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Purpose: This study aims to comprehensively delineate the phenotypic spectrum of ACTL6B-related disorders, previously associated with both autosomal recessive and autosomal dominant neurodevelopmental disorders. Molecularly, the role of the nucleolar protein ACTL6B in contributing to the disease has remained unclear., Methods: We identified 105 affected individuals, including 39 previously reported cases, and systematically analysed detailed clinical and genetic data for all individuals. Additionally, we conducted knockdown experiments in neuronal cells to investigate the role of ACTL6B in ribosome biogenesis., Results: Biallelic variants in ACTL6B are associated with severe-to-profound global developmental delay/intellectual disability (GDD/ID), infantile intractable seizures, absent speech, autistic features, dystonia, and increased lethality. De novo monoallelic variants result in moderate-to-severe GDD/ID, absent speech, and autistic features, while seizures and dystonia were less frequently observed. Dysmorphic facial features and brain abnormalities, including hypoplastic corpus callosum, parenchymal volume loss/atrophy, are common findings in both groups. We reveal that in the nucleolus, ACTL6B plays a crucial role in ribosome biogenesis, in particular in pre-rRNA processing., Conclusion: This study provides a comprehensive characterization of the clinical spectrum of both autosomal recessive and dominant forms of ACTL6B-associated disorders. It offers a comparative analysis of their respective phenotypes provides a plausible molecular explanation and suggests their inclusion within the expanding category of 'ribosomopathies'., (Copyright © 2024. Published by Elsevier Inc.)

Introduction: Social media has allowed patients with rare diseases to connect and discuss their experiences with others online. This study analyzed various social media platforms to better understand the patient's perception of arteriovenous malformation., Methods: Twitter, Instagram, and TikTok were searched to find posts about patients' experiences with arteriovenous malformations (AVM). Posts unrelated to the patient's experience were excluded. Posts were coded for the relevant themes related to their experience with the disease, as well as engagement, and gender., Results: The most common theme was raising awareness about the condition (87.0%). Recounting symptoms (50.2%), spreading positivity (17.5%), and survival (8.3%) were other common themes. Other prevalent themes were pain (5.2%) and fear of a rare disease (3.5%). Approximately half of AVM-related Instagram (47.93%) and TikTok (52.94%) posts raised awareness about the condition. Most Instagram (67.75%) and TikTok (89.71%) posts focused on recovery and rehabilitation. Most TikTok posts discussed "survival" or "death" (57.35%), while the majority focused on spreading positivity (79.41%). Most posts were made by women (69.6%). Females were more likely than males to post about the scientific explanation of AVMs ( p  = 0.003)., Conclusion: Social media allows patients across the country and the globe to discuss their experiences with uncommon diseases and connect with others. It also allows AVM patients to share their experiences with other patients and the public., Competing Interests: Declaration of conflicting interestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background and Objectives: Predicting functional outcomes after surgical management of ruptured aneurysms is essential. This study sought to validate the modified Southwestern Aneurysm Severity Index (mSASI), which predicts functional outcomes 1 year after treatment., Methods: The surgical arm of a randomized controlled trial, the Barrow Ruptured Aneurysm Trial, was used to validate the mSASI model. mSASI scores incorporating the Hunt and Hess scale, Non-Neurological American Society of Anesthesiologists Physical Classification Status, imaging findings, and other modifiers were assigned and evaluated against the Glasgow Outcome Scale (GOS) score at 1 year. The model's performance was assessed for discrimination and calibration. Similar evaluations were constructed using the modified Rankin Scale (mRS) as the 1-year functional outcome measurement. Long-term outcomes (3, 6, 10 years) were also evaluated., Results: Of 280 clinical trial patients treated surgically, 242 met the inclusion criteria. The mean age was 54.1 ± 12.9 years; 31% were men. Favorable GOS score (4-5) and mRS score (0-2) at 1 year were observed in 73.6% and 66.1% of patients, respectively. The mSASI model predicted unfavorable GOS score at 1 year with fair to good discrimination (area under the curve = 0.75, 95% CI = 0.68-0.82) and accurate calibration (R 2 = 0.98). Similar results were obtained when mRS was used as the outcome measure (area under the curve = 0.75, 95% CI = 0.68-0.82; R 2 = 0.95)., Conclusion: The mSASI model was externally validated in our cohort to predict functional outcomes using the GOS or mRS scores 1 year after surgery. This index may be used for prognosticating outcomes of patients undergoing surgery for ruptured aneurysms at short-term and long-term intervals., (Copyright © Congress of Neurological Surgeons 2024. All rights reserved.)

Background and Objectives: Microsurgical resection is the only curative intervention for symptomatic brainstem cavernous malformations (BSCMs), but the management of these lesions in older adults (≥65 years) is not well described. This study sought to address this gap by examining the safety and efficacy of BSCM resection in a cohort of older adults., Methods: Records of patients who underwent BSCM resection over a 30-year period were reviewed retrospectively. Baseline characteristics and outcomes were compared between older (≥65 years) and younger (<65 years) patients., Results: Of 550 patients with BSCM who met inclusion criteria, 41 (7.5%) were older than 65 years. Midbrain (43.9% vs 26.1%) and medullary lesions (19.5% vs 13.6%) were more common in the older cohort than in the younger cohort ( P = .01). Components of the Lawton BSCM grading system (ie, lesion size, crossing axial midpoint, developmental venous anomaly, and timing of hemorrhage) were not significantly different between cohorts ( P ≥ .11). Mean (SD) Elixhauser comorbidity score was significantly higher in older patients (1.86 [1.06]) than in younger patients (0.66 [0.95]; P < .001). Older patients were significantly more likely than younger patients to have poor outcomes at final follow-up (28.9% vs 13.8%, P = .01; mean follow-up duration, 28.7 [39.1] months). However, regarding relative neurological outcome (preoperative modified Rankin Scale to final modified Rankin Scale), rate of worsening was not significantly different between older and younger patients (23.7% vs 14.9%, P = .15)., Conclusion: BSCMs can be safely resected in older patients, and when each patient's unique health status and life expectancy are taken into account, these patients can have outcomes similar to younger patients., (Copyright © Congress of Neurological Surgeons 2024. All rights reserved.)

The Woven EndoBridge (WEB) and other intrasaccular flow disruptors show promise in treating intracranial aneurysms. We performed a bibliometric analysis to provide novel insights into the trends and trajectory of these devices. We systematically assessed bibliometric data such as citations, journals, study designs, open access status, and multi-institutional involvement for the top-cited articles on WEB and other disruptors. The top 100 cited studies on the WEB had citations from 7 to 144 (mean ± standard deviation 35.6 ± 29.5), while only 33 studies were published for other intrasaccular flow disruptors (4.24 ± 8.45). Of the other devices, the Contour has the most publications (n = 21). Retrospective reviews were the most common study design for both WEB and other intrasaccular devices. France published the most studies in the top 100 WEB papers (n = 35), while Germany led for other flow disruptors (n = 10). In all studies analyzed, no senior authors from Africa are present. The top 100 WEB publications had a higher mean citation count (35.6 vs. 4.24, P<0.001), higher mean citations per year (5.24 vs. 1.03, P<0.01), and a higher proportion of multi-institutional collaborations (44.0% vs. 12.1%, P<0.01) than other intrasaccular flow disruptors. In conclusion, countries with the most publications on WEB are not necessarily the ones leading the way with newer intrasaccular devices, while study designs remain similar. There is a limited contribution to the literature outside of Europe and North America. Our findings identify notable collaborators and trends, providing a snapshot of the field and a roadmap for future research., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: Extended reality (XR) includes augmented reality (AR), virtual reality (VR), and mixed reality (MR). Endovascular neurosurgery is uniquely positioned to benefit from XR due to the complexity of cerebrovascular imaging. Given the different XR modalities available, as well as unclear clinical utility and technical capabilities, we clarify opportunities and obstacles for XR in training vascular neurosurgeons., Methods: A systematic review following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines was conducted. Studies were critically appraised using ROBINS-I., Results: 19 studies were identified. 13 studies used VR, while 3 studies used MR, and 3 studies used AR. Regarding specific educational applications, VR was used for simulation in 10 studies and anatomical modeling in 3 studies. AR was only used for live intra-operative guidance ( n  = 3 studies). MR was only used for modeling and intra-operative teaching. Considering disease-specific uses, XR enhanced trainee understanding of intracranial aneurysms ( n  = 12 studies) and stroke ( n  = 7). XR trained surgeons in diverse neurosurgical procedures, including aneurysm coiling ( n  = 5 studies), diagnostic angiography ( n  = 5), and thrombectomy ( n  = 5)., Conclusions: Anatomical modeling with VR and MR enhances neurovascular anatomy education with patient-specific, 3-D models from imaging data. AR and MR enable live intra-operative guidance, allowing experienced surgeons to remotely instruct novices, potentially improving patient care and reducing geographic disparities. AR overlays enhance instruction by allowing the surgeon to highlight key procedural aspects during training. Inaccurate tracking of surgical tools is an XR technological barrier for modeling and intra-operative training. Importantly, the most reported application of XR is VR for simulation-using platforms like the Mentice VIST and Angio Mentor. 10 studies examine VR for simulation, showing enhanced procedural performance and reduced fluoroscopy use after short training, although long-term outcomes have not been reported. Early-stage trainees benefited the most. Simulation improved collaboration between neurosurgeons and the rest of the surgical team, a promising role in interprofessional teamwork. Given the strength of VR for simulation, MR for simulation is an important gap in the literature for future studies. In conclusion, XR holds promise for transforming neurosurgical education and practice for simulation, but technological research is needed in modeling and intra-procedural training., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (© 2024 Patel, Covell, Patel, Kandregula, Palepu, Gajjar, Shekhtman, Sioutas, Dhanaliwala, Gade, Burkhardt and Srinivasan.)

Developmental and epileptic encephalopathies (DEEs) feature altered brain development, developmental delay and seizures, with seizures exacerbating developmental delay. Here we identify a cohort with biallelic variants in DENND5A, encoding a membrane trafficking protein, and develop animal models with phenotypes like the human syndrome. We demonstrate that DENND5A interacts with Pals1/MUPP1, components of the Crumbs apical polarity complex required for symmetrical division of neural progenitor cells. Human induced pluripotent stem cells lacking DENND5A fail to undergo symmetric cell division with an inherent propensity to differentiate into neurons. These phenotypes result from misalignment of the mitotic spindle in apical neural progenitors. Cells lacking DENND5A orient away from the proliferative apical domain surrounding the ventricles, biasing daughter cells towards a more fate-committed state, ultimately shortening the period of neurogenesis. This study provides a mechanism for DENND5A-related DEE that may be generalizable to other developmental conditions and provides variant-specific clinical information for physicians and families., (© 2024. The Author(s).)

Background and Objectives: Flow diversion (FD) of intracranial aneurysms (IAs) is an increasingly used and efficacious treatment modality. Transcirculation approaches, or approaches that cross the contralateral or anteroposterior arterial supply before reaching a target vessel, have been used to treat cerebrovascular pathologies when traditional approaches are unsuitable or require intraoperative complication management. This study sought to review IAs treated with FD using a transcirculation approach to determine the technique's safety and efficacy., Methods: A systematic review of the PubMed, Scopus, Web of Science, and Embase databases was completed per Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Studies were included if they described transcirculation approaches in adult patients with IAs undergoing FD. Outcomes of interest included intraoperative complications and aneurysm occlusion rates., Results: Twelve studies with 19 patients (N = 19, mean age = 54.1 y, 89.5% female) were identified. Wide-necked (N = 5, 26.3%) and saccular (N = 5, 26.3%) aneurysms were most represented, while 57.9% (N = 11) of aneurysms were unruptured and 15.8% (N = 3) of aneurysms were ruptured. The mean aneurysm sac and neck size were 16.9 mm and 11.9 mm, respectively. The most commonly deployed flow diverter was the Pipeline Embolization Device (N = 14, 73.9%). Successful FD (complete occlusion and/or good wall apposition) was recorded in 84.6% of qualifying patients with follow-up data, while 2 patients (15.4%) developed an intraoperative carotid-cavernous fistula., Conclusion: Transcirculation approaches to FD offer neurointerventionalists a safe and efficacious method for device deployment, rescue scenarios, and challenging anatomy. Prospective studies may determine the most appropriate indications for transcirculation approaches to FD, while novel, lower profile devices may improve its technical feasibility and safety., (Copyright © Congress of Neurological Surgeons 2024. All rights reserved.)

Background and Objectives: Robotic neurointervention enhances procedural precision, reduces radiation risk, and improves care access. Originally for interventional cardiology, the CorPath GRX platform has been used in neurointerventions. Recent studies highlight robotic cerebral angiography benefits, but information on spinal angiography is limited. While a new generation of robotic solutions is on the horizon, this series evaluates our experience with the CorPath GRX in spinal angiographic procedures, addressing a key gap in neurointerventional research., Methods: In this single-center retrospective case series, we analyzed 11 patients who underwent robotic-assisted diagnostic procedures with the CorPath GRX system from February 2022 to March 2023 at our institution. A descriptive synthesis was performed on the demographic, baseline, surgical, and postoperative data collected., Results: The average age of the 11 patients was 54 ± 20.34 years, with six (54.55%) female. The mean body mass index was 29.58 ± 7.86, and 7 (63.64%) were non-smokers. Of the 11 procedures using the CorPath GRX system, four (36.36%) were partially converted to manual technique. General anesthesia was used in nine cases (81.82%), and right-side femoral access in ten (90.91%) patients. Mean fluoroscopy time was 24.81 ± 10.19 min, contrast dose 174.09 ± 57.31 mL, dose area product 472.23 ± 437.57 Gy·cm², and air kerma 2438.84 ± 2107.06 mGy. No robot-related complications and minimal procedure-related complications were reported., Conclusion: The CorPath GRX system, a robotic-assisted platform, has proven reliable and safe in spinal angiography, evidenced by its enhanced procedural accuracy and reduced radiation exposure for operators., Competing Interests: Authors’ contributionsAll authors contributed equally to this work. Conceptualization: VMS; methodology: OS and GS; formal analysis: MD and IMM; resources: OS, GS, GP, and IMM; funding acquisition: JKB and VMS; project administration: JKB, VMS, and BJ; supervision: VMS, BJ, and BP; Writing—original draft: OS, GS, and IMM; Writing—review and editing: BJ, BP, JKB, and VMS. Declaration of conflicting interestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Neurosurgeons often use radiation to visualize blood vessels and implants intraoperatively. However, high exposure to radiation increases one's cancer risk. This study aims to investigate intraoperative ionizing radiation exposure awareness and associated morbidity among neurosurgeons., Methods: An anonymized 30-question survey about their intraoperative radiation exposure, protective measures, radiation knowledge, and any conditions that can arise from protracted radiation exposure was disseminated to 3344 American Association of Neurological Surgeons members., Results: A total of 227 (6.8%) neurosurgeons completed the survey. Most neurosurgeons (61, 27%) performed 2-4 surgeries per week necessitating radiation (61, 27%), did not use a dosimeter (134, 59%), and wore a lead apron (89%) and a thyroid shield (75%). Only 7 (3%) of respondents could correctly identify the safety limit for occupational radiation. One hundred and thirty-four (59%) respondents correctly identified the relationship between distance and radiation dose reduction. Two hundred and thirteen (94%) neurosurgeons reported concern about occupational radiation exposure. No significant association was found between occupational radiation exposure and the rate of cataracts, combined cancer, and skin cancer. Multivariate logistic regression adjusting for age and cancer history found that the likelihood of developing leukemia (P = 0.02) and nonmalignant thyroid nodular disease (P = 0.01) is positively associated with increased total occupational radiation exposure., Conclusions: There is a need for improved radiation safety awareness among neurosurgeons, especially in the context of rising usage of minimally invasive surgery. This can allow for a greater understanding of radiation-associated risks among neurosurgeons and guide the implementation of safer practices., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Introduction: United States stroke systems are increasingly transitioning from alteplase (TPA) to tenecteplase (TNK). Real-world data on the safety and effectiveness of replacing TPA with TNK before large vessel occlusion (LVO) stroke endovascular treatment (EVT) are lacking., Methods: Four Pennsylvania stroke systems transitioned from TPA to TNK during the study period 01/2020-06/2023. LVO stroke patients who received intravenous thrombolysis with TPA or TNK before EVT were reviewed. Multivariate logistic analysis was conducted adjusting for age, sex, National Institute of Health Stroke Scale (NIHSS), occlusion site, last-known-well-to-intravenous thrombolysis time, interhospital-transfer and stroke system., Results: Of 635 patients, 309 (48.7%) received TNK and 326 (51.3%) TPA prior to EVT. The site of occlusion was the M1 middle cerebral artery (MCA) (47.7%), M2 MCA (25.4%), internal carotid artery (14.0%), tandem carotid with M1 or M2 MCA (9.8%) and basilar artery (3.1%). A favorable functional outcome (90-day mRS ≤ 2) was observed in 47.6% of TNK and 49.7% of TPA patients (p = 0.132). TNK versus TPA groups had similar rates of early recanalization (11.9% vs. 8.4%, p = 0.259), successful endovascular reperfusion (93.5% vs. 89.3%, p = 0.627), symptomatic intracranial hemorrhage (3.2% vs. 3.4%, p = 0.218) and 90-day all-cause mortality (23.1% vs. 21.5%, p = 0.491)., Conclusions: This U.S. multicenter real-world clinical experience demonstrated that switching from TPA to TNK before EVT for LVO stroke resulted in similar endovascular reperfusion, safety, and functional outcomes., (© 2024. The Author(s).)

Objective: The cerebral and spinal venous systems have similar functions but unique anatomical and physiological properties. CSF occupies space in the cranial and spinal vaults, is continuously produced, and has many roles, including maintaining a favorable environment for CNS structures. The influence of the cerebrospinal venous system on CSF dynamics has been theorized since the 1940s. Newer studies suggest venous outflow pattern alterations in response to changes in body position. However, the relationship of postural cerebrospinal venous outflow shifts with and their influence on CSF homeostasis is not well understood., Methods: The authors searched the published literature related to the anatomy and function of vertebral venous plexus (VVP), CSF, and positional cerebral venous flow characteristics. A comprehensive collection of literature was compiled and reviewed, and the relationship between cerebrospinal and venous system changes and alterations in body positions, with an emphasis on the craniocervical system, is discussed., Results: The VVP is a network of valveless veins extending from the sacrum to the cranium that are interconnected with the cranial dural sinuses. The internal VVP occupies space within the extradural spinal canal and functions to return spinal venous blood to the heart, but it has additional properties, including the capability of bidirectional venous flow, an intraspinal dilatory capacity, and a role in cerebral venous outflow. When one rises to the upright position, CSF shifts toward the spinal canal and force vectors change, leading to reduced intracranial CSF pressure; simultaneously, cerebral venous outflow shifts from the jugular vein to the VVP outflow pathway. The venous outflow shift mechanism and its purpose are poorly understood. The authors review the known physiology of the system, identify gaps in knowledge to direct future research, and propose an interpretation of these data, concluding that position-dependent CSF and cerebrospinal venous shifts are part of a complementary positional craniospinal pressure regulation system that must be kept in balance for optimal CNS function., Conclusions: Current knowledge of the cerebrospinal venous anatomy, dynamic flow characteristics in response to gravity, and the venous system's influence on CSF suggests that the VVP plays a role in influencing CSF pressure, and the authors hypothesize that it plays a role in supporting intracranial pressure in the upright body posture. Further research is needed to better characterize the functional relationship of the VVP to CSF dynamics as well as identify potentially related disease states.

Background: SINO syndrome (Spastic paraplegia, Intellectual disability, Nystagmus and Obesity) is a rare autosomal dominant condition caused by heterozygous variants in KIDINS220. A total of 12 individuals are reported, comprising eight with SINO and four with an autosomal recessive condition attributed to bi-allelic KIDINS220 variants., Methods: In our international cohort, we have comprised 14 individuals, carrying 13 novel pathogenic KIDINS220 variants in heterozygous form. We assessed clinical and molecular data of our cohort and previously reported individuals and based on functional experiments reached a better understanding of the pathogenesis behind KIDINS220-related disease., Results: Using fetal tissue and in vitro assays, we demonstrate that the variants generate KIDINS220 truncated forms that mislocalize in punctate intracellular structures, with decreased levels of the full-length protein, suggesting a trans-dominant negative effect. 92% had their diagnosis within three years, with symptoms of developmental delay, spasticity, hypotonia, lack of eye contact and nystagmus. We identified a KIDINS220 variant associated with fetal hydrocephalus and show that 58% of examined individuals present brain ventricular dilatation. We extend the phenotypic spectrum of SINO syndrome to behavioral manifestations not previously highlighted., Conclusion: Our study provides further insights into the clinical spectrum, etiology and predicted functional impact of KIDINS220 variants., (Copyright © 2024. Published by Elsevier Inc.)

Background: With transradial access (TRA) being more progressively used in neuroendovascular procedures, we compared TRA with transfemoral access (TFA) in middle meningeal artery embolization (MMAE) for chronic subdural hematoma (cSDH)., Methods: Consecutive patients undergoing MMAE for cSDH at 14 North American centers (2018-23) were included. TRA and TFA groups were compared using propensity score matching (PSM) controlling for: age, sex, concurrent surgery, previous surgery, hematoma thickness and side, midline shift, and pretreatment antithrombotics. The primary outcome was access site and overall complications, and procedure duration; secondary endpoints were surgical rescue, radiographic improvement, and technical success and length of stay., Results: 872 patients (median age 73 years, 72.9% men) underwent 1070 MMAE procedures (54% TFA vs 46% TRA). Access site hematoma occurred in three TFA cases (0.5%; none required operative intervention) versus 0% in TRA (P=0.23), and radial-to-femoral conversion occurred in 1% of TRA cases. TRA was more used in right sided cSDH (58.4% vs 44.8%; P<0.001). Particle embolics were significantly higher in TFA while Onyx was higher in TRA (P<0.001). Following PSM, 150 matched pairs were generated. Particles were more utilized in the TFA group (53% vs 29.7%) and Onyx was more utilized in the TRA group (56.1% vs 31.5%) (P=0.001). Procedural duration was longer in the TRA group (median 68.5 min (IQR 43.1-95) vs 59 (42-84); P=0.038), and radiographic success was higher in the TFA group (87.3% vs 77.4%; P=0.036). No differences were noted in surgical rescue (8.4% vs 10.1%, P=0.35) or technical failures (2.4% vs 2%; P=0.67) between TFA and TRA. Sensitivity analysis in the standalone MMAE retained all associations but differences in procedural duration., Conclusions: In this study, TRA offered comparable outcomes to TFA in MMAE for cSDH in terms of access related and overall complications, technical feasibility, and functional outcomes. Procedural duration was slightly longer in the TRA group, and radiographic success was higher in the TFA group, with no differences in surgical rescue rates., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Objectives: To present a case series of novel CHD2 variants in patients presenting with genetic epileptic and developmental encephalopathy., Background: CHD2 gene encodes an ATP-dependent enzyme, chromodomain helicase DNA-binding protein 2, involved in chromatin remodeling. Pathogenic variants in CHD2 are linked to early-onset conditions such as developmental and epileptic encephalopathy, drug-resistant epilepsies, and neurodevelopmental disorders. Approximately 225 diagnosed patients from 28 countries exhibit various allelic variants in CHD2, including small intragenic deletions/insertions and missense, nonsense, and splice site variants., Results: We present the molecular and clinical characteristics of 17 unreported individuals from 17 families with novel pathogenic or likely pathogenic variants in CHD2 . All individuals presented with severe global developmental delay, childhood-onset myoclonic epilepsy, and additional neuropsychiatric features, such as behavioral including autism, ADHD, and hyperactivity. Additional findings include abnormal reflexes, hypotonia and hypertonia, motor impairment, gastrointestinal problems, and kyphoscoliosis. Neuroimaging features included hippocampal signal alterations (4/10), with additional volume loss in 2 cases, inferior vermis hypoplasia (7/10), mild cerebellar atrophy (4/10), and cerebral atrophy (1/10)., Discussion: Our study broadens the geographic scope of CHD2-related phenotypes, providing valuable insights into the prevalence and clinical characteristics of this genetic disorder in previously underrepresented populations., Competing Interests: P. Striano received support from Italian MoH (Ricerca Corrente 2023) and Fondazione San Paolo. Research supported by PNRR-MUR-M4C2 PE0000006 Research Program MNESYS—A multiscale integrated approach to the study of the nervous system in health and disease. IRCCS G. Gaslini is a member of ERN-Epicare. Go to Neurology.org/NG for full disclosures., (Copyright © 2024 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.)

We review the technique for carotid endarterectomy (CEA) and direct carotid access for distal thrombectomy after attempted proximal thrombectomy in the setting of tandem occlusions. A patient in their 70s presented with right facial droop and drooling and was found to have critical left carotid stenosis with filling defect in the cavernous segment of the left internal carotid artery consistent with vessel occlusion, Thrombolysis in Cerebral Infarction (TICI) 0, and left M2 middle cerebral artery (MCA) occlusion. After multiple attempts with different wire shapes guided by microcatheter injections within the carotid bulb, we were unable to cross the occlusion. Conversion to open CEA with distal thrombectomy was elected. Following closure of the arteriotomy, direct carotid access using a 5Fr radial artery sheath was achieved within the open surgical field for distal thrombectomy. A 5Fr aspiration catheter was navigated to the left M2 MCA where a stent retriever was then recaptured and TICI 2B reperfusion was achieved., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: Posterior fossa arteriovenous malformations (AVMs) represent 7% to 15% of all intracranial AVMs and are associated with an increased risk of hemorrhage, morbidity, and mortality compared with supratentorial AVMs, thus prompting urgent and definitive treatment. Cerebellopontine angle (CPA) AVMs are a unique group of posterior fossa AVMs incorporating characteristics of brainstem and cerebellar lesions, which are particularly amenable to microsurgical resection. This study reports the clinical, radiological, operative, and outcome features of patients with CPA AVMs in a large cohort., Methods: The authors conducted a single-surgeon, 2-institution retrospective cohort study of all consecutive patients with CPA AVMs treated with microsurgical resection during a 25-year period., Results: CPA AVMs represented 22% (38 of 176) of all infratentorial AVMs resected by the senior author. Overall, 38 patients (22 [58%] male and 16 [42%] female) met the study inclusion criteria and were analyzed. Most patients presented with hemorrhage (n = 29, 76%). The median age at surgery was 56 (range 6-82) years. Subtypes included 22 (58%) petrosal cerebellar AVMs, 11 (29%) lateral pontine AVMs, and 5 (13%) AVMs involving both the brainstem and cerebellum. Most AVM niduses were small (< 3 cm; n = 35, 92%) and compact (n = 31, 82%). Fourteen (37%) patients harbored flow-related aneurysms. Twenty (53%) patients underwent preoperative embolization. Complete angiographic obliteration was achieved with microsurgery in 35 (92%) patients. Five (13%) patients with poor neurological conditions at presentation died before hospital discharge. Of the 7 (18%) patients with new postoperative neurological deficits, 5 had transient deficits. The median (interquartile range) follow-up was 1.7 (0.5-3.2) years; 32 (84%) patients were alive at last follow-up, and 30 (79%) had achieved a favorable neurological outcome (modified Rankin Scale [mRS] score 0-2). The only independent predictor of unfavorable postoperative outcome (mRS score 3-6) was the preoperative mRS score (p = 0.002)., Conclusions: CPA AVMs are unique posterior fossa lesions, including petrosal cerebellar and lateral pontine AVMs. The "backdoor resection" technique provides a safe and efficient strategy with high obliteration rates and a low risk of treatment-related morbidity. Microsurgical resection should be considered the frontline treatment for most CPA AVMs, except for those with a significant diffuse brainstem component.

Transcirculation catheterization, also known as the retrograde approach, involves the navigation of a catheter or other endovascular device from one arterial circulation to the other (right to left, or anterior to posterior).1-4 We present a case of a complex vertebrobasilar junction aneurysm previously treated by bilateral vertebral artery deconstruction, precluding antegrade access (video 1). Following the creation of a protective occipital artery to posterior inferior cerebellar artery (PICA) bypass, the patient was treated with transcirculation placement of a Pipeline embolization device (PED).5-9 The right internal carotid artery was accessed with a guide catheter using a transradial approach. The microwire-microcatheter combination was then tracked through the right posterior communicating artery, down the basilar trunk, and to the left PICA. The PED was successfully deployed from the left vertebral artery to the mid-basilar artery. At 3-month follow-up, the aneurysm was completely obliterated. The nuances of transcirculation technique, especially for flow diversion, are discussed. (Used with permission from Barrow Neurological Institute, Phoenix, Arizona, USA.)neurintsurg;jnis-2023-021363v1/V1F1V1Video 1Transcirculation retrograde placement of a Pipeline embolization device for treatment of a vertebrobasilar junction aneurysm previously treated by bilateral vertebral artery deconstruction, precluding antegrade access., Competing Interests: Competing interests: AFD and FCA serve on the Editorial Board of the Journal of NeuroInterventional Surgery (JNIS)., (© Author(s) (or their employer(s)) 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Based on a limited number of reported families, biallelic CA8 variants have currently been associated with a recessive neurological disorder named, cerebellar ataxia, mental retardation, and dysequilibrium syndrome 3 (CAMRQ-3)., Objectives: We aim to comprehensively investigate CA8-related disorders (CA8-RD) by reviewing existing literature and exploring neurological, neuroradiological, and molecular observations in a cohort of newly identified patients., Methods: We analyzed the phenotype of 27 affected individuals from 14 families with biallelic CA8 variants (including data from 15 newly identified patients from eight families), ages 4 to 35 years. Clinical, genetic, and radiological assessments were performed, and zebrafish models with ca8 knockout were used for functional analysis., Results: Patients exhibited varying degrees of neurodevelopmental disorders (NDD), along with predominantly progressive cerebellar ataxia and pyramidal signs and variable bradykinesia, dystonia, and sensory impairment. Quadrupedal gait was present in only 10 of 27 patients. Progressive selective cerebellar atrophy, predominantly affecting the superior vermis, was a key diagnostic finding in all patients. Seven novel homozygous CA8 variants were identified. Zebrafish models demonstrated impaired early neurodevelopment and motor behavior on ca8 knockout., Conclusion: Our comprehensive analysis of phenotypic features indicates that CA8-RD exhibits a wide range of clinical manifestations, setting it apart from other subtypes within the category of CAMRQ. CA8-RD is characterized by cerebellar atrophy and should be recognized as part of the autosomal-recessive cerebellar ataxias associated with NDD. Notably, the presence of progressive superior vermis atrophy serves as a valuable diagnostic indicator. © 2024 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society., (© 2024 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.)

Background: Melphalan, which is poorly soluble at room temperature, is widely used for the treatment of retinoblastoma by selective ophthalmic artery infusion. Evomela, a propylene glycol-free formulation of melphalan with improved solubility and stability, has recently been used as an alternative.To compare the safety and efficacy of Evomela with standard-formulation melphalan (SFM) in the treatment of retinoblastoma by selective ophthalmic artery infusion., Methods: We performed a retrospective case-control study of patients with retinoblastoma undergoing selective ophthalmic artery infusion with SFM or Evomela at a single institution. Cycle-specific percent tumor regression (CSPTR) was estimated by comparing photos obtained during pretreatment examination under anesthesia (EUA) with those obtained during post-treatment EUA 3-4 weeks later. CSPTR, ocular salvage rates, complication rates, operation times (unadjusted and adjusted for difficulty of ophthalmic artery catheterization), and intraprocedural dose expiration rates were compared between Evomela- and SFM-treated groups. Univariate and multivariate analyses were performed., Results: Ninety-seven operations (melphalan: 45; Evomela: 52) for 23 patients with 27 retinoblastomas were studied. The ocular salvage rate was 79% in the SFM-treated group and 69% in the Evomela-treated group. Multivariate regression controlling for tumor grade, patient age, and treatment history revealed no significant differences in ocular salvage rate, CSPTR, complication rates, or operation times. Although the dose expiration rate was higher for the SFM-treated group, the difference was not statistically significant. Notably, there were no ocular or cerebral ischemic complications., Conclusion: Evomela has non-inferior safety and efficacy relative to SFM when used for the treatment of retinoblastoma by selective ophthalmic artery infusion., Competing Interests: Competing interests: FCA is presently on the editorial board of the Journal of Neurointerventional Surgery., (© Author(s) (or their employer(s)) 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Flow diversion is a unique interventional tool with evolving roles in the treatment of intracranial aneurysms.1 Although flow diversion strategies can be highly effective in appropriately selected patients, their off-label use is controversial. As flow diversion indications have expanded, so has the incidence of treatment failure, resulting in an evolving subgroup of patients with atypical lesions that require complex salvage strategies, such as cerebrovascular bypass.2,3 We report a residual dolichoectatic superior cerebellar artery aneurysm in which flow diversion failed, which was treated through superficial temporal artery to superior cerebellar artery bypass.4,5 Being a single case report, institutional review board approval was not needed. Patient consent was obtained. Used with permission from Barrow Neurological Institute, Phoenix, Arizona., (Copyright © Congress of Neurological Surgeons 2024. All rights reserved.)

Background: Disorders of consciousness impair early recovery after aneurysmal subarachnoid hemorrhage (aSAH). Modafinil, a wakefulness-promoting agent, is efficacious for treating fatigue in stroke survivors, but data pertaining to its use in the acute setting are scarce. This study sought to assess the effects of modafinil use on mental status after aSAH., Methods: Modafinil timing and dosage, neurological examination, intubation status, and physical and occupational therapy participation were documented. Repeated-measures paired tests were used for a before-after analysis of modafinil recipients. Propensity score matching (1:1 nearest neighbor) for modafinil and no-modafinil cohorts was used to compare outcomes., Results: Modafinil (100-200 mg/day) was administered to 21% (88/422) of aSAH patients for a median (IQR) duration of 10.5 (4-16) days and initiated 14 (7-17) days after aSAH. Improvement in mentation (alertness, orientation, or Glasgow Coma Scale score) was documented in 87.5% (77/88) of modafinil recipients within 72 hours and 86.4% (76/88) at discharge. Of 37 intubated patients, 10 (27%) were extubated within 72 hours after modafinil initiation. Physical and occupational therapy teams noted increased alertness or participation in 47 of 56 modafinil patients (83.9%). After propensity score matching for baseline covariates, the modafinil cohort had a greater mean (SD) change in Glasgow Coma Scale score than the no-modafinil cohort at discharge (2.2 [4.0] vs. -0.2 [6.32], P = 0.003)., Conclusions: A temporal relationship with improvement in mental status was noted for most patients administered modafinil after aSAH. These findings, a favorable adverse-effect profile, and implications for goals-of-care decisions favor a low threshold for modafinil initiation in aSAH patients in the acute-care setting., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Galenic dural arteriovenous fistulas account for 1 of the 6 types of tentorial dural arteriovenous fistulas (type I). These fistulas are located around the great cerebral vein of Galen, with dural arterial supply through the tentorial arteries of Bernasconi and Cassinari or other posterior fossa branches, such as the posterior meningeal artery. In this case, a man in his 60s presented with a headache and was found to have this high-risk lesion, which was Borden grade III and Cognard grade IV. The lesion persisted despite embolization through the middle meningeal artery. This video discusses the microsurgical management of this lesion through a torcular craniotomy and a posterior interhemispheric approach for clipping. Because this is a single case report, institutional review board approval was not needed. The patient consented to the procedure. Used with permission from Barrow Neurological Institute, Phoenix, Arizona. Images at 7:19 and 7:49 in Surgical Video are used with permission from Lawton, Michael T.; Sanchez-Mejia, Rene O.; Pham, Diep; Tan, Jeffrey; Halbach, Van V. Tentorial Dural Arteriovenous Fistulae: Operative Strategies and Microsurgical Results for Six Types. Operative Neurosurgery 62(3): 110-125, 2008., (Copyright © Congress of Neurological Surgeons 2024. All rights reserved.)

Introduction: Distal navigability and imprecise delivery of embolic agents are two limitations encountered during liquid embolization of cerebrospinal lesions. The dual-lumen Scepter Mini balloon (SMB) microcatheter was introduced to overcome these conventional microcatheters' limitations with few small single-center reports suggesting favorable results., Methods: A series of consecutive patients undergoing SMB-assisted endovascular embolization were extracted from prospectively maintained registries in seven North-American centers (November 2019 to September 2022)., Results: Fifty-four patients undergoing 55 embolization procedures utilizing SMB were included (median age 58.5; 48.1% females). Cranial dural arteriovenous fistula embolization was the most common indication (54.5%) followed by cranial arteriovenous malformation (27.3%). Staged/pre-operative embolization was done in 36.4% of cases; and 83.6% of procedures using Onyx-18. Most procedures utilized a transarterial approach (89.1%), and SMB-induced arterial-flow arrest concurrently with transvenous embolization was used in 10.9% of procedures. Femoral access/triaxial setups were utilized in the majority of procedures (65.5% and 60%, respectively). The median vessel diameter where the balloon was inflated of 1.8 mm, with a median of 1.5 cc of injected embolic material per procedure. Technical failures occurred in 5.5% of cases requiring aborting/replacement with other devices without clinical sequelae in any of the patients, with SMB-related procedural complications of 3.6% without clinical sequelae. Radiographic imaging follow-up was available in 76.9% of the patients (median follow-up 3.8 months), with complete occlusion (100%) or >50% occlusion in 92.5% of the cases, and unplanned retreatments in 1.8%., Conclusion: The SMB microcatheter is a useful new adjunctive device for balloon-assisted embolization of cerebrospinal lesions with a high technical success rate, favorable outcomes, and a reasonable safety profile., Competing Interests: Declaration of conflicting interestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Competing Interests: Competing interests: None declared.

Background: Chronic subdural hematoma (CSDH) often requires surgical evacuation, but recurrence rates remain high. Middle meningeal artery (MMA) embolization (MMAE) has been proposed as an alternative or adjunct treatment. There is concern that prior surgery might limit patency, access, penetration, and efficacy of MMAE, such that some recent trials excluded patients with prior craniotomy. However, the impact of prior open surgery on MMA patency has not been studied., Methods: A retrospective analysis was conducted on patients who underwent MMAE for cSDH (2019-2022), after prior surgical evacuation or not. MMA patency was assessed using a six-point grading scale., Results: Of the 109 MMAEs (84 patients, median age 72 years, 20.2% females), 58.7% were upfront MMAEs, while 41.3% were after prior surgery (20 craniotomies, 25 burr holes). Median hematoma thickness was 14 mm and midline shift 3 mm. Hematoma thickness reduction, surgical rescue, and functional outcome did not differ between MMAE subgroups and were not affected by MMA patency or total area of craniotomy or burr-holes. MMA patency was reduced in the craniotomy group only, specifically in the distal portion of the anterior division (p = 0.005), and correlated with craniotomy area (p < 0.001)., Conclusion: MMA remains relatively patent after burr-hole evacuation of cSDH, while craniotomy typically only affects the frontal-distal division. However, MMA patency, evacuation method, and total area do not affect outcomes. These findings support the use of MMAE regardless of prior surgery and may influence future trial inclusion/exclusion criteria. Further studies are needed to optimize the timing and techniques for MMAE in cSDH management., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Introduction: Endothelial cells (ECs) continuously line the cerebrovasculature. Molecular aberrations in the ECs are hallmarks and contributory factors to the development of cerebrovascular diseases, including intracranial aneurysms and arteriovenous malformations (AVMs). Endovascular biopsy has been introduced as a method to harvest ECs and obtain relevant biologic information. We aimed to summarize the literature on endovascular biopsy in neurointerventional surgery., Methods: We conducted a comprehensive literature search in multiple databases, identifying eligible studies focusing on neurosurgical applications of endovascular biopsy. The systematic review followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. The relevant information was collected, including study characteristics, biopsy techniques, and key findings., Results: Nine studies met the inclusion criteria and were included. The studies involved the collection of ECs using various endovascular devices including coils, guide wires, different stents, and forceps. Endothelial-enrichment techniques, such fluorescence-activated cell sorting (FACS), collected ECs and facilitated downstream applications of bulk or single-cell RNA sequencing (scRNAseq). The studies provided insights into gene expression profiles and identified potential biomarkers associated with intracranial aneurysms. However, challenges were observed in obtaining an adequate number of ECs and identifying consistent biomarkers., Conclusion: Endovascular biopsy of endothelial cells (ECs) in cerebrovascular pathologies shows promise for gene expression profiling. However, many studies have been limited in sample size and underpowered to identify "signature genes" for aneurysm growth or rupture. Advancements in minimally invasive biopsy methods have potential to facilitate applications of precision medicine in the treatment of cerebrovascular disorders., Competing Interests: Declaration of conflicting interestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Flow-diverting devices (FDDs), such as the Pipeline Embolization Device, have been gaining traction for treating challenging posterior circulation aneurysms. Few previous studies have focused on using FDDs to treat aneurysms of the basilar quadrifurcation., Methods: We retrospectively reviewed the use of FDDs to treat patients with basilar quadrifurcation aneurysms. Patients were assessed for aneurysm type, previous aneurysm treatment, technical success, periprocedural complications, and long-term aneurysm occlusion., Results: 34 patients were assessed; aneurysms of the basilar apex (n=23) or superior cerebellar artery (SCA) (n=7), or both (n=1), and posterior cerebral artery (PCA) (n=3). The mean (SD) largest aneurysm dimension was 8.7 (6.1) mm (range 1.9-30.8 mm). 14 aneurysms were previously surgically clipped or endovascularly coiled. All aneurysms had a saccular morphology. Complete or near-complete occlusion was achieved in 30 of 34 patients (88%) at final angiographic follow-up, a mean (SD) of 6.6 (5.4) months (range 0-19 months) postoperatively. No patient experienced postoperative symptomatic occlusions of the SCA or PCA; 4 patients developed asymptomatic posterior communicating artery occlusions; 28 patients (82%) experienced no complications; whereas 3 (9%) experienced major complications and 3 (9%) experienced minor complications; and 1 patient died as a result of subarachnoid hemorrhage., Conclusion: Flow diversion may be a safe and effective option to treat basilar quadrifurcation aneurysms. Previously treated basilar quadrifurcation aneurysms with recurrence or residual lesion may benefit from additional treatment with an FDD. Further prospective studies should be directed toward validating these findings., Competing Interests: Competing interests: Dr Ducruet is a consultant for Penumbra, Medtronic, Stryker, Cerenovus, and Koswire. Drs Ducruet and Albuquerque serve on the editorial board of the Journal of NeuroInterventional Surgery. Content included in this article was presented as a digital poster at the American Association of Neurological Surgeons Annual Meeting, April 29–May 1, 2022, in Philadelphia, PA., (© Author(s) (or their employer(s)) 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Controversy remains regarding the appropriate screening for intracranial aneurysms or for the treatment of aneurysmal subarachnoid hemorrhage (aSAH) for patients without known high-risk factors for rupture. This study aimed to assess how sex affects both aSAH presentation and outcomes for aSAH treatment., Method: A retrospective cohort study was conducted of all patients treated at a single institution for an aSAH during a 12-year period (August 1, 2007-July 31, 2019). An analysis of women with and without high-risk factors was performed, including a propensity adjustment for a poor neurologic outcome (modified Rankin Scale [mRS] score > 2) at follow-up., Results: Data from 1014 patients were analyzed (69% [n = 703] women). Women were significantly older than men (mean ± SD, 56.6 ± 14.1 years vs 53.4 ± 14.2 years, p < 0.001). A significantly lower percentage of women than men had a history of tobacco use (36.6% [n = 257] vs 46% [n = 143], p = 0.005). A significantly higher percentage of women than men had no high-risk factors for aSAH (10% [n = 70] vs 5% [n = 16], p = 0.01). The percentage of women with an mRS score > 2 at the last follow-up was significantly lower among those without high-risk factors (34%, 24/70) versus those with high-risk factors (53%, 334/633) (p = 0.004). Subsequent propensity-adjusted analysis (adjusted for age, Hunt and Hess grade, and Fisher grade) found no statistically significant difference in the odds of a poor outcome for women with or without high-risk factors for aSAH (OR = 0.7, 95% CI = 0.4-1.2, p = 0.18)., Conclusions: A higher percentage of women versus men with aSAH had no known high-risk factors for rupture, supporting more aggressive screening and management of women with unruptured aneurysms., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Austria, part of Springer Nature.)

Background and Objectives: The choice of anesthesia type (general anesthesia [GA] vs nongeneral anesthesia [non-GA]) in middle meningeal artery embolization (MMAE) procedures for chronic subdural hematomas (cSDH) differs between institutions and left to care team discretion given lack of standard guidelines. We compare the outcomes of GA vs non-GA in MMAE., Methods: Consecutive patients receiving MMAE for cSDH at 14 North American centers (2018-2023) were included. Clinical, cSDH characteristics, and technical/clinical outcomes were compared between the GA/non-GA groups. Using propensity score matching (PSM), patients were matched controlling for age, baseline modified Rankin Scale, concurrent/prior surgery, hematoma thickness/midline shift, and baseline antiplatelet/anticoagulation. The primary end points included surgical rescue and radiographic success rates (≥50% reduction in maximum hematoma thickness with minimum 2 weeks of imaging). Secondary end points included technical feasibility, procedural complications, and functional outcomes., Results: Seven hundred seventy-eight patients (median age 73 years, 73.2% male patients) underwent 956 MMAE procedures, 667 (70.4%) were non-GA and 280 were GA (29.6%). After running 1:3 PSM algorithm, this resulted in 153 and 296 in the GA and non-GA groups, respectively. There were no baseline/procedural differences between the groups except radial access more significantly used in the non-GA group (P = .001). There was no difference between the groups in procedural technical feasibility, complications rate, length of stay, surgical rescue rates, or favorable functional outcome at the last follow-up. Subsequent 1:1 sensitivity PSM retained the same results. Bilateral MMAE procedures were more performed under non-GA group (75.8% vs 67.2%; P = .01); no differences were noted in clinical/radiographic outcomes between bilateral vs unilateral MMAE, except for longer procedure duration in the bilateral group (median 73 minutes [IQR 48.3-100] vs 54 minutes [39-75]; P < .0001). Another PSM analysis comparing GA vs non-GA in patients undergoing stand-alone MMAE retained similar associations., Conclusion: We found no significant differences in radiological improvement/clinical outcomes between GA and non-GA for MMAE., (Copyright © Congress of Neurological Surgeons 2024. All rights reserved.)

Introduction: The internet is an essential resource for patients and their loved ones to understand their medical conditions, and professional medical organizations have taken great strides to develop educational material targeting patients. The average American reads at a seventh to eighth grade reading level, hence it is important to understand the readability of this medical information to ensure patients comprehend what is being presented., Methods: In January 2023, online patient education material was downloaded from major cerebrovascular healthcare organizations and assessed using eight assessments, including Bormuth Cloze Mean, Bormuth Grade Placement, Coleman-Liau (grade levels), Coleman-Liau (predictive cloze scores), Flesch Reading Ease (FRE), and Fry., Results: A total of 32 files were extracted from six organizations and analyzed across 15 readability measures. None of the organizations met the federal government guidelines for grade-level readability. This held constant across all measured tests. Two organizations had above a postgraduate level. The FRE graphs do not identify any organizations with material below a ninth grade reading level, while the American Association of Neurological Surgeons (AANS) and the Society of Interventional Radiology (SIR) have a postgraduate readability level. The Fry graphs show similar results, with AANS/CNS Cerebrovascular Section, Society of NeuroInterventional Surgery (SNIS), SIR, and AANS having college-level readability. The lowest readability across all measures is only at an early seventh grade reading level., Conclusions: Current health literacy content for cerebrovascular patients is far above the recommended readability level. We provide straightforward suggestions for how major professional organizations should improve their informational material on cerebrovascular diseases to improve patient understanding., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Introduction: Increasing life expectancy has caused growing concern about maintaining viable neurointerventional practices due to altered Medicare payment structures. This study analyzes the financial trends of three common diagnostic tests for cerebrovascular disease: cerebral digital subtraction angiography (DSA), computed tomography angiography (CTA), and magnetic resonance angiography (MRA)., Methods: Medicare Part B National Summary Data files from 2013 to 2020 were queried by Current Procedural Terminology (CPT) codes for DSA (36221-36228), CTA (70496, 70498), and MRA (70544-70547, 70549). Inflation-adjusted charges and reimbursement were calculated using the U.S. City Average Consumer Price Index for Medical Services. Regression analysis was performed on charges, reimbursement, and volume., Results: A total of 1,519,245 diagnostic procedures were conducted between 2013 and 2020 (782,370 angiograms, 246,603 CTAs, and 490,272 MRAs). A total of $41.005 million was reimbursed by Medicare in 2020 for these diagnostic procedures. The annual percent change in volume for all procedures was -2.90%. From 2013 to 2020, inflation-adjusted: Medicare total physician reimbursement decreased for cerebral angiograms (-4.12%, p = 0.007), CTAs (-2.77%, p = 0.458), and MRAs (-9.06%, p < 0.001). Procedural volume billed to Medicare decreased for cerebral angiograms (-4.63%, p = 0.007) and MRAs (-2.94%, p = 0.0.81) and increased for CTAs (+3.15%, p = 0.004). The greatest increase in Medicare reimbursement (+66.75%) came from CPT code 36224, "place catheter carotid artery", and the greatest decrease in Medicare reimbursement (-8.66%) came from CPT code 36226, "place catheter vertebral artery.", Conclusions: This study provides an analysis of Medicare reimbursement trends for routine cerebrovascular angiogram techniques. The findings highlight a decline in Medicare reimbursements for neurointerventionalists., Competing Interests: Declaration of conflicting interestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

We report the third case of FADS due to biallelic DOK7 variants, which further strengthens the association of DOK7 with this lethal phenotype and lack of genotype phenotype correlation., (© 2023 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Background and Objectives: Revascularizing the postcommunicating segment of the anterior cerebral artery (ACA) using extracranial donor sites requires long interposition grafts. The superficial temporal artery (STA) is frequently used for extracranial-intracranial ACA revascularization. However, the length of either STA branch is not sufficient to reach the ACA with a proper caliber match, so an interposition graft is required. The aim of this study was to evaluate a bypass that uses the 2 main branches of the STA to reach the A3 (pericallosal) segment of the ACA., Methods: The frontal and parietal branches of the STA were dissected from 10 cadaveric specimens. The middle internal frontal artery (MIFA) was exposed through an anterior interhemispheric approach. An interposition graft technique was applied using the parietal branch of the STA (pSTA) to connect the frontal branch of the STA (fSTA) with the MIFA. The bypass code is fSTA (E-Ec) pSTA + pSTA (E-Sc) MIFA. Measurements of length and caliber were taken at the anastomotic sites for the distal branches of the STA and the MIFA., Results: The mean (SD) diameter of the MIFA measured 1.4 (0.2) mm, similar to the calibers of the frontal and parietal branches of the STA. The mean (SD) length of the end-to-side STA-MIFA bypass was 145.5 (7.4) mm, and the mean (SD) length of the donor-graft construct measured 204.2 (27.9) mm. This bypass design resulted in a surplus donor graft length of 38%., Conclusion: Using the pSTA as an interposition graft proved to be a successful technique for creating an STA-MIFA bypass, yielding excess donor graft length that facilitated an unstrained bypass construct. This approach offers several advantages, including a single skin incision, ample graft length, caliber compatibility, and a straightforward technical execution., (Copyright © Congress of Neurological Surgeons 2024. All rights reserved.)