic MMD hemispheres. This risk increased with certain other variants and is observed in choroidal anastomosis–positive hemispheres. A comprehensive evaluation of RNF213 variants and angioarchitectures is essential for predicting the phenotype of asymptomatic hemispheres in MMD. [ABSTRACT FROM AUTHOR]

Head CT, which includes the facial region, can visualize faces using 3D reconstruction, raising concern that individuals may be identified. We developed a new de-identification technique that distorts the faces of head CT images. Head CT images that were distorted were labeled as "original images" and the others as "reference images." Reconstructed face models of both were created, with 400 control points on the facial surfaces. All voxel positions in the original image were moved and deformed according to the deformation vectors required to move to corresponding control points on the reference image. Three face detection and identification programs were used to determine face detection rates and match confidence scores. Intracranial volume equivalence tests were performed before and after deformation, and correlation coefficients between intracranial pixel value histograms were calculated. Output accuracy of the deep learning model for intracranial segmentation was determined using Dice Similarity Coefficient before and after deformation. The face detection rate was 100%, and match confidence scores were < 90. Equivalence testing of the intracranial volume revealed statistical equivalence before and after deformation. The median correlation coefficient between intracranial pixel value histograms before and after deformation was 0.9965, indicating high similarity. Dice Similarity Coefficient values of original and deformed images were statistically equivalent. We developed a technique to de-identify head CT images while maintaining the accuracy of deep-learning models. The technique involves deforming images to prevent face identification, with minimal changes to the original information. [ABSTRACT FROM AUTHOR]

Background  Clinical behavior of pituitary neoplasms is peculiar and notoriously difficult to predict. While aggressive tumors are common, metastasis is very rare, can be highly delayed, and there are no histological or clinical features to meaningfully predict this happening. Endocrinologically silent tumors are particularly difficult, as there is less opportunity to detect early metastasis. Together, this amounts to a situation of uncertainty over the appropriate management of such tumors before and after metastasis. Case Description  The authors report two cases of nonfunctioning aggressive pituitary adenoma (APA) each requiring two transsphenoidal surgeries, a transcranial resection and radiotherapy. Both these tumors subsequently metastasized caudally along the neuraxis, years later, as a null cell carcinoma associated with a germline CHEK2 mutation and a silent Crooke's cell carcinoma. The former represents a novel oncogenetic association. Conclusion  Delayed drop dural metastasis of pituitary carcinoma is becoming increasingly recognized. Surgical resection of the distant disease to confirm the diagnosis and relieve the mass effect, followed by temozolomide chemotherapy, is the current treatment of choice. The need for both long-term follow-up in patients with APA, and a high degree of suspicion toward dural-based radiographic findings is emphasized. [ABSTRACT FROM AUTHOR]

Background: A duplicated middle cerebral artery (DMCA) is an anatomical variant that includes duplication of the middle cerebral artery (MCA) and an anomalous vessel originating between the anterior choroidal artery (AChA) and the distal end of the internal carotid artery (ICA). Here, we present a case report of an ICA aneurysm with a DMCA and the AChA originating from the dome, which was successfully treated with clipping. Case Description: In a 64-year-old man, preoperative angiography revealed an unruptured right ICA aneurysm with a maximum diameter of 4.3 mm, and fusion three-dimensional computer graphics revealed that a DMCA and the AChA originated from the dome. The aneurysm enlarged; therefore, clipping was performed. The closure of the aneurysm while preserving the patency of the DMCA and AChA was identified using intraoperative microvascular Doppler ultrasonography and indocyanine green video angiography. The postoperative course was uneventful, and no ischemic lesions were confirmed on MR imaging. Conclusion: To the best of our knowledge, this is the first report of an ICA aneurysm with a DMCA and the AChA arising from the dome. In such cases, the anatomy of the DMCA and AChA should be well-characterized before treatment. [ABSTRACT FROM AUTHOR]

Background: Vestibular schwannomas (VS) present at variable size with heterogeneous symptomatology. Modern treatment paradigms for large VS include gross total resection, subtotal resection (STR) in combination with observation, and/or radiation to achieve optimal function preservation, whereas treatment is felt to be both easier and safer for small VS. The objective is to better characterize the presentation and surgical outcomes of large and small VS. Methods: We collected data of patients who had surgically treated VS with a posterior fossa diameter of 4.0 cm or larger (large tumor group, LTG) and smaller than 1.0 cm in cisternal diameter (small tumor group, STG). Statistical significance was defined as p < 0.05. Results: LTG included 48 patients (average tumor size: 44.9 mm) and STG 38 (7.9 mm). Patients in STG presented more frequently with tinnitus and sudden hearing loss. Patients in LTG underwent more STR than STG (50.0% vs. 2.6%, p < 0.0001). LTG had more complications (31.3% vs. 13.2%, p = 0.049). Postoperative facial nerve function in STG was significantly better than LTG. STG had better hearing preoperatively (p < 0.0001) and postoperatively than LTG (p = 0.0002). Postoperative headache was more common in STG (13.2% vs. 2.1%, p = 0.045). The rate of recurrence/progression needing treatment was not statistically different between the groups (12.5% in LTG vs. 7.9% in STG, p = 0.49). Those patients who required periprocedural cerebrospinal fluid diversion had higher risk of infection (20.8% vs 4.8%, p = 0.022). Conclusion: Large and small VS present differently. LTG showed more unsatisfactory outcomes in facial nerve function and postoperative hearing despite maximal efforts undertaken toward function-preservation strategy; however, similar tumor control was achieved. [ABSTRACT FROM AUTHOR]

Background  Consensus in timing of radiotherapy is yet to be established in esthesioneuroblastoma (ENB). Objective  This study was aimed to investigate if planned adjuvant radiotherapy improves tumor control after complete margin negative resection of low Hyams' grade (1 or 2) ENB. Methods  A retrospective review of patients with pathologically confirmed negative margin resection of Kadish's stage B or C and Hyams' grade 1 and 2 ENBs was conducted. Seventeen patients meeting the criteria were divided into the following two groups for cohort study: (1) those who underwent planned immediate postoperative adjuvant radiotherapy (IR group) and (2) those who did not (delayed radiotherapy [DR] group). Results  The IR group included nine patients (Kadish's stage B in one and stage C in eight; Hyams' grade 1 in two and grade 2 in seven). Mean follow-up was 140.8 months. Seven patients (78%) had disease progression (DP) at a median of 88 months (four with cervical lymph node metastasis [CLNM], one with distant metastasis, and two with both local recurrence and CLNM). One patient experienced frontal lobe abscess. The DR group included eight patients (Kadish's stage B in six and stage C in two; all Hyams' grade 2). Mean follow-up was 123.3 months. Four (50%) patients who developed DP (all local recurrence) were salvaged with surgery and adjuvant radiotherapy at a median of 37.5 months. There was no statistically significant difference in DP rate (p  = 0.23), time to DP (p  = 0.26), or the local tumor control rate (p  = 0.23). Conclusion  In our limited cohort, immediate postoperative radiotherapy did not demonstrate superiority in tumor control, although risk of radiotherapy toxicity appears low. [ABSTRACT FROM AUTHOR]

Distal anterior cerebral artery (DACA) aneurysms are rare, accounting for 1–9% of all intracranial aneurysms. Previous systematic reviews have highlighted that given the markedly increased incidence of major complications after endovascular treatment, microsurgical clipping is the more attractive treatment option with generally excellent clinical outcomes. Subcallosal DACA aneurysms constitute a rare subset of these aneurysms, requiring special anatomic considerations—particularly with regard to the approach. The aim of this study is to review the technical nuances of microsurgical treatment of subcallosal DACA aneurysms, including review of contemporary techniques through presentation of a microneurosurgical operative video. This is a retrospective case series and intraoperative microsurgical videos review. Three subcallosal DACA aneurysms were identified via retrospective query of our institutional neurosurgical database from December 2017 to May 2018. Two were female; median age was 74 years (range 70–83); all 3 underwent bifrontal craniotomy via bicoronal skin incision for aneurysm clipping. Aneurysms were located in left pericallosal–callosomarginal artery junction, bifurcation of azygos A2, and pericallosal artery related with azygos A2, and the anterior interhemispheric approach was used in all 3 operations. No acute stroke, hemorrhage, or major complications occurred, and all patients remained neurologically intact at the time of last follow-up (median 3 months, range 1–6). Although DACA aneurysms are rare, they represent an important variant for cerebrovascular neurosurgeons where microsurgical clipping can have better angiographic outcomes than endovascular treatment. Detail-oriented anterior interhemispheric arachnoid dissection through bifrontal craniotomy with its lower margin sitting at the superior orbital rim maximizes safe and effective clipping of subcallosal DACA aneurysms. [ABSTRACT FROM AUTHOR]

Purpose: Cavernous sinus syndrome is a rare phenomenon, characterized by simultaneous neuropathies of cranial nerves III-VI. Various pathological processes have been reported as precipitating etiologies, including infection, inflammation, vascular lesions, and neoplasms.Purpose: We report a unique case series of cavernous sinus syndrome attributable to prolonged Trendelenburg or prone positioning during non-cranial procedures and review the pertinent literature to enlighten on this rare but catastrophic phenomenon.Methods: Retrospective case series.Results: In the past year we encountered two patients who presented with acute cavernous sinus syndrome upon awakening from non-cranial operations. One patient underwent an extensive urologic resection of a bladder malignancy positioned in Trendelenburg for approximately 4 h. The second patient underwent a lumbar laminectomy and discectomy in prone position. Both patients were discovered to have infarcted large pituitary macroadenomas as the etiology of their acute ophthalmoplegias, and transnasal, transsphenoidal resection was performed acutely to decompress the cavernous sinus contents. Pathologic analysis of the resected specimens in each case confirmed necrotic, infarcted pituitary adenoma. Both patients made a complete recovery with no evidence of residual or recurrent tumor in short term follow-up.Conclusion: We report a brief case series of acute cavernous sinus syndrome resulting from dependent positioning during non-cranial operations in patients with pituitary macroadenoma. Although rare, this highlights a potential danger of “head down” positioning in patients with intracranial pathology—particularly in or around the sella and cavernous sinus. Despite multiple cranial neuropathies upon presentation, both patients made complete recovery following surgical decompression of the cavernous sinuses. [ABSTRACT FROM AUTHOR]

Introduction Non-saccular aneurysms of the posterior circulation are uncommon but highly dangerous lesions. Flow diverter stents have been demonstrated to be effective treatments of various anterior circulation aneurysms, particularly large and giant proximal internal carotid artery aneurysms. However, evidence regarding the treatment of non-saccular posterior circulation aneurysms with flow diverters is lacking. Methods A systematic literature review of the English language literature since 2007 was conducted using PubMed, MEDLINE, and Embase. Keywords and MeSH terms included flow diversion, flow diverter, pipeline, surpass, intracranial aneurysm, vertebrobasilar, and non-saccular. Case reports were excluded. Angiographic and clinical outcomes were pooled using a random effects meta-analysis. results 13 retrospective non-comparative studies reporting 129 patients and 131 aneurysm treatments were included. The average number of flow diverters per aneurysm was 4.33. Immediate complete or near complete occlusion of the aneurysm occurred in 25% (95% CI 1% to 60%), and long term occlusion in 52% (29-76%). Periprocedural stroke occurred in 23% of cases. Good long term neurologic outcome (modified Rankin Scale score ≤2) was achieved in 51% (95% CI 31% to 71%). Overall mortality was 21% (95% CI 7% to 38%) and morbidity was 26% (12%-42%). Retreatment was required in 5% (95% CI 0% to 14%). Good neurologic outcome rate was higher in vertebral artery aneurysms (83%) than other locations (18-33%). Conclusions Flow diversion is a feasible and efficacious treatment for non-saccular aneurysms in the posterior circulation. However, the intervention carries a significant risk of periprocedural stroke, and is still associated with high overall mortality. Further studies are needed to identify significant treatment risk factors and optimize patient selection. [ABSTRACT FROM AUTHOR]

Background: The frontal bridging vein, which is the venous drainage route of the frontal cortex into the superior sagittal sinus (SSS), sometimes poses an obstacle in the anterior interhemispheric approach during surgery for anterior cerebral artery aneurysms. Although severe complications including venous infarction or edema due to damage to the bridging vein are well known, only a few reports have discussed how to avoid venous injury when we must sacrifice the bridging vein to obtain an appropriate surgical field. This report describes a microvascular technique performed in two patients who underwent rerouting of the bridging vein to obtain an appropriate anterior interhemispheric surgical corridor to treat a ruptured anterior cerebral artery aneurysm. The hindering bridging vein was resected from the entrance to the SSS and anastomosed toward the adjacent cortical vein. Methods: A 65-year-old male and a 43-year-old male were admitted to our hospital for sudden headache. Computed tomography, magnetic resonance angiogram, or digital subtraction angiography demonstrated a subarachnoid hemorrhage and an anterior cerebral artery aneurysm in both patients. In both cases, a relatively robust bridging vein, which appeared problematic to sacrifice, was draining into the SSS, resulting in a limited surgical corridor. Thus, we performed cortical vein reconstruction, and the aneurysms were successfully clipped under a wider surgical view. Results: We confirmed completed clipping without postoperative venous complications. One patient demonstrated patency of reconstructed venous flow by digital subtraction angiography. No apparent cognitive impairment was seen in either patient. Conclusions: This technique may be useful for obtaining an appropriate surgical corridor when the frontal bridging vein may be damaged. [ABSTRACT FROM AUTHOR]

Trigeminal schwannoma is a rare skull base tumor that can be managed in a variety of treatments including image observation, surgery, stereotactic radiosurgery, such as gamma knife radiosurgery (GKS), and combination of these. Endoscopic transnasal resection is very effective when the tumor is not invading far laterally, or the risk of cerebrospinal fluid (CSF) leak is estimated to be low. A 74-year-old man with a history of prostate cancer and diabetes presented with left oculomotor nerve palsy over a month. Magnetic resonance images (MRI) demonstrated a 25-mm mass in the left cavernous sinus protruding to the left orbit via the superior orbital fissure (Fig. 1). The patient underwent endoscopic transnasal surgery to decompress the mass. The surgery was uneventful, and postoperative MRI demonstrated satisfactory subtotal resection of the mass (Fig. 2). The final pathology returned as schwannoma. At 1-year follow-up, the tumor slowly enlarged, and the patient underwent GKS with a marginal dose of 14 Gy. At the last follow-up, 4 months after GKS, the tumor was stable. Unfortunately the patient deceased from the known prostate cancer. Endoscopic transnasal surgery was especially useful in this case, considering the preoperative known cancer state that management of this benign tumor did not ruin the quality of life of this patient while minimizing hospitalization, as achieving satisfactory tumor control with aid from postoperative GKS, minimizing complications. The link to the video can be found at: https://youtu.be/Q0Ugc2VFV4w. [ABSTRACT FROM AUTHOR]

Background: Acute extracranial internal carotid artery (ICA) occlusion by a huge cardiogenic embolus is rare, but can be catastrophic. Methods: Seven patients with acute ischemic stroke due to embolic occlusion of extracranial ICA who underwent emergent cervical surgical embolectomy were retrospectively reviewed. Diagnosis was made in six patients with magnetic resonance imaging (MRI) with optional digital subtraction angiography (DSA), while computed tomography (CT) and DSA were used in one patient with an implanted pacemaker. Clinical outcomes, including recanalization rate, recanalization time, complications, modified Rankin scale (mRS) at 3 months, and National Institute of Health Stroke Scale (NIHSS) score improvement at 1 month were evaluated. Results: Complete recanalization was obtained in seven patients (100 %). Median recanalization time from symptom onset and from start of surgery was 402 and 40 min, respectively. All seven patients showed severe left ventricular hypertrophy (LVH) according to an increased cardiothoracic ratio (CTR) ≥50 %. Complications included recurrence of cardioembolic stroke with the right middle cerebral artery occlusion, minimal expansion of infarction, and aggravation of heart failure, each in one patient, respectively. Four (57.1 %) patients had a history or postoperative recurrence of cardioembolic stroke. Median NIHSS at 1 month was 2 (range, 0-30). Median mRS at 3 months was 2 (range, 0-5). Five patients (71.4 %) had a favorable outcome (mRS2). Conclusions: Cervical surgical embolectomy for acute extracranial ICA occlusion resulted in a high complete recanalization rate with an acceptable safety profile. A possible association between severe cardiac illness and huge embolus occluding proximal large artery was suggested. [ABSTRACT FROM AUTHOR]

Background: Executive functions are complex cognitive control functions that include cognitive flexibility, inhibition (self-control, self-regulation), and working memory. Bilateral frontal lobe ischemia is associated with cognitive impairment, especially in the context of dysexecutive syndrome. This report describes two patients who underwent bilateral anterior cerebral artery (ACA) reconstruction by A3-A3 anastomosis in conjunction with superficial temporal artery (STA)-radial artery (RA) graft-A3 bypass to treat bilateral ACA steno-occlusive hemodynamic ischemia accompanying cognitive and executive dysfunction. Method: A 74-year-old woman and a 73-year-old woman were admitted to our hospital for unilateral cerebral infarction in the frontal lobe. Magnetic resonance angiogram (MRA) demonstrated severe bilateral ACA steno-occlusive pathology in both patients. Considering the presence of impaired cognitive function with dysexecutive syndrome as well as hemodynamic compromise shown by single photon emission computed tomography (SPECT), we proceeded with A3-A3 anastomosis in conjunction with STA-RA-A3 bypass. Various neuropsychiatric tests were performed before and after the surgery. Results: We confirmed good bypass patency without periprocedural complications. One patient recovered from apallic and bedridden status and regained ambulatory condition and ability to take in an oral diet. Another patient demonstrated improved scores in several cognitive tests with some persistent executive dysfunction. Conclusions: Bilateral ACA revascularization was technically feasible in two patients. This bypass surgery could have some positive effects in some basic cognitive function, such as memory, attention, and concentration by bilateral ACA hemodynamic improvement, although executive function, which is specific to prefrontal function, might not be reversible. [ABSTRACT FROM AUTHOR]

Background: The white-collar sign (WCS) is represented by the formation of neointimal tissue at the level of the aneurysm neck as the successful outcome on follow-up angiography after coil embolization. WCS has been reported only in aneurysms treated with Matrix® coils. This is the first study to report WCS emergence in aneurysms treated with bare platinum coils, and potential factors associated with WCS emergence were evaluated. Method: Total 130 unruptured (female: male ratio, 100: 30; mean age, 60 years) cerebral aneurysms were treated with coil embolization. Embolization status was assessed immediately and 1 year after treatment, and emergence of WCS in follow-up angiography was assessed. We evaluated the association between WCS emergence and aneurysm location, dome diameter, neck diameter, dome-neck ratio, and type of coil used (bare platinum or bioactive). Results: WCS appeared in nine aneurysms (6.9 %), of which six were treated only with bare platinum coils. Neck diameter was significantly smaller in the WCS-positive group than in the WCS-negative group. The proportion of aneurysms treated with bioactive coils was not significantly different between the groups. Immediate embolization status in the WCS-positive group tended to be slightly better than that in the WCS-negative group. No aneurysmal morphological characteristics other than small neck diameter were associated with WCS emergence. Conclusions: WCS is not specific to bioactive coil usage. Small neck diameter was significantly associated with WCS emergence in our series. Further investigations to clarify the predictors of WCS will contribute to progress of aneurysmal embolization. [ABSTRACT FROM AUTHOR]

Background  The far-lateral approach is an effective skull base technique that provides access to the lower clivus and premedullary area. This approach is also useful for maximal visualization and resection of large posterior fossa tumors with extensive medial extension, especially when aggressive resection is ideal for a malignant tumor in a young patient, or it is technically challenging because of tumor calcification. We demonstrate a microsurgical operative video to describe technical pearls in this difficult situation. Case Presentation  A 45-year-old man with history of "hoarseness" for up to two decades was presented with imbalance and mild dysphagia over 3 years. Imaging demonstrated a 55-mm left cerebellopontine angle (CPA) tumor extending medially across the midline with severe calcification (Figs. 1 and 2). His neurological examination revealed left facial numbness, complete left facial weakness, left deafness, complete left vocal paralysis, as well as severe left hemibody ataxia. The tumor was resected via a left suboccipital craniotomy with far lateral approach including drilling to the occipital condyle and C1 laminectomy. Initial manipulation of the inferior pole of the tumor resulted in asystole which was managed successfully with glycol pyrolate. Pathology demonstrated IDH-1 wild type, MGMT-methylated glioblastoma. The patient subsequently underwent adjuvant chemoradiation. Conclusion  The far-lateral approach is an effective approach for maximal safe resection of a malignant brainstem, cerebellar, and CPA tumor. The link to the video can be found at: https://youtu.be/AIGebJPJEnw. [ABSTRACT FROM AUTHOR]