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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.

Purpose: Mechanical thrombectomy (MT) is typically performed by experienced neurointerventional radiologists. However, logistical and geographic limitations often hinder access to rapid MT. This study reports the first clinical experience using TEGUS teleproctoring to support MT conducted by general interventional radiologists (IR) at non-comprehensive stroke centers, compared to on-site proctoring outcomes., Methods: The Arnau de Vilanova Hospital in Spain used to transfer stroke patients requiring MT to a comprehensive Stroke Center 160 km away. To overcome COVID-19 mobility restrictions, the Tegus Teleproctoring System was installed. Before teleproctoring, the general interventional radiologist underwent six months of neurointerventional training at a primary stroke center. From April 2021 to May 2023, general IR conducted MT either with on-site proctor supervision or teleproctoring support. We aim to compare clinical outcome of patients receiving MT according to proctoring method., Results: During the study, 49 MTs were performed: 15 with TEGUS teleproctoring and 34 with on-site proctoring. Both groups had similar baseline characteristics, except for NIHSS scores (Tegus 9 [IQR 6-20] vs 18 [IQR 12-22], p = 0.034). No significant differences were found in door-to-revascularization time (82 ± 28.2 vs 84 ± 26.4) min, p = (0.895). The final mTICI distribution and 90-day mRS scores were comparable after adjusting by stroke severity. There were no reports of symptomatic intracranial hemorrhage in either group., Conclusion: This study shows the feasibility of Tegus remote teleproctoring during emergent cases of MT in a remote hospital. It could improve the learning curve of interventional radiologists with limited experience in MT, and lower the territorial inequity associated to MT., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany.)

Purpose: We aim to propose a visual quantitative score for muscle edema in lower limb MRI to contribute to the diagnosis of idiopathic inflammatory myopathy (IIM)., Material and Methods: We retrospectively evaluated 85 consecutive patients (mean age 57.4 ± 13.9 years; 56.5% female) with suspected IIM (muscle weakness and/or persistent hyper-CPK-emia with/without myalgia) who underwent MRI of lower limbs using T2-weighted fast recovery-fast spin echo images and fat-sat T2 echo planar images. Muscle inflammation was evaluated bilaterally in 11 muscles of the thigh and eight muscles of the leg. Edema in each muscle was graded according to a four-point Likert-type scale adding up to 114 points ([11 + 8)] × 3 × 2). Diagnostic accuracy of the total edema score was explored by assessing sensitivity and specificity using the area under the ROC curve. Final diagnoses were made by a multidisciplinary Expert Consensus Panel applying the Bohan and Peter diagnostic criteria whenever possible., Results: Of the 85 included patients, 34 (40%) received a final diagnosis of IIM (IIM group) while 51 (60%) received an alternative diagnosis (non-IIM group). A cutoff score ≥ 18 was able to correctly classify patients having an IIM with an area under the curve of 0.85, specificity of 96%, and sensitivity of 52.9%., Conclusion: Our study demonstrates that a quantitative MRI score for muscle edema in the lower limbs (thighs and legs) aids in distinguishing IIM from conditions that mimic it., (© 2024. The Author(s).)

Background and Purpose: Endovascular robotic devices may enable experienced neurointerventionalists to remotely perform endovascular thrombectomy. This study aimed to assess the feasibility, safety, and efficacy of robot-assisted endovascular thrombectomy compared with manual procedures by operators with varying levels of experience, using a 3D printed neurovascular model., Materials and Methods: M1 MCA occlusions were simulated in a 3D printed neurovascular model, linked to a CorPath GRX robot in a biplane angiography suite. Four interventionalists performed manual endovascular thrombectomy ( n  = 45) and robot-assisted endovascular thrombectomy ( n  = 37) procedures. The outcomes included first-pass recanalization (TICI 2c-3), the number and size of generated distal emboli, and procedural length., Results: A total of 82 experimental endovascular thrombectomies were conducted. A nonsignificant trend favoring the robot-assisted endovascular thrombectomy was observed in terms of final recanalization (89.2% versus manual endovascular thrombectomy, 71.1%; P  = .083). There were no differences in total mean emboli count (16.54 [SD, 15.15] versus 15.16 [SD, 16.43]; P  = .303). However, a higher mean count of emboli of > 1 mm was observed in the robot-assisted endovascular thrombectomy group (1.08 [SD, 1.00] versus 0.49 [SD, 0.84]; P  = .001) compared with manual endovascular thrombectomy. The mean procedural length was longer in robot-assisted endovascular thrombectomy (6.43 [SD, 1.71] minutes versus 3.98 [SD, 1.84] minutes; P  < .001). Among established neurointerventionalists, previous experience with robotic procedures did not influence recanalization (95.8% were considered experienced; 76.9% were considered novices; P  = .225)., Conclusions: In a 3D printed neurovascular model, robot-assisted endovascular thrombectomy has the potential to achieve recanalization rates comparable with those of manual endovascular thrombectomy within competitive procedural times. Optimization of the procedural setup is still required before implementation in clinical practice., (© 2024 by American Journal of Neuroradiology.)

Background: To describe high-resolution brain vessel wall MRI (VW-MRI) patterns and morphological brain findings in central nervous system (CNS) vasculitis patients., Methods: Fourteen patients with confirmed CNS Vasculitis from two tertiary centers underwent VW-MRI using a 3T scanner. The images were reviewed by two neuroradiologists to assess vessel wall enhancement characteristics and locations., Results: Fourteen patients were included (six females; average age 48 ± 19 years). Diagnoses included primary CNS vasculitis (PCNSV) in six patients and secondary CNS vasculitis (SCNSV) in eight, half of which were infection-related. Thirteen patients showed vessel wall enhancement, which was intense in eleven patients (84.6%) and concentric in twelve (92.3%), affecting the anterior circulation in nine patients (69.2%), posterior in two patients (15.4%), and both circulations in two patients (15.4%). The enhancement patterns were similar across different CNS vasculitis types. DWI changes corresponded with areas of vessel wall enhancement in 77% of patients. Conclusions : CNS vasculitis is often associated with intense, concentric vessel wall enhancement in VW-MRI, especially in the anterior circulation. The consistent presence of DWI alterations in affected territories suggests a possible link to microembolization or hypoperfusion. These imaging findings complement parenchymal brain MRI and MRA/DSA data, potentially increasing the possibility of a clinical diagnosis of CNS vasculitis.

Background: Transfemoral access is predominantly used for mechanical thrombectomy in patients with stroke with a large vessel occlusion. Following the interventional cardiology guidelines, routine transradial access has been proposed as an alternative, although its safety and efficacy remain controversial. We aim to explore the noninferiority of radial access in terms of final recanalization., Methods: The study was an investigator-initiated, single-center, evaluator-blinded, noninferiority randomized clinical trial. Patients with stroke undergoing mechanical thrombectomy, with a patent femoral artery and a radial artery diameter ≥2.5 mm, were randomly assigned (1:1) to either transradial (60 patients) or transfemoral access (60 patients). The primary binary outcome was the successful recanalization (expanded Treatment in Cerebral Ischemia score, 2b-3) assigned by blinded evaluators. We established a noninferiority margin of -13.2%, considering an acceptable reduction of 15% in the expected recanalization rates., Results: From September 2021 to July 2023, 120 patients were randomly assigned and 116 (58 transradial access and 58 transfemoral access) with confirmed intracranial occlusion on the initial angiogram were included in the intention-to-treat analysis. Successful recanalization was achieved in 51 (87.9%) patients assigned to transfemoral access and in 56/58 (96.6%) patients assigned to transradial (adjusted 1 side risk difference [RD], -5.0% [95% CI, -6.61% to +13.1%]) showing noninferiority of transradial access. Median time from angiosuite arrival to first pass (femoral, 30 [interquartile range, 25-37] minutes versus radial: 41 [interquartile range, 33-62] minutes; P <0.001) and from angiosuite arrival to recanalization (femoral: 42 (IQR, 28-74) versus radial: 59.5 (IQR, 44-81) minutes; P <0.050) were longer in the transradial access group. Both groups presented 1 severe access complication and there was no difference in the rate of access conversion: transradial 7 (12.1%) versus transfemoral 5 (8.6%) ( P =0.751)., Conclusions: Among patients who underwent mechanical thrombectomy, transradial access was noninferior to transfemoral access in terms of final recanalization. Procedural delays may favor transfemoral access as the default first-line approach., Registration: URL: https://www.clinicaltrials.gov; Unique identifier: NCT05225636., Competing Interests: Disclosures Dr Requena reported receiving personal fees as consultor of Anaconda Biomed outside the submitted work. Dr Rubiera reported compensation from Bayer for data and safety monitoring services. Dr Tomasello reported receiving personal fees from Anaconda Biomed, Balt, Medtronic, Perflow, Ivascular, and Stryker outside the submitted work. Dr Ribo reported receiving personal fees from Anaconda Biomed, AptaTargets, Cerenovus, Johnson & Johnson, Medtronic, Methinks, Philips, Sanofi, Stryker, and Rapid AI outside the submitted work and is co-principal investigator of the WE-TRUST trial ([Workflow Optimization to Reduce Time to Endovascular Reperfusion in Stroke Treatment]; NCT04701684). The other authors report no conflicts.

Background: Symptomatic carotid artery stenosis is a significant contributor to ischemic strokes. Carotid artery stenting (CAS) is usually indicated for secondary stroke prevention. This study evaluates the safety and efficacy of CAS performed within a short time frame from symptom onset., Methods: We conducted a single-center, retrospective study of consecutive patients who underwent CAS for symptomatic carotid stenosis within eight days of symptom onset from July 2019 to January 2022. Data on demographics, medical history, procedural details, and follow-up outcomes were analyzed. The primary outcome measure was the recurrence of the stroke within the first month post-procedure. Secondary outcomes included mortality, the rate of intra-procedural complications, and hyperperfusion syndrome., Results: We included 93 patients with a mean age of 71.7 ± 11.7 years. The median time from symptom onset to CAS was 96 h. The rate of stroke recurrence was 5.4% in the first month, with a significant association between the number of stents used and increased recurrence risk. Mortality within the first month was 3.2%, with an overall mortality rate of 11.8% after a median follow-up of 19 months. Intra-procedural complications were present in five (5.4%) cases and were related to the number of stents used ( p  = 0.002) and post-procedural angioplasty ( p  = 0.045). Hyperperfusion syndrome occurred in 3.2% of cases., Conclusion: Early CAS within the high-risk window post-symptom onset is a viable secondary stroke prevention strategy in patients with symptomatic carotid artery stenosis. The procedure rate of complication is acceptable, with a low recurrence of stroke. However, further careful selection of patients for this procedural strategy is crucial to optimize outcomes., Competing Interests: Declaration of conflicting interestsThe authors declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: Manuel Requena has a consulting agreement with Anaconda Biomed. Dr Tomasello has received personal fees from Anaconda Biomed, Balt, Medtronic, Perflow, and Stryker. MRi has a consulting agreement with Medtronic, Stryker, Johnson and Johnson, Perflow Medical, Anaconda Biomed, Apta Targets, Ceronovus, Philips, Sanofi, and Rapid AI.

The endosomal sorting complex required for transport (ESCRT) machinery is essential for membrane remodeling and autophagy and it comprises three multi-subunit complexes (ESCRT I-III). We report nine individuals from six families presenting with a spectrum of neurodevelopmental/neurodegenerative features caused by bi-allelic variants in SNF8 (GenBank: NM&#95;007241.4), encoding the ESCRT-II subunit SNF8. The phenotypic spectrum included four individuals with severe developmental and epileptic encephalopathy, massive reduction of white matter, hypo-/aplasia of the corpus callosum, neurodevelopmental arrest, and early death. A second cohort shows a milder phenotype with intellectual disability, childhood-onset optic atrophy, or ataxia. All mildly affected individuals shared the same hypomorphic variant, c.304G>A (p.Val102Ile). In patient-derived fibroblasts, bi-allelic SNF8 variants cause loss of ESCRT-II subunits. Snf8 loss of function in zebrafish results in global developmental delay and altered embryo morphology, impaired optic nerve development, and reduced forebrain size. In vivo experiments corroborated the pathogenicity of the tested SNF8 variants and their variable impact on embryo development, validating the observed clinical heterogeneity. Taken together, we conclude that loss of ESCRT-II due to bi-allelic SNF8 variants is associated with a spectrum of neurodevelopmental/neurodegenerative phenotypes mediated likely via impairment of the autophagic flux., Competing Interests: Declaration of interests The authors declare no competing interests., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Borderline Personality Disorder (BPD) is a severe mental disorder, characterized by deficits in emotion regulation, interpersonal dysfunctions, dissociation and impulsivity. Brain abnormalities have been generally explored; however, the specific contribution of different limbic structures to BPD symptomatology is not described. The aim of this study is to cover this gap, exploring functional and structural alterations of amygdala and insula and to highlight their contribution to neuropsychiatric symptoms. Twenty-eight BPD patients (23.7 ± 3.42 years; 6 M/22F) and twenty-eight matched healthy controls underwent a brain MR protocol (1.5 T, including a 3D T1-weighted sequence and resting-state fMRI) and a complete neuropsychiatric assessment. Volumetry, cortical thickness and functional connectivity of amygdala and insula were evaluated, along with correlations with the neuropsychiatric scales. BPD patients showed a lower cortical thickness of the left insula (p = 0.027) that negatively correlated with the Anger Rumination Scale (p = 0.019; r = - 0.450). A focused analysis on female patients showed a significant reduction of right amygdala volumes in BPD (p = 0.037), that correlate with Difficulties in Emotion Regulation Scale (p = 0.031; r =  - 0.415), Beck Depression Inventory (p = 0.009; r =  - 0.50) and Ruminative Response Scale (p = 0.045; r =  - 0.389). Reduced functional connectivity was found in BPD between amygdala and frontal pole, precuneus and temporal pole. This functional connectivity alterations correlated with Anger Rumination Scale (p = .009; r = - 0.491) and Barratt Impulsiveness Scale (p = 0.020; r = - 0.447). Amygdala and insula are altered in BPD patients, and these two limbic structures are implicated in specific neuropsychiatric symptoms, such as difficulty in emotion regulation, depression, anger and depressive rumination., (© 2023. The Author(s).)

Background: Cognitive improvement after endovascular embolization of an intracranial dural arteriovenous fistula (dAVF) remains unexplored. We aim to investigate cognitive changes following endovascular embolization of dAVFs., Methods: Neuropsychology in dural ArterIal Fistula (NAIF) was a prospective multicentric study including patients with an angiographic diagnosis of dAVF who underwent endovascular embolization over the course of 4 years. A complete neuropsychological evaluation comprising five cognitive domains (attention and executive functions, memory, language, praxis, gnosis) was performed at baseline and 3 months follow-up. Mean Z scores for cognitive tests were compared pre- and post-treatment using paired sample t-tests, where higher Z scores indicate better cognition. Effect sizes were computed as Cohen's d., Results: A total of 32 patients (mean age 61.1±15.4 years, 10 (31.3%) females) were included. Patients exhibited improved performance in attention and executive functions: executive functions-attention (+0.282, P=0.009, d=0.29), executive functions-fluencies (+0.283, P=0.029, d=0.4), and executive functions-processing speed (+0.471, P=0.039, d=0.41). There was an increase in memory: verbal learning and verbal delayed recall scores (+0.513, P<0.001, d=0.55, and +0.385, P=0.001, d=0.41, respectively), while verbal recognition parameters (+0.839, P=0.086, d=0.37) and visual memory (delayed recall) (+0.430, P=0.060, d=0.35) displayed trends toward improved performance. Regarding language, there was significant overall improvement (+0.300, P=0.014, d=0.24), but neither praxis nor gnosis changed significantly. These cognitive outcomes were independent of the severity (measured as Cognard classification), and no patient experienced cognitive worsening., Conclusion: This study suggests that endovascular embolization confers cognitive benefits on dAVF patients undergoing endovascular embolization and may be beneficial even for patients with a low risk of hemorrhage., Competing Interests: Competing interests: This study was supported by Medtronic. The supporter had no role in the design, conduct, collection, management, analysis, and interpretation of the data, preparation and approval of the manuscript. Dr Gramegna had full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis. Dr Tomasello has received personal fees from Anaconda Biomed, Balt, Medtronic, Perflow, and Stryker outside the submitted work. No other disclosures were reported., (© Author(s) (or their employer(s)) 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: The purpose of this study was to investigate if vessel-wall magnetic resonance imaging (VW-MRI) could differentiate among primary headaches disorders, such as migraine and cluster headache (CH), and detect the presence of neurogenic inflammation., Background: The pathophysiology of primary headaches disorders is complex and not completely clarified. The activation of nociceptive trigeminal afferents through the release of vasoactive neuropeptides, termed "neurogenic inflammation," has been hypothesized. VW-MRI can identify vessel wall changes, reflecting the inflammatory remodeling of the vessel walls despite different etiologies., Methods: In this case series, we enrolled seven patients with migraine and eight patients with CH. They underwent a VW-MRI study before and after the intravenous administration of contrast medium, during and outside a migraine attack or cluster period. Two expert neuroradiologists analyzed the magnetic resonance imaging (MRI) studies to identify the presence of vessel wall enhancement or other vascular abnormalities., Results: Fourteen out of 15 patients had no enhancement. One out of 15, with migraine, showed a focal parietal enhancement in the intracranial portion of a vertebral artery, unmodified during and outside the attack, thus attributable to atherosclerosis. No contrast enhancement attributable to neurogenic inflammation was observed in VW-MRI, both during and outside the attack/cluster in all patients. Moreover, MRI angiography registered slight diffuse vasoconstriction in one of seven patients with migraine during the attack and in one of eight patients with cluster headache during the cluster period; both patients had taken triptans as symptomatic therapy for pain., Conclusions: These preliminary results suggest that VW-MRI studies are negative in patients with primary headache disorders even during migraine attacks or cluster periods. The VW-MRI studies did not detect signs of neurogenic inflammation in the intracranial intradural vessels of patients with migraine or CH., (© 2022 The Authors. Headache: The Journal of Head and Face Pain published by Wiley Periodicals LLC on behalf of American Headache Society.)

Introduction: The iNedit balloon distal access catheter is a novel thrombectomy device. It has an inner diameter of 0.058″, proximal outer diameter of 2.13 mm, and distal outer diameter of 1.67mm. It is compatible with a 0.088″ guide catheter and includes a balloon located 5 cm from the catheter tip, enabling proximal flow restriction and combined therapy with stent retrievers. We investigate the appraisal of the use, safety, and efficacy of the iNedit catheter in the first-in-human study., Methods: In the preliminary cases that demanded training on the product previous to a multicentric study, prospective data were collected on 22 consecutive patients treated with the iNedit catheter to perform thrombectomy for acute ischemic stroke due to large vessel occlusion within 24 h. The outcome measures consisted of several evaluations of user experience rated on a 5-point scale ranging from 1 (bad) to 5 (excellent), as well as assessments of procedural safety outcomes such as artery perforation and arterial occlusion, procedural efficacy outcomes including first-pass effect (Thrombolysis In Cerebral Infarction [TICI] 2c/3) and final recanalization (TICI 2b/3), and clinical efficacy outcomes such as a 3-month 0-2 modified Rankin Scale (mRS)., Results: The mean age was 72 ± 12 years old; median National Institute Health Stroke Scale was 17 (11-19). Sites of primary occlusion were: 2 internal carotid artery, 12 M1-MCA, 7 M2-MCA, and one P1. Median score evaluation of the appraisal of use was 4- IQR [4-5]. The median number of passes was 1 [IQR 1-2]. First pass complete recanalization rate was 50% and the final recanalization rate was 94.45%. No artery perforation and arterial occlusion. Good functional outcome mRS 0-2 was achieved in 50% of patients., Conclusions: In this initial clinical experience, iNedit device achieved a high rate of first-pass effect and final recanalization rate with no safety concerns, thus favoring a high percentage of good clinical outcomes., Competing Interests: Declaration of conflicting interestsThe author(s) declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: Dr Tomasello has received personal fees from Anaconda Biomed, Balt, Medtronic, Perflow, and Stryker. Dr Macho has received personal fees from, Balt, Medtronic, Stryker and iVascular.

Background: The aim of this is to explore the histological basis of vessel wall enhancement (WE) on magnetic resonance imaging (MRI), which is a strong radiological biomarker of aneurysmal prone to rupture compared to other classical risk predictors (e.g., PHASES score, size, morphology)., Methods: A prospective observational study was performed including all consecutive patients presenting with a saccular intracranial aneurysm at Vall d'Hebron University Hospital between October 2017 and May 2019. The patients underwent high-resolution 3 T MRI, and their aneurysms were classified into asymptomatic, symptomatic, and ruptured. A histological and immunohistochemical study was performed in a subgroup of patients (n = 20, of which 15 presented with WE). Multiple regression analyses were performed to identify predictors of rupture and aneurysm symptoms., Results: A total of 132 patients were enrolled in the study. WE was present in 36.5% of aneurysms: 22.9% asymptomatic, 76.9% symptomatic, and 100% ruptured. Immunohistochemical markers associated with WE were CD3 T cell receptor (p = 0.05) and CD45 leukocyte common antigen (p = 0.05). Moreover, WE is an independent predictor of symptomatic and ruptured aneurysms (p < 0.001)., Conclusions: Aneurysms with WE present multiple histopathological changes that may contribute to wall disruption and represent the pathophysiological basis of radiological WE. Moreover, WE is an independent diagnostic predictor of aneurysm symptoms and rupture., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Austria, part of Springer Nature.)

Introduction: Mechanical thrombectomy (MT) with combined treatment including both a stent retriever and distal aspiration catheter may improve recanalization rates in patients with acute ischemic stroke (AIS) due to large vessel occlusion (LVO). Here, we evaluated the effectiveness and safety of the REACT aspiration catheter used with a stent retriever., Methods: This prospective study included consecutive adult patients who underwent MT with a combined technique using REACT 68 and/or 71 between June 2020 and July 2021. The primary endpoints were final and first pass mTICI 2b-3 and mTICI 2c-3 recanalization. Analysis was performed after first pass and after each attempt. Secondary safety outcomes included procedural complications, symptomatic intracranial hemorrhage (sICH) at 24 h, in-hospital mortality, and 90-day functional independence (modified Rankin Scale [mRS] 0-2)., Results: A total of 102 patients were included (median age 78; IQR: 73-87; 50.0% female). At baseline, median NIHSS score was 19 (IQR: 11-21), and ASPECTS was 9 (IQR: 8-10). Final mTICI 2b-3 recanalization was achieved in 91 (89.2%) patients and mTICI 2c-3 was achieved in 66 (64.7%). At first pass, mTICI 2b-3 was achieved in 55 (53.9%) patients, and mTICI 2c-3 in 37 (36.3%). The rate of procedural complications was 3.9% (4/102), sICH was 6.8% (7/102), in-hospital mortality was 12.7% (13/102), and 90-day functional independence was 35.6% (36/102)., Conclusion: A combined MT technique using a stent retriever and REACT catheter resulted in a high rate of successful recanalization and first pass recanalization in a sample of consecutive patients with AIS due to LVO in clinical use., Competing Interests: Declaration of conflicting interestsThe author(s) declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: MRi has modest ownership and serves on the advisory board of Methinks Software; he has a consulting agreement with Medtronic, Stryker, Johnson and Johnson, Perflow Medical, Anaconda Biomed, Apta Targets, Ceronovus, Philips, Sanofi, and Rapid AI. AT has received personal fees from Anaconda Biomed, Balt, Medtronic, Perflow, and Stryker.

Background: Flow diverters have emerged in recent years as a safe and effective treatment for intracranial aneurysms, with expanding indications. The Derivo embolization device (DED) is a second-generation flow diverter with a surface finish that may reduce thrombogenicity. We report our multicenter experience evaluating its safety and efficacy., Methods: We retrospectively analyzed all patients treated with the DED in eight centers in Spain between 2016 and 2020. Demographics, clinical data, procedural complications, morbidity and aneurysm occlusion rates were collected., Results: A total of 209 patients with 250 aneurysms were treated (77.5% women). The majority of aneurysms were located in the internal carotid artery (86.8%) and most (69.2%) were small (<10 mm) with a median maximum diameter of 5.85 mm and median neck size of 4 mm. DED deployment was successful in all cases, despite two malfunctioning devices (1%). Major complications occurred in nine patients (4.3%), while mild neurologic clinical events were registered in 23 (11%); four patients died (1.9%). A total of 194 aneurysms had an angiographic follow-up at 6 months and showed complete aneurysm occlusion in 75% of cases. Twelve-month follow-up was available for 112 of the treated aneurysms, with a total occlusion rate of 83%., Conclusion: The DED is a second-generation surface-modified flow diverter that presents an option for treatment of intracranial aneurysms with comparable safety and efficacy to other available flow diverter devices. Nonetheless, risks are not negligible, and must be balanced against the natural history risk of cerebral aneurysms, considering the tendency to widen indications for treatment of smaller and less complex lesions in day-to-day use., Competing Interests: Competing interests: AT has received personal fees from Anaconda Biomed, Balt, Medtronic, Perflow, and Stryker., (© Author(s) (or their employer(s)) 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

The number of stentriever passes during endovascular thrombectomy impacts clinical outcomes in acute ischemic stroke. Previous studies suggest that the simultaneous double stent retriever technique (DSRT) could improve the efficacy and reduce the number of passes. We aim to analyze the degree of vessel wall injury according to the number of passes and technique (single vs. simultaneous devices). Histological changes were evaluated in renal arteries (RAs) of swine models after thrombectomy (1, 2, or 3 passes) with single stent (SSRT) and DSRT. Thrombectomy passes were performed in 12 RA: 3 samples from each artery were studied by optical microscopy to assess a vascular damage score. All thirty-six samples showed endothelial denudation and different degrees of damage in the deepest layers of the arterial wall; however, all arteries remained patent by the time of assessment. In all cases, the degree of vascular injury increased with the number of passes. Compared with a SSRT, DSRT showed a higher severity of histological damage corresponding to the damage caused by 1.4 SSRT passes. However, in distal arteries, vascular damage was relatively similar when comparing SSRT with multiple passes and DSRT with one pass. The degree of vessel injury increases with the number of passes. Even though histological damage per pass was 1.4 higher with DSRT than SSRT, short-term vessel patency was not compromised after up to 3 DSRT passes. Further studies are needed to characterize the risk-benefit ratio of the DSRT in routine clinical practice., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

The anterior optic pathway (AOP) is a system of three structures (optic nerves, optic chiasma, and optic tracts) that convey visual stimuli from the retina to the lateral geniculate nuclei. A successful reconstruction of the AOP using tractography could be helpful in several clinical scenarios, from presurgical planning and neuronavigation of sellar and parasellar surgery to monitoring the stage of fiber degeneration both in acute (e.g., traumatic optic neuropathy) or chronic conditions that affect AOP structures (e.g., amblyopia, glaucoma, demyelinating disorders or genetic optic nerve atrophies). However, its peculiar anatomy and course, as well as its surroundings, pose a serious challenge to obtaining successful tractographic reconstructions. Several AOP tractography strategies have been adopted but no standard procedure has been agreed upon. We performed a systematic review of the literature according to the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) 2020 guidelines in order to find the combinations of acquisition and reconstruction parameters that have been performed previously and have provided the highest rate of successful reconstruction of the AOP, in order to promote their routine implementation in clinical practice. For this purpose, we reviewed data regarding how the process of anatomical validation of the tractographies was performed. The Cochrane Handbook for Systematic Reviews of Interventions was used to assess the risk of bias and thus the study quality We identified thirty-nine studies that met our inclusion criteria, and only five were considered at low risk of bias and achieved over 80% of successful reconstructions. We found a high degree of heterogeneity in the acquisition and analysis parameters used to perform AOP tractography and different combinations of them can achieve satisfactory levels of anterior optic tractographic reconstruction both in real-life research and clinical scenarios. One thousand s/mm 2 was the most frequently used b value, while both deterministic and probabilistic tractography algorithms performed morphological reconstruction of the tract satisfactorily, although probabilistic algorithms estimated a more realistic percentage of crossing fibers (45.6%) in healthy subjects. A wide heterogeneity was also found regarding the method used to assess the anatomical fidelity of the AOP reconstructions. Three main strategies can be found: direct visual direct visual assessment of the tractography superimposed to a conventional MR image, surgical evaluation, and computational methods. Because the latter is less dependent on a priori knowledge of the anatomy by the operator, computational methods of validation of the anatomy should be considered whenever possible., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 The Authors. Published by Elsevier Inc. All rights reserved.)

Myotonic dystrophy type 1 (DM1) is a genetic disorder caused by a (CTG) expansion in the DM protein kinase (DMPK) gene, representing the most common adult muscular dystrophy, characterized by a multisystem involvement with predominantly skeletal muscle and brain affection. Neuroimaging studies showed widespread white matter changes and brain atrophy in DM1, but only a few studies investigated the role of white matter metabolism in the pathophysiology of central nervous system impairment. We aim to reveal the relationship between the metabolic profile of parieto-occipital white matter (POWM) as evaluated with proton MR spectroscopy technique, with the visuoperceptual and visuoconstructional dysfunctions in DM1 patients. MR spectroscopy (3 Tesla) and neuropsychological evaluations were performed in 34 DM1 patients (19 F, age: 46.4 ± 12.1 years, disease duration: 18.7 ± 11.6 years). The content of neuro-axonal marker N-acetyl-aspartate, both relative to Creatine (NAA/Cr) and to myo-Inositol (NAA/mI) resulted significantly lower in DM1 patients compared to HC (p-values < 0.0001). NAA/Cr and NAA/mI correlated with the copy of the Rey-Osterrieth complex figure (r = 0.366, p = 0.033; r = 0.401, p = 0.019, respectively) and with Street’s completion tests scores (r = 0.409, p = 0.016; r = 0.341, p = 0.048 respectively). The proportion of white matter hyperintensities within the MR spectroscopy voxel did not correlate with the metabolite content. In this study, POWM metabolic alterations in DM1 patients were not associated with the white matter morphological changes and correlated with specific neuropsychological deficits.

Purpose: To describe the clinical phenotype of a cohort of patients with Wolfram syndrome (WS), focusing on the pattern of optic atrophy correlated with brain magnetic resonance imaging (MRI) measurements, as compared with patients with OPA1-related dominant optic atrophy (DOA)., Design: Retrospective, comparative cohort study., Methods: We reviewed 25 patients with WS and 33 age-matched patients affected by OPA1-related DOA. Ophthalmologic, neurologic, endocrinologic, and MRI data from patients with WS were retrospectively retrieved. Ophthalmologic data were compared with data from patients with OPA1-related DOA and further analyzed for age dependency dividing patients in age quartiles. In a subgroup of patients with WS, we correlated the structural damage assessed by optical coherence tomography (OCT) with brain MRI morphologic measurements. Visual acuity (VA), visual field mean defect (MD), retinal nerve fiber layer (RNFL), and ganglion cell layer (GCL) thickness were assessed by OCT and MRI morphologic measurements of anterior and posterior visual pathways., Results: Optic atrophy was present in 100% of patients with WS. VA, MD, and RNFL thickness loss were worse in patients with WS with a faster decline since early age as compared with patients with DOA, who displayed a more stable visual function over the years. Conversely, GCL sectors were overall thinner in patients with DOA since early age compared to patients with WS, in which GCL thickness started to decline later in life. The neuroradiologic subanalysis on 11 patients with WS exhibited bilateral thinning of the anterior optic pathway, especially the prechiasmatic optic nerves and optic tracts. Optic tract thinning was significantly correlated with GCL thickness but not with RNFL parameters., Conclusions: Our results showed a generally more severe and diffuse degeneration of both anterior and posterior visual pathways in patients with WS, with fast deterioration of visual function and structural OCT parameters since early age. The pattern observed with OCT suggests that retinal ganglion cell axonal degeneration (ie, RNFL) precedes cellular body atrophy (ie, GCL) by about a decade. This differs substantially from DOA, in which a more stable visual function is evident with predominant early loss of GCL, indirectly supporting the lack of a primary mitochondrial dysfunction in patients with WS., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Tractography based on multishell diffusion-weighted magnetic resonance imaging (DWI) can be used to estimate the course of myelinated white matter tracts and nerves, yielding valuable information regarding normal anatomy and variability. DWI is sensitive to the local tissue microstructure, so tractography can be used to estimate tissue properties within nerve tracts at a resolution of millimeters. This study aimed to test the applicability of the method using a disease with a well-established pattern of myelinated nerve involvement. Eight patients with LHON and 13 age-matched healthy controls underwent tractography of the anterior optic pathway. Diffusion parameters were compared between groups, and for the patient group correlated with clinical/ophthalmological parameters. Tractography established the course of the anterior optic pathway in both patients and controls. Localized changes in fractional anisotropy were observed, and related to estimates of different tissue compartments within the nerve and tract. The proportion of different compartments correlated with markers of disease severity. The method described allows both anatomical localization and tissue characterization in vivo, permitting both visualization of variation at the individual level and statistical inference at the group level. It provides a valuable adjunct to ex vivo anatomical and histological study of normal variation and disease processes.

Vessel Wall MRI (VW-MRI) is an emerging MR sequence used for diagnosis, characterization, and treatment planning of cerebrovascular diseases. Although VW-MRI is not yet routinely used, most papers have emphasized its role in several aspects of the management of cerebrovascular diseases. Nowadays, no VW-MRI sequence optimized for the intracranial imaging is commercially available, thus the Spin Echo sequences are the more effective sequences for this purpose. Moreover, as one of the principal technical requirements for intracranial VW-MR imaging is to achieve both the suppression of blood in vessel lumen and of the outer cerebrospinal fluid, different suppression techniques have been developed. This short report provides the technical parameters of our VW-MR sequence developed over 3-years' experience., (© 2022. The Author(s).)

Purpose: Biliary ductal injuries are challenging to treat, and often lead to severe morbidity and mortality. The first-line approach involves endoscopic retrograde cholangiopancreatography with sphincterotomy and, in case of refractory leakage, long-lasting percutaneous transhepatic biliary drainage, endoscopic or percutaneous injection of sclerosing agents and/or coiling can be used. We describe a treatment procedure using microcatheter-mediated percutaneous or endoscopic argon plasma coagulation (APC)., Materials and Methods: Three patients (7-year-old male, 14-year-old male, 81-year-old female) with refractory postsurgical and/or post-traumatic bile leaks underwent percutaneous (n = 2) or endoscopic (n = 1) APC through a detachable microcatheter., Results: The procedure was technically feasible in all patients. Postoperative imaging showed complete occlusion of biliary leakage. The technique was uneventful intraoperatively with no adverse events occurring during recovery or follow-up., Conclusion: Our initial experience demonstrates that refractory bile duct leaks may be successfully treated with microcatheter-mediated APC endoscopically or percutaneously. Further research is needed to confirm the safety, efficacy, and clinical indications for this innovative technique., (© 2021. Springer Science+Business Media, LLC, part of Springer Nature and the Cardiovascular and Interventional Radiological Society of Europe (CIRSE).)

Introduction: The mitochondrial DNA (mtDNA) m.3243A > G mutation in the MT-TL1 gene results in a multi-systemic disease, that is commonly associated with neurodegenerative changes in the brain., Methods: Seventeen patients harboring the m3243A > G mutation were enrolled (age 43.1 ± 11.4 years, 10 M/7F). A panel of plasma biomarkers including lactate acid, alanine, L-arginine, fibroblast growth factor 21 (FGF-21), growth/differentiation factor 15 (GDF-15) and circulating cell free -mtDNA (ccf-mtDNA), as well as blood, urine and muscle mtDNA heteroplasmy were evaluated. Patients also underwent a brain standardized MR protocol that included volumetric T1-weighted images and diffusion-weighted MRI. Twenty sex- and age-matched healthy controls were included. Voxel-wise analysis was performed on T1-weighted and diffusion imaging, respectively with VBM (voxel-based morphometry) and TBSS (Tract-based Spatial Statistics). Ventricular lactate was also evaluated by 1 H-MR spectroscopy., Results: A widespread cortical gray matter (GM) loss was observed, more severe (p < 0.001) in the bilateral calcarine, insular, frontal and parietal cortex, along with infratentorial cerebellar cortex. High urine mtDNA mutation load, high levels of plasma lactate and alanine, low levels of plasma arginine, high levels of serum FGF-21 and ventricular lactate accumulation significantly (p < 0.05) correlated with the reduced brain GM density. Widespread microstructural alterations were highlighted in the white matter, significantly (p < 0.05) correlated with plasma alanine and arginine levels, with mtDNA mutation load in urine, with high level of serum GDF-15 and with high content of plasma ccf-mtDNA., Conclusions: Our results suggest that the synergy of two pathogenic mechanisms, mtDNA-related mitochondrial respiratory deficiency and defective nitric oxide metabolism, contributes to the brain neurodegeneration in m.3243A > G patients., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Objective: Friedreich ataxia (FRDA) is an inherited neurological disease defined by progressive movement incoordination. We undertook a comprehensive characterization of the spatial profile and progressive evolution of structural brain abnormalities in people with FRDA., Methods: A coordinated international analysis of regional brain volume using magnetic resonance imaging data charted the whole-brain profile, interindividual variability, and temporal staging of structural brain differences in 248 individuals with FRDA and 262 healthy controls., Results: The brainstem, dentate nucleus region, and superior and inferior cerebellar peduncles showed the greatest reductions in volume relative to controls (Cohen d = 1.5-2.6). Cerebellar gray matter alterations were most pronounced in lobules I-VI (d = 0.8), whereas cerebral differences occurred most prominently in precentral gyri (d = 0.6) and corticospinal tracts (d = 1.4). Earlier onset age predicted less volume in the motor cerebellum (r max  = 0.35) and peduncles (r max  = 0.36). Disease duration and severity correlated with volume deficits in the dentate nucleus region, brainstem, and superior/inferior cerebellar peduncles (r max  = -0.49); subgrouping showed these to be robust and early features of FRDA, and strong candidates for further biomarker validation. Cerebral white matter abnormalities, particularly in corticospinal pathways, emerge as intermediate disease features. Cerebellar and cerebral gray matter loss, principally targeting motor and sensory systems, preferentially manifests later in the disease course., Interpretation: FRDA is defined by an evolving spatial profile of neuroanatomical changes beyond primary pathology in the cerebellum and spinal cord, in line with its progressive clinical course. The design, interpretation, and generalization of research studies and clinical trials must consider neuroanatomical staging and associated interindividual variability in brain measures. ANN NEUROL 2021;90:570-583., (© 2021 The Authors. Annals of Neurology published by Wiley Periodicals LLC on behalf of American Neurological Association.)

Major cerebral vessels have been proposed as a target of defective mitochondrial metabolism in patients with mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes syndrome (MELAS). Cerebral angiographic techniques are not routinely performed in MELAS patients. A systematic literature review was performed to identify studies describing major vessel caliber alterations in MELAS. Twenty-three studies reporting on 46 MELAS patients were included. Alterations in major caliber vessels were present in 59% (27/46) of patients. Dilation occurred in 37% (17/46) of patients, and in 88% (15/17) of them during a stroke-like episode (SLE). Stenosis was reported in 24% (11/46) of patients: 36% (4/11) related to an SLE and 64% (7/11) to dissections or degenerative changes. During an SLE, identification of intracranial vessels dilation or stenosis could be a selection tool for new treatment protocols. Outside SLE, identification of major cerebral vessels dissections and degenerative changes may help to prevent subsequent complications., (Copyright © 2021 Elsevier Masson SAS. All rights reserved.)

Objective: The purpose of this study was to investigate correlations between brain proton magnetic resonance spectroscopy ( 1 H-MRS) findings with serum biomarkers and heteroplasmy of mitochondrial DNA (mtDNA) mutations. This study enrolled patients carrying mtDNA mutations associated with Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-like episodes (MELAS), and MELAS-Spectrum Syndrome (MSS)., Methods: Consecutive patients carrying mtDNA mutations associated with MELAS and MSS were recruited and their serum concentrations of lactate, alanine, and heteroplasmic mtDNA mutant load were evaluated. The brain protocol included single-voxel 1 H-MRS (1.5T) in the medial parieto-occipital cortex (MPOC), left cerebellar hemisphere, parieto-occipital white matter (POWM), and lateral ventricles. Relative metabolite concentrations of N-acetyl-aspartate (NAA), choline (Cho), and myo-inositol (mI) were estimated relative to creatine (Cr), using LCModel 6.3., Results: Six patients with MELAS (age 28 ± 13 years, 3 [50%] female) and 17 with MSS (age 45 ± 11 years, 7 [41%] female) and 39 sex- and age-matched healthy controls (HC) were enrolled. These patients demonstrated a lower NAA/Cr ratio in MPOC compared to HC (p = 0.006), which inversely correlated with serum lactate (p = 0.021, rho = -0.68) and muscle mtDNA heteroplasmy (p < 0.001, rho = -0.80). Similarly, in the cerebellum patients had lower NAA/Cr (p < 0.001), Cho/Cr (p = 0.002), and NAA/mI (p = 0.001) ratios, which negatively correlated with mtDNA blood heteroplasmy (p = 0.001, rho = -0.81) and with alanine (p = 0.050, rho = -0.67). Ventricular lactate was present in 78.3% (18/23) of patients, correlating with serum lactate (p = 0.024, rho = 0.58)., Conclusion: Correlations were found between the peripheral and biochemical markers of mitochondrial dysfunction and brain in vivo markers of neurodegeneration, supporting the use of both biomarkers as signatures of MELAS and MSS disease, to evaluate the efficacy of potential treatments., (© 2021 The Authors. Annals of Clinical and Translational Neurology published by Wiley Periodicals LLC on behalf of American Neurological Association.)

Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) is a rare autosomal recessive disease caused by TYMP mutations and thymidine phosphorylase (TP) deficiency. Thymidine and deoxyuridine accumulate impairing the mitochondrial DNA maintenance and integrity. Clinically, patients show severe and progressive gastrointestinal and neurological manifestations. The onset typically occurs in the second decade of life and mean age at death is 37 years. Signs and symptoms of MNGIE are heterogeneous and confirmatory diagnostic tests are not routinely performed by most laboratories, accounting for common misdiagnosis. Factors predictive of progression and appropriate tests for monitoring are still undefined. Several treatment options showed promising results in restoring the biochemical imbalance of MNGIE. The lack of controlled studies with appropriate follow-up accounts for the limited evidence informing diagnostic and therapeutic choices. The International Consensus Conference (ICC) on MNGIE, held in Bologna, Italy, on 30 March to 31 March 2019, aimed at an evidence-based consensus on diagnosis, prognosis, and treatment of MNGIE among experts, patients, caregivers and other stakeholders involved in caring the condition. The conference was conducted according to the National Institute of Health Consensus Conference methodology. A consensus development panel formulated a set of statements and proposed a research agenda. Specifically, the ICC produced recommendations on: (a) diagnostic pathway; (b) prognosis and the main predictors of disease progression; (c) efficacy and safety of treatments; and (f) research priorities on diagnosis, prognosis, and treatment. The Bologna ICC on diagnosis, management and treatment of MNGIE provided evidence-based guidance for clinicians incorporating patients' values and preferences., (© 2020 SSIEM.)

Background: In idiopathic normal pressure hydrocephalus (iNPH), gait and balance impairment is the most frequent symptom, and it is often associated with a higher fall risk. In a prior study, the anterior callosal angle (ACA) was validated as a reliable marker to discriminate iNPH from Alzheimer's disease and healthy controls. However, the potential correlation between the ACA with clinical symptoms and functional outcomes has not been assessed. The objective of this study is to determine the utility of the ACA in predicting gait improvement after ventriculoperitoneal (VP) shunting., Methods: Patients with probable iNPH who underwent shunt placement at a single institution were prospectively enrolled from May 2015 to May 2019. Patients were assessed preoperatively and at 6 months postoperatively following a standard clinical and MRI protocol. Callosal angle (CA) and ACA were calculated from 3 T MRI preoperatively and at 6 months postoperatively. CA and ACA were tested for correlation with clinical scores., Results: Forty-seven patients with probable INPH who completed 6-month postoperative follow-up were enrolled in the study. Baseline ACA was significantly correlated with preoperative fall risk, gait, and balance impairment assessed with Tinetti POMA scale. Additionally, baseline ACA differentiated patients who experienced improvement at Tinetti POMA scale after surgery., Conclusions: The baseline ACA is a useful neuroradiological marker to differentiate patients by fall risk and has significant correlation with the improvement in gait and balance impairment following surgery. This study demonstrated that the ACA may be a complementary tool to the CA in predicting shunt responsiveness in iNPH.

We report the longest follow-up of clinical and biochemical features of two previously reported adult mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) patients treated with liver transplantation (LT), adding information on a third, recently transplanted, patient. All three patients overcame the early post-operative period and tolerated immunosuppressive therapy. Plasma nucleoside levels dramatically decreased, with evidence of clinical improvement of ambulation and neuropathy. Conversely, other features of MNGIE, as gastrointestinal dysmotility, low weight, ophthalmoparesis, and leukoencephalopathy were essentially unchanged. A similar picture characterized two patients treated with allogenic hematopoietic stem cell transplantation (AHSCT). In conclusion, LT promptly and stably normalizes nucleoside imbalance in MNGIE, stabilizing or improving some clinical parameters with marginal periprocedural mortality rate as compared to AHSCT. Nevertheless, restoring thymidine phosphorylase (TP) activity, achieved by both LT and AHSCT, does not allow a full clinical recovery, probably due to consolidated cellular damage and/or incomplete enzymatic tissue replacement.

Introduction: Endovascular treatment with a covered heparin-bonded stent graft has been shown to be feasible and safe for treatment of peripheral artery disease, but its role in carotid disease remains unclear. The purpose of this study was to determine the feasibility and safety of a covered stent graft in treating cervical carotid artery disease in a consecutive series of patients with acute ischemic stroke (AIS) due to tandem lesion in a single high-volume Comprehensive Stroke Center., Methods: A retrospective review of all patients that underwent carotid endovascular interventions during mechanical thrombectomy for AIS using a self-expanding covered stent graft at Vall d'Hebron University Hospital between 2016 and 2018 was conducted. Patient clinical and angiographical characteristics as well as postoperative outcome and follow-up were recorded., Results: A total of eight patients were treated with the covered stents, and we observed significant improvement in stroke severity at 24 h in 5/8 patients (mean NIHSS 18 ± 5 vs 12 ± 8, p = 0.02). Successful recanalization (TICI 2b-3) after thrombectomy was achieved in 5/8 (62.5%) patients. One stent occluded during the procedure. At 3-month follow-up, stents were patent in three (37.5%) patients (two with mRS 3 and one with mRS 1). The stents of the remaining four patients (62.5%) were occluded (one with mRS 2 and three with mRS 4), although the patients did not show signs of stroke recurrence., Conclusion: The preliminary results of our study show that the self-expanding covered stent graft did not achieve satisfactory patency at 90-days among patients with AIS and tandem lesions, suggesting that its use in the carotid circulation may not be recommended in the context of AIS.