Superficial siderosis in long-standing pilocytic astrocytoma.

BACKGROUND: Pilocytic astrocytoma (PA) rarely spreads along neuraxis, and association with superficial siderosis (SS) and chronic signs of intracranial hypertension is exceptional. CASE REPORT: A 48-year-old woman presented with slow onset hearing loss in the past year. Clinical examination revealed dysarthria, positive Romberg test, and severe optic neuropathy. Cerebrospinal fluid (CSF) analysis showed numerous red blood cells, increased proteins and LDH, and high opening pressure. Brain and spine MRI demonstrated extensive superficial siderosis, bone remodeling of the skull base and spine, and diffuse nodular leptomeningeal enhancement. Histological examination of a nodule in the dorsal spine evidenced PA. CONCLUSION: We report a case of PA associated with dural remodeling and SS. The mechanism of SS is unclear but might be related to meningeal tumor infiltration and altered CSF composition and resorption.

Plasma Lipid Profiling Contributes to Untangle the Complexity of Moyamoya Arteriopathy.

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.

Characteristics of Moyamoya Disease in the Older Population: Is It Possible to Define a Typical Presentation and Optimal Therapeutical Management?

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.

Vascular Remodeling in Moyamoya Angiopathy: From Peripheral Blood Mononuclear Cells to Endothelial Cells.

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 CD45dimCD34+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.

Magnetic Resonance Spectroscopy in Adolescent Cannabis Users: Metabolites in the Anterior Cingulate Cortex Reflects Individual Differences in Personality Traits and can Affect Rehabilitation Compliance.

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.

Neurovascular Perfusion Study With DynaPBV During Preoperative Balloon Test Occlusion: A Feasibility Study on Aneurysm and Tumor Lesions.

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.

Treatment of intracranial aneurysms using the pipeline flex embolization device with shield technology: angiographic and safety outcomes at 1-year follow-up.

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.

Periprocedural outcomes and early safety with the use of the Pipeline Flex Embolization Device with Shield Technology for unruptured intracranial aneurysms: preliminary results from a prospective clinical study.

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.

Preoperative embolization of carotid chemodectoma: a technical challenge that can be customized according to angioarchitecture. Illustrative cases.

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.

Paediatric Stroke: Review of the Literature and Possible Treatment Options, including Endovascular Approach.

Stroke is among the top 10 causes of death in childhood. More than half of the surviving children have long-term neurological sequelae. Ischemic stroke (IS) includes arterial ischemic stroke and cerebral venous thrombosis with venous infarction. Haemorrhagic stroke (HS) includes intracerebral haematoma or subarachnoid haemorrhage. Risk factors for stroke are different in children and in adults. 10-30% of IS have no identified risk factors. However, multiple risk factors are recognizable in the majority of stroke in children; thus, a comprehensive diagnostic evaluation is crucial. Vascular abnormalities, such as arteriovenous malformations, aneurysms, vessel dissection, stenosis, and moyamoya disease, are frequently associated with both IS and HS and lead to high recurrence rates. Endovascular and surgical treatment options are sometimes indicated, performed on the basis of expert opinion, and extrapolated from the adult procedures. In the present paper, we review the recent literature and we discuss the treatment in five cases managed at our institutions.

Angiographically proven cervical venous engorgement: a possible concurrent cause in the pathophysiology of Hirayama's myelopathy.

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.

Spontaneous partial thrombosis of a basilar artery giant aneurysm in a child.

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.

CT and MR imaging findings and their implications in the follow-up of patients with intracranial aneurysms treated with endosaccular occlusion with onyx.

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.