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Background: Magnetic resonance-guided focused ultrasound (MRgFUS) targeting the thalamic ventral intermediate nucleus (VIM) is an innovative treatment for drug-refractory essential tremor (ET). The relationship between lesion characteristics, dentate-rubro-thalamic-tract (DRTT) involvement and clinical benefit remains unclear., Objectives: To investigate whether clinical outcome is related to lesion volume and/or its overlap with the DRTT. To compare the reliability of probabilistic versus deterministic tractography in reconstructing the DRTT and improving VIM targeting., Methods: Forty ET patients who underwent MRgFUS thalamotomy between 2019 and 2022 were retrospectively analyzed. Clinical outcomes and adverse effects were recorded at 1/6/12 months after the procedure. The DRTT was generated using deterministic and probabilistic tractography on preoperative diffusion-tensor 3 T-images and location and volume of the lesion were calculated., Results: Probabilistic tractography identified both decussating (d-DRTT) and non-decussating (nd-DRTT) components of the DRTT, whereas the deterministic approach only identified one component overlapping with the nd-DRTT. Despite the lesions predominantly intersecting the medial portion of the d-DRTT, with a significantly greater overlap in responder patients, we observed only a non-significant correlation between tremor improvement and increased d-DRTT-lesion overlap (r = 0.22, P = 0.20). The lesion volume demonstrated a significant positive correlation with clinical improvement at 1-day MRI (r = 0.42, P < 0.01)., Conclusion: Variability in the reconstructed DRTT position relative to the lesion center of mass, even among good responders, suggests that this fiber bundle is unlikely to be considered the sole target for a successful MRgFUS thalamotomy in ET. Indirect individualized targeting allows for more precise and reproducible identification of actual treatment coordinates than the direct method., (© 2024 The Author(s). Movement Disorders Clinical Practice published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.)

The development of virtual care options, including virtual hospital platforms, is rapidly changing the healthcare, mostly in the pandemic period, due to difficulties in in-person consultations. For this purpose, in 2020, a neurological Virtual Hospital (NOVHO) pilot study has been implemented, in order to experiment a multidisciplinary second opinion evaluation system for neurological diseases. Cerebrovascular diseases represent a preponderant part of neurological disorders. However, more than 30% of strokes remain of undetermined source, and rare CVD (rCVD) are often misdiagnosed. The lack of data on phenotype and clinical course of rCVD patients makes the diagnosis and the development of therapies challenging. Since the diagnosis and care of rCVDs require adequate expertise and instrumental tools, their management is mostly allocated to a few experienced hospitals, making difficult equity in access to care. Therefore, strategies for virtual consultations are increasingly applied with some advantage for patient management also in peripheral areas. Moreover, health data are becoming increasingly complex and require new technologies to be managed. The use of Artificial Intelligence is beginning to be applied to the healthcare system and together with the Internet of Things will enable the creation of virtual models with predictive abilities, bringing healthcare one step closer to personalized medicine. Herein, we will report on the preliminary results of the NOVHO project and present the methodology of a new project aimed at developing an innovative multidisciplinary and multicentre virtual care model, specific for rCVD (NOVHO-rCVD), which combines the virtual hospital approach and the deep-learning machine system., (© 2023. Fondazione Società Italiana di Neurologia.)

Purpose: Fibrocartilaginous nucleus pulposus components herniation and embolism rarely causes acute ischaemic events involving the spinal cord. Few reports have suggested this as a mechanism leading to anterior spinal artery syndrome. The purpose of this study was to evaluate the topography and pattern of this rare myelopathy by MRI., Methods: A retrospective observational case series of patients, admitted to our Institute between 2008 and 2021, with a diagnosis of fibrocartilaginous embolism based on typical clinical and radiological features., Results: Five patients were identified (2 men and 3 women; range 13-38 years). No one had pre-existing vascular risk factors. All referred potential precipitating event in the 24 h prior to symptom onset. MRI findings showed increased signal intensity of the spinal cord on T2-weighted images in all cases and degenerative disc changes opposite to it in four of them. The outcome was poor: three showed only partial sensitivity and motor improvement (mRs 4, 3, and 2, respectively); one completely recovered except for isolated hand paresis (mRs 1); and one remained severely neurologically affected (mRs 5)., Conclusions: Fibrocartilaginous embolism must be a differential diagnosis in case of otherwise unexplained spinal cord infarction in adult and paediatric low risk population. Neuroradiological findings such as abnormal spinal cord signal intensity and degenerative disc changes can aid in early diagnosis of this rare myelopathy. The prevalent myelopathy location was thoracic. All signal alterations were detected in the anterior region of the spinal cord in the territories of the anterior spinal artery., (© 2022. Fondazione Società Italiana di Neurologia.)

Moyamoya arteriopathy (MA) is a rare cerebrovascular disorder characterized by ischemic/hemorrhagic strokes. The pathophysiology is unknown. A deregulation of vasculogenic/angiogenic/inflammatory pathways has been hypothesized as a possible pathophysiological mechanism. Since lipids are implicated in modulating neo-vascularization/angiogenesis and inflammation, their deregulation is potentially involved in MA. Our aim is to evaluate angiogenic/vasculogenic/inflammatory proteins and lipid profile in plasma of MA patients and control subjects (healthy donors HD or subjects with atherosclerotic cerebrovascular disease ACVD). Angiogenic and inflammatory protein levels were measured by ELISA and a complete lipidomic analysis was performed on plasma by mass spectrometry. ELISA showed a significant decrease for MMP-9 released in plasma of MA. The untargeted lipidomic analysis showed a cumulative depletion of lipid asset in plasma of MA as compared to HD. Specifically, a decrease in membrane complex glycosphingolipids peripherally circulating in MA plasma with respect to HD was observed, likely suggestive of cerebral cellular recruitment. The quantitative targeted approach demonstrated an increase in free sphingoid bases, likely associated with a deregulated angiogenesis. Our findings indicate that lipid signature could play a central role in MA and that a detailed biomarker profile may contribute to untangle the complex, and still obscure, pathogenesis of MA.

Whereas several studies have been so far presented about the surgical outcomes in terms of mortality and perioperative complications for elderly patients submitted to neurosurgical treatments, the management of elderly moyamoya patients is unclear. This review aims to explore the available data about the clinical manifestation, characteristics, and outcome after surgery of older patients with moyamoya arteriopathy (MA). We found only two articles strictly concerning elderly patients with MA. We have also evaluated other reported adult series of moyamoya patients, including elderly cases in their analysis. Patients with MA above 50 years old may be considered a peculiar subset in which patients are often presenting with ischemic symptoms and a higher Suzuki grade. Conservative treatment may be proposed in asymptomatic or stable cases due to their fragility and possible increase of post-operative complications, while the best surgical options in symptomatic cases are still under investigation, although we believe that a minimal invasive superficial temporal artery-middle cerebral artery bypass could be considered the treatment of choice for the immediate effect on brain perfusion with a limited rate of post-operative complications.

The pathophysiological mechanisms of Moyamoya angiopathy (MA), which is a rare cerebrovascular condition characterized by recurrent ischemic/hemorrhagic strokes, are still largely unknown. An imbalance of vasculogenic/angiogenic mechanisms has been proposed as one possible disease aspect. Circulating endothelial progenitor cells (cEPCs) have been hypothesized to contribute to vascular remodeling of MA, but it remains unclear whether they might be considered a disease effect or have a role in disease pathogenesis. The aim of the present study was to provide a morphological, phenotypical, and functional characterization of the cEPCs from MA patients to uncover their role in the disease pathophysiology. cEPCs were identified from whole blood as CD45 dim CD34 + CD133 + mononuclear cells. Morphological, biochemical, and functional assays were performed to characterize cEPCs. A significant reduced level of cEPCs was found in blood samples collected from a homogeneous group of adult (mean age 46.86 ± 11.7; 86.36% females), Caucasian, non-operated MA patients with respect to healthy donors (HD; p = 0.032). Since no difference in cEPC characteristics and functionality was observed between MA patients and HD, a defective recruitment mechanism could be involved in the disease pathophysiology. Collectively, our results suggest that cEPC level more than endothelial progenitor cell (EPC) functionality seems to be a potential marker of MA. The validation of our results on a larger population and the correlation with clinical data as well as the use of more complex cellular model could help our understanding of EPC role in MA pathophysiology.

Background: Combining MRI techniques with machine learning methodology is rapidly gaining attention as a promising method for staging of brain gliomas. This study assesses the diagnostic value of such a framework applied to dynamic susceptibility contrast (DSC)-MRI in classifying treatment-naïve gliomas from a multi-center patients into WHO grades II-IV and across their isocitrate dehydrogenase (IDH) mutation status., Methods: Three hundred thirty-three patients from 6 tertiary centres, diagnosed histologically and molecularly with primary gliomas (IDH-mutant = 151 or IDH-wildtype = 182) were retrospectively identified. Raw DSC-MRI data was post-processed for normalised leakage-corrected relative cerebral blood volume (rCBV) maps. Shape, intensity distribution (histogram) and rotational invariant Haralick texture features over the tumour mask were extracted. Differences in extracted features across glioma grades and mutation status were tested using the Wilcoxon two-sample test. A random-forest algorithm was employed (2-fold cross-validation, 250 repeats) to predict grades or mutation status using the extracted features., Results: Shape, distribution and texture features showed significant differences across mutation status. WHO grade II-III differentiation was mostly driven by shape features while texture and intensity feature were more relevant for the III-IV separation. Increased number of features became significant when differentiating grades further apart from one another. Gliomas were correctly stratified by mutation status in 71% and by grade in 53% of the cases (87% of the gliomas grades predicted with distance less than 1)., Conclusions: Despite large heterogeneity in the multi-center dataset, machine learning assisted DSC-MRI radiomics hold potential to address the inherent variability and presents a promising approach for non-invasive glioma molecular subtyping and grading.

To report feasibility, safety, and technical advantages of flat-detector computed tomography perfusion (FD-CTP) during balloon test occlusion (BTO) angiography studies, 10 patients patients scheduled for BTO were evaluated. Cerebral blood volume maps were extracted from FD-CTP images acquired during the test. The FD-CTP perfusion combined with BTO is feasible and safe in intracranial tumor, and aneurysm cases in which vessel sacrifice should be considered.

Introduction: The anterior cingulate cortex (ACC) has shown to play a role in impulsivity, fear, and anxiety. Considering, its high glutamate receptor density, it was chosen as a region of interest to investigate the role of glutamate transmission in drug dependance. We investigated the correlations between personality trait scores and glutamate-to-glutamine (Glx) ratio concentrations in the ACC in order to evaluate if (1) personality traits may increase the probability of drug use and (2) drug use can modify cerebral metabolic pattern contributing to addictive behaviors., Materials and Methods: Glx ratio concentrations in the ACC region were measured with high-resolution multivoxel proton magnetic resonance spectroscopy (1H-MRS). Personality traits were evaluated utilizing Cloninger's TCI-revised test. Bivariate correlations between personality scores of 28 teens cannabis users (males, mean age = 18.54 ± 2.80) were evaluated., Results: In the ACC, we observed negative correlation between GG concentrations (r = -0.44, P = 0.05) and co-operativeness values (CO), choline (cho), and novelty seeking (NS) values (r = -0,45, P = 0.05). Low levels of glutamate and high levels of cho in the ACC were closely related to the CO and NS personality traits., Conclusions: Metabolic and personality patterns seems to be related to the risk of substance predisposition in adolescents. Our data contribute a possible support to the "top-down" control of the ACC on brain metabolism, due to the particular cerebral metabolic pattern found in "drug-using" adolescents., Competing Interests: None

The technique of direct intratumoral injection of permanent liquid agents has evolved significantly over the past few decades. The percutaneous direct puncture technique is being utilised more and more for embolisation of head and neck hypervascular lesions. We describe a case treated via the direct percutaneous transnasal (TN) puncture technique for embolisation of a sinonasal hypervascular tumour, performed in a 21-year-old male patient. Due to extensive vascularisation of the tumour, preoperative embolisation was requested by our otolaryngologist team in order to minimise intraoperative bleeding and reduce surgical morbidity. A primary attempt at trans-arterial embolisation through selective ophthalmic artery catheterisation was unsuccessful due to unfavourable anatomy. An extensive devascularisation of the nasal fossa lesion was obtained using a single injection of low-density polymeric embolic agent (SQUID®). Post-operative eradication was uneventful. Final histopathology exam revealed vascular tumour consistent with a rare sinonasal glomangiopericytoma. To our knowledge, this is the first reported case of a rare sinonasal glomangiopericytoma treated by direct TN injection, utilising the liquid polymer agent SQUID®.

Purpose: The Pipeline Embolization Device (PED) is a routine first-line treatment option for intracranial aneurysms (IAs). The Pipeline Flex Embolization Device with Shield Technology (Pipeline Shield) is an updated version of the PED which has been modified to include a surface phosphorylcholine biocompatible polymer. Its early technical success and safety have been reported previously. Here, we assessed the long-term safety and efficacy of the Pipeline Shield for the treatment of IAs., Materials and Methods: The Pipeline Flex Embolization Device with Shield Technology (PFLEX) study was a prospective, single-arm, multicenter study for the treatment of unruptured IAs using the Pipeline Shield. The primary endpoint was a major stroke in the territory supplied by the treated artery or neurologic death at 1-year post-procedure. Angiographic outcomes were also assessed by an independent radiology laboratory at 6 months and 1 year., Results: Fifty patients (mean age, 53 years; 82% female) with 50 unruptured IAs were treated. Mean aneurysm diameter was 8.82±6.15 mm. Of the target aneurysms, 38/50 (76%) were small (<10 mm), 11/50 (22%) were large (≥10 and<25 mm), and 1/50 (2%) was giant (≥25 mm). Forty-seven (94%) were located in the internal carotid artery and three (6%) in the vertebral artery. At 1-year post-procedure, no major strokes or neurologic deaths were reported, and complete occlusion was achieved in 27/33 (81.8%). There were no instances of aneurysm recurrence or retreatment., Conclusions: Our 1-year follow-up concerning angiographic and safety outcomes corroborate previous evidence that the Pipeline Shield is a safe and effective treatment for IAs., Trial Registration Number: NCT02390037., Competing Interests: Competing interests: MM-G serves as a proctor and consultant for Medtronic. SML proctors and consults for Medtronic. TL previously consulted and proctored for Covidien, Stryker, and MicroVention, and currently serves as a proctor and consultant for Sequent Medical. EPB receives honoraria from Medtronic and serves as a consultant for Medtronic., (© Author(s) (or their employer(s)) 2019. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Background and Purpose: The Pipeline Embolization Device (PED) has become a routine first-line option for treatment of intracranial aneurysms (IAs). We assessed the early safety and technical success of a new version of PED, Pipeline Flex Embolization Device with Shield Technology (Pipeline Shield), which has the same design and configuration but has been modified to include a surface synthetic biocompatible polymer., Materials and Methods: The Pipeline Flex Embolization Device with Shield Technology (PFLEX) study is a prospective, single-arm, multicenter study for the treatment of unruptured IAs using Pipeline Shield. The primary study endpoints included the occurrence of major stroke in the territory supplied by the treated artery or neurologic death at 1 year post-procedure. Secondary endpoints included the rate of Pipeline Shield-related or procedure-related serious or non-serious adverse events. Analyses were conducted to evaluate early safety findings in the 30-day post-procedure period as well as technical procedural success outcomes., Results: Fifty patients with 50 unruptured target IAs were enrolled. Mean aneurysm diameter was 8.82±6.15 mm. Thirty-eight aneurysms (76%) were small (<10 mm). Device deployment was technically successful with 98% of devices. Complete wall apposition was achieved immediately post-procedure in 48 cases (96%). No major strokes or neurologic deaths were reported in the 30-day post-procedure period., Conclusions: The results of this first experience with the new Pipeline Flex corroborate the early safety of the device. Mid-term and long-term follow-up examinations will provide data on safety outcomes at the 6-month and 1-year follow-up periods., Clinical Trial Registration: NCT02390037., Competing Interests: Competing interests: MM-G serves as a proctor and consultant for Medtronic. SML receives honoraria from Medtronic in relation to proctoring, speaking, and consulting. TL previously consulted and proctored for Covidien, Stryker, and MicroVention, and currently serves as a proctor and consultant for Sequent Medical. EPB receives honoraria from Medtronic and serves as a consultant for Medtronic., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.)

Background Idiopathic intracranial hypertension (IIH) is associated with obesity, and obesity is associated with binge eating disorder (BED). The aim of this paper is to address the presence and impact of BED in patients undergoing an IIH diagnostic protocol. Methods This was a cross-sectional study. Consecutive patients suspected of IIH underwent neurological, neuro-ophthalmologic and psychological examinations, neuroimaging studies and intracranial pressure (ICP) measurements through lumbar puncture in the recumbent position. IIH diagnosis was based on International Classification of Headache Disorders, 2nd Edition criteria; BED diagnosis was based on Diagnostic and Statistical Manual of Mental Disorders, 5th Edition criteria. The presence of oligoclonal bands (OCBs) in the cerebrospinal fluid was also assessed. Results Forty-five patients were enrolled: 33 were diagnosed with IIH and five of them (15%) were obese with BED. Compared to non-obese patients, those who were obese, and particularly those who were obese with BED, were more likely to have an IIH diagnosis (χ 2  = 14.3; p = 0.001), ICP > 200 mmH 2 O (χ 2  = 12.7; p = 0.002) and history of abuse or neglect (χ 2  = 11.2; p = 0.004). No association with OCBs was found. Conclusions We reported for the first time the presence of BED among patients with IIH and showed that BED is associated to IIH, ICP and history of abuse or neglect.

Preoperative embolization of carotid paraganglioma is increasingly used to reduce the risk of surgical bleeding obtaining a more precise and extensive tumor resection. Currently the technique can be customized in consideration of the different tumor angioarchitecture, thanks to embolic materials with different vascular penetration: solid particles of polyvinyl-alcohol (PVA), histoacryl glue, and liquid polymer (Onyx(®)). Two cases of swelling dissection with volume progression over time came to our observation. Both patients (45 and 70-year-old women) were studied with neck ultrasound imaging and computed tomography (CT) and/or magnetic resonance (MR). In both cases the radiological investigations confirmed the clinical suspicion of carotid paraganglioma. Both patients were sent for angiography control and preoperative devascularization of the lesion. The treatment was performed under general anesthesia using, depending on the type of tumor vasculature, in case 1 solid particles of PVA and histoacryl glue, and in case 2 Onyx(®) (Covidien, Irvine). The patients successfully underwent excision of the lesion via cervicotomy within a week from the embolization procedure, without any complications. The histological findings confirmed the diagnosis of chemodectoma, and macrophage infiltrates were seen in the regions previously embolized. At one-year follow-up, there was no evidence of recurrent disease. The preoperative embolization of vascular lesions of the neck represents a safe technique that yields the best results when customized over the type of vascularization of the lesion, enabling the use of the most appropriate materials, depending on the case to be treated.

The objective of this study is to discuss the possible role of cervical posterior epidural plexus engorgement during cervical flexion in the pathogenesis of Hirayama myelopathy. In Hirayama disease, MRI during neck flexion often shows that the posterior dura detaches from the posterior arches compressing the spinal cord. Autopsies demonstrated asymmetric changes in the anterior horns consistent with chronic ischemic damage, attributed to arterial insufficiency during flexion or to microcirculatory changes due to compression by the tight dura. In a 15-year-old patient with 5-year history of distal upper limbs weakness, MRI demonstrated marked venous engorgement of the posterior epidural plexus in cervical flexion, confirmed by angiography. Laminectomy from C3 to C6 with duraplasty was performed. At one-year follow-up, the clinical condition of the patient remained stable. In Hirayama myelopathy, compression of the spinal cord by the tight dura is probably the most important pathogenetic factor. However, venous congestion in flexion might play an additional role in determining spinal cord ischemic changes.

Intracranial pseudoaneurysms represent a potentially fatal complication of intracranial surgery. Our purpose is to describe their neuroradiological characteristics, prognostic features and possible treatment. Eight cases of postsurgical intracranial pseudoaneurysms have been observed at our institution since 1988. Four were observed following transsphenoidal (TS) surgery and four after pterional craniotomies. Two types of iatrogenic pseudoaneurysms were observed: "fusiform", probably due to weakening of the adventitia during surgical peeling of the tumour from the artery (three cases) and "saccular", occurring after a more focal or complete laceration of the vessel (five cases), more often after TS surgery. A thorough preoperative neuroradiological examination may identify anatomical conditions at risk for development of this severe complication. Postoperative neuroradiological follow-up is mandatory in cases in which unusual bleeding has occurred during the perioperative period, but absence of bleeding does not exclude the possible development of a pseudoaneurysm. Endovascular treatment of pseudoaneurysms represents a safe and durable procedure, specifically in those cases in which damage to the carotid siphon occurred during TS surgery.

We report the complete, spontaneous obliteration of a partially thrombosed dissecting giant aneurysm in the basilar artery by occlusion of both the lumen of the aneurysm and the parent artery in a 15-year-old girl.

Background and Purpose: Our purpose was to describe the CT and MR features of intracranial aneurysms occluded with the liquid polymer Onyx., Methods: At two centers, 35 aneurysms in 33 patients and 11 in nine patients were treated with the polymer. In 17 patients, adjunctive stents were placed at the aneurysm neck. All but three aneurysms originated from the internal carotid artery (ICA). Eighteen were giant; 15, large; and 13, small. Patients underwent pre- and postprocedural CT and/or MR imaging; MR angiograms (MRAs) were available in 22. In 35 patients (38 aneurysms), 3-month and/or 1-year follow-up angiograms were obtained for correlation with sectional images., Results: Except in two small aneurysms, polymer filling created beam hardening artifacts on CT scans. In 10 aneurysms, the polymer did not fill the aneurysm sac entirely; five showed recanalization at follow-up. On MR images (all sequences), the polymer appeared hypointense, probably because of its tantalum content; it did not create artifacts. MRAs falsely suggested reduced or absent ICA flow in 11 of 22 patients, nine of whom with stents. In the rest, MRA provided results comparable to those of selective angiography. In 12 patients, postprocedural imaging revealed new lesions., Conclusion: Onyx appears hypointense on MR images, with no artifact, and it does not interfere with MRA except in patients with stents. MR imaging may reveal new parenchymal lesions, even in asymptomatic patients. In the immediate control and follow-up of polymer-treated aneurysms, MR imaging and MRA may be preferred. CT may show the degree of filling in the aneurysmal sac, but Onyx creates artifacts that hinder CT evaluation.

Background and Purpose: We present a retrospective review of our experience in the endovascular treatment of posterior cerebral artery (PCA) aneurysms. We detail the anatomic location of these aneurysms, the technique of endovascular treatment, morphologic results, and clinical outcome. We also discuss the segmental anatomy of the PCA as it relates to the various neurologic deficits that may result from occlusion of the parent artery., Methods: From 1993 to 1998, 20 patients (12 female, eight male; mean age, 44 yrs) harboring a PCA aneurysm were treated via an endovascular approach. One patient had two aneurysms, comprising a total of 21 lesions. Fourteen (66%) of 21 aneurysms were saccular in nature, five (24%) were giant serpentine aneurysms, and two (10%) were posttraumatic. All aneurysms were treated using Guglielmi detachable coils (GDC) either by selective obliteration of the aneurysm sac or by parent artery occlusion., Results: Fourteen (66%) of the 21 aneurysms were successfully treated with preservation of the parent artery. In the remaining seven (33%), the parent artery was permanently occluded. The overall complication rate in this series was 15%, with a permanent morbidity rate of 10% and a 0% mortality rate., Conclusion: Aneurysms of the PCA are rare compared with other locations in the intracranial circulation. Saccular PCA aneurysms can be treated effectively, by use of GDC, to obliterate the aneurysm yet preserve the parent artery. Fusiform and giant serpentine aneurysms of the PCA can effectively be treated by permanent occlusion of the parent artery; in these cases, thorough knowledge of the PCA segmental anatomy is crucial in order to select the site of occlusion and to avoid major neurologic deficits.