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OBJECTIVES First aim of study was to compare the diagnostic accuracy of high resolution T2-WI (HRT2-WI) and gadolinium-enhanced T1-weighted image (Gd T1-WI) sequences in quantitative evaluation of vestibular schwannomas (VS). The second one was to determine through qualitative evaluation when Gd-injection should be recommended. METHODS Two observers in consensus retrospectively reviewed 137 magnetic resonance imaging (MRI) scans of patients with histological diagnosis of VS (33 women, 26 men), 116 with both HRT2-WI and Gd T1-WI. The examinations were subdivided in: surveillance (10), pretreatment (30), posttreatment (43), and posttreatment follow-up (33) studies. Quantitative evaluation was based on size measurement of the lesion. Structural details of the lesion, facial nerve course, and involvement of the fundus of the internal auditory canal were assessed for the qualitative evaluation in both sequences. RESULTS No statistically significant changes were demonstrated between size measurement in the HRT2-WI and Gd T1-WI (p = 0.329). Sensitivity, specificity, and accuracy of HRT2-WI in the detection of lesional size were 90.4, 98.9, 92.5%, respectively. HRT2-WI was worse for characterization of structural details in pretreatment, posttreatment, and posttreatment follow-up examinations. HRT2-WI showed better or equal capability for all the groups in the demonstration of the facial nerve course. In the evaluation of the involvement of the fundus of the internal auditory canal, HRT2-WI showed worse results or had a complementary role for the posttreatment and posttreatment follow-up groups, while in the other groups was considered mainly equal or complementary. Only in the surveillance group, HRT2-WI was never worse for all the criteria. CONCLUSION Results for quantitative evaluation were similar in both the sequences. Gadolinium injection can be avoided only in surveillance studies.

Purpose To evaluate any possible correlation between the presence of Isocitrate DeHydrogenase 1 mutation (IDH1m) and specific DTI (Diffusion Tensor Imaging) metrics, such as Fractional Anisotropy (FA), Mean Diffusivity (MD), Radial Diffusivity (RD) and Axial Diffusivity (AD). Methods We retrospectively analyzed 47 patients who underwent an advanced-MR study with DTI followed by surgical intervention with a subsequent histologic diagnosis of High-Grade Glioma (HGG) and immunohistochemical evaluation of IDH1 (Isocitrate DeHydrogenase) mutation status. For each DTI metrics we measured the ratio between tumor and normal tissue and we evaluated the correlation with IDH1 mutation. Results We observed a positive correlation with IDH1 status and RD and MD data. No correlation was demonstrated between IDH1 status and FA and AD. Discussion Our results support the hypothesis that the number of residual axonal fibers, extracellular matrix composition and the presence of colliquated tissue, may together contribute to a global RD increase in HGG, with a relatively higher increase in IDH1m tumors. Conclusions Our data are in favor of a need for multimodal advance evaluation of HGG. DTI metrics help to analyze IDH1 mutation status, in order to better characterize the lesions and to tailor treatment and follow up.

Purpose Acute ischemic stroke (AIS) due to tandem lesions (TLs) of extracranial Internal Carotid Artery and Anterior Cerebral Circulation are challenging for endovascular treatment (EVT). This study aims to evaluate feasibility, safety and efficacy of EVT for TLs’ AIS, with or without emergent carotid artery stenting (eCAS), in a real-life scenario. Methods Retrospective review of prospectively collected non-randomized thrombectomy databases from five stroke centers between 2015 and 2019. Consecutive patients with TLs’ AIS were selected. Clinical, neuroimage and procedure features, as well as antiplatelet therapy regimen, were evaluated. Primary outcome was 90-day mRS ≤ 2. Secondary outcomes included: mTICI score 2b-3, extracranial recanalization, procedural complications, symptomatic intracerebral hemorrhage (SICH) and 90-day mortality. Results Two hundred twenty-seven patients were enrolled (67.8% males; mean age 65.9 ± 12.9 years). We obtained mTICI 2b-3 in 77.1%, extracranial recanalization in 86.8%, 90-day mRS (available in 201/227 cases) ≤ 2 in 49.8%. Procedural complications occurred in 16.7%, SICH in 9.7%; 90-day mortality rate (available in 201/227 cases) was 14.4%. The strongest predictors of good clinical outcome were young age (p p = 0.008), high baseline ASPECTS (p p = 0.013) and extracranial recanalization (p = 0.001). The most significant predictors of SICH were low baseline ASPECTS (p p p = 0.002). Conclusion In our real-life series, the EVT for TLs’ AIS was feasible, safe and effective in improving 90-day functional outcome with acceptable morbi-mortality rates. ECAS increased the risk of SICH, independently from the antiplatelet therapy regimen.

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.

Objectives Bipolar disorder (BD) is a psychiatric condition causing shifts in mood, energy and activity levels severely altering the quality of life of the patients even in the euthymic phase. Although widely accepted, the neurobiological bases of the disorder in the euthymic phase remain elusive. This study aims at characterizing resting state functional activity of the BD euthymic phase in order to better understand the pathogenesis of the disease and build future neurobiological models. Methods Fifteen euthymic BD patients (10 females; mean age 40.2; standard deviation 13.5; range 20-61) and 27 healthy controls (HC) (21 females; mean age 37; standard deviation 10.6; range 22-60) underwent a 3T functional MRI scan at rest. Resting state activity was extracted through independent component analysis (ICA) run with automatic dimensionality estimation. Results ICA identified 22 resting state networks (RSNs). Within-network analysis revealed decreased connectivity in the visual, temporal, motor and cerebellar RSNs of BD patients vs HC. Between-network analysis showed increased connectivity between motor area and the default mode network (DMN) partially overlapping with the fronto-parietal network (FPN) in BD patients. Conclusion Within-network analysis confirmed existing evidence of altered cerebellar, temporal, motor and visual networks in BD. Increased connectivity between the DMN and the motor area network suggests the presence of alterations of the fronto-parietal regions, precuneus and cingulate cortex in the euthymic condition. These findings indicate that specific connectivity alterations might persist even in the euthymic state suggesting the importance of examining both within and between-network connectivity to achieve a global understanding of the BD euthymic condition.

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.

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 &plusmn, 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.

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®.

PurposeThe 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 methodsThe 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.ResultsFifty 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 (ConclusionsOur 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 numberNCT02390037

BackgroundSacral dural arteriovenous fistulas (DAVFs) are rare vascular abnormalities of the spine characterised by slowly progressive symptoms that can mimic different myelopathy disorders.ObjectTo report our single Institution experience with sacral DAVFs.MethodsWe retrospectively reviewed the clinical records of patients admitted from 1 January 2006 to 31 December 2016 with a diagnosis of sacral DAVFs, treated by endovascular embolisation or surgical clipping. Clinical presentation, imaging characteristics, treatment results and follow-up were analysed.ResultsWe identify 13 patients with sacral DAVFs supplied by lateral sacral arteries. Clinical presentation was characterised by different degrees of motor weakness and sphincter disturbances. In all patients, spinal MRI showed spinal cord hyperintensities with enhancement and prominent perimedullary vessels. Selective internal iliac angiography was mandatory to identify the exact location of the fistula. A complete embolisation was achieved in eight patients performing a single endovascular embolisation and in three patients performing a single surgical disconnection: two patients required combined procedures. Follow-up imaging showed a complete resolution of the spinal cord hyperintensities in 81% of patients and a reduction of the intramedullary enhancement in 91%. Gait improvement was observed in 73% of patients, while remaining stable in 27%. Sphincter disturbances improved in 36% of patients and remained stable in 64%.ConclusionAwareness of sacral location of DAVFs is critical because standard spinal angiography will not identify sacral supplies, unless internal iliac arteries are properly examined. In our experience, the endovascular treatment show results comparable to surgery when the fistula point is correctly disconnected.

Filum terminale arteriovenous fistulae are a rare type of arteriovenous shunt generally characterized by a single direct communication between the artery of the filum terminale and a single draining vein. These intradural arteriovenous shunts are three times more common in men than women (mean age 55 years). Symptoms are related to venous congestion, vascular hypertension, and a putative chronic steal phenomenon which result in spinal cord ischemia and myelopathy. Interestingly, hemorrhage has never been reported as a mode of presentation. MRI demonstrates increased flow voids and T2 changes involving the conus and the lower spinal cord, and these findings are not dissimilar from those seen with the more common type 1 spinal dural arteriovenous fistulae. Thus conventional spinal angiography is necessary for a definitive diagnosis and to localize exactly the site of the fistula. Both surgical interruption of the fistula and endovascular embolization are safe and effective therapeutic modalities. However, because of the very small caliber of the feeding artery, endovascular therapy is often not feasible; and thus, surgery remains the method more commonly utilized for their treatment. Definitive treatment consists of obliteration of the direct arteriovenous shunt. In this review, we describe the anatomy, pathophysiology, clinical presentation, imaging, and treatment options of these less common intradural arteriovenous shunts.

On a voluntary basis, 10 adolescents with hemiparesis due to cerebral palsy and 11 neurologically healthy control subjects participated in this proof-of-concept pilot study. The aim was to examine the effects of robot-assisted training for the unaffected arm in patients with hemiparetic cerebral palsy. Baseline comparison between the unaffected arm of the hemiparetic patients with cerebral palsy and the dominant arm of healthy control subjects showed significant differences on the Jebsen-Taylor Hand Function test and action planning ability tests. Within-group comparison after ten 30-minute sessions (five days a week for two consecutive weeks) of robot-assisted training for the unaffected arm showed significant improvements in patients with cerebral palsy on the Jebsen-Taylor Hand Function test (performed at both hands) and action planning ability test (evaluated at the unaffected arm). Our findings are in line with previous evidences of action planning deficits at the unaffected arm in patients with hemiparetic cerebral palsy and support the hypothesis that robot-assisted training for the unaffected arm may be useful to improve manual dexterity and action planning in patients with hemiparesis due to cerebral palsy.

GENetics of mOyaMoyA (GEN-O-MA) project is a multicenter observational study implemented in Italy aimed at creating a network of centers involved in moyamoya angiopathy (MA) care and research and at collecting a large series and bio-repository of MA patients, finally aimed at describing the disease phenotype and clinical course as well as at identifying biological or cellular markers for disease progression. The present paper resumes the most important study methodological issues and preliminary results. Nineteen centers are participating to the study. Patients with both bilateral and unilateral radiologically defined MA are included in the study. For each patient, detailed demographic and clinical as well as neuroimaging data are being collected. When available, biological samples (blood, DNA, CSF, middle cerebral artery samples) are being also collected for biological and cellular studies. Ninety-eight patients (age of onset mean ± SD 35.5 ± 19.6 years; 68.4% females) have been collected so far. 65.3% of patients presented ischemic (50%) and haemorrhagic (15.3%) stroke. A higher female predominance concomitantly with a similar age of onset and clinical features to what was reported in previous studies on Western patients has been confirmed. An accurate and detailed clinical and neuroimaging classification represents the best strategy to provide the characterization of the disease phenotype and clinical course. The collection of a large number of biological samples will permit the identification of biological markers and genetic factors associated with the disease susceptibility in Italy.

Divry van Bogaert Syndrome (DBS) is a familial juvenile-onset disorder characterized by livedo racemosa, white matter disease, dementia, epilepsy and angiographic finding of "cerebral angiomatosis". A similar syndrome including livedo racemosa and cerebrovascular disease, often associated with anticardiolipin antibodies, has been described as Sneddon Syndrome (SS) highlighting the question whether these two conditions have to be considered different entities or indeed different features of a unique syndrome. Herein, we report the clinical, neuroradiological, histopathological findings and follow up of three cases diagnosed as Divry-van Bogaert Syndrome, including an updated review of literature of both DBS and SS cases. Our findings support the assumption that DBS and SS are different disease entities. DBS is characterized by the typical angiographic feature of angiomatosis, a hereditary trait and a juvenile onset of cognitive impairment and leukoaraiosis, whereas SS has less severe manifestations of cerebrovascular disease associated with livedo racemosa but without the characteristic cerebral angiography. The report of our cases and the literature review underline the necessity of a detailed work-up and the collection of larger series to better clarify the DBS and SS phenotype and course.

Background Hippocampal abnormalities have been largely reported in patients with schizophrenia and bipolar disorder, and are considered to be involved in the pathophysiology of the psychosis. The hippocampus consists of several subfields but it remains unclear their involvement in the early stages of psychosis. Aim The aim of this study was to investigate volumetric alterations in hippocampal subfields in patients at the first-episode psychosis (FEP). Methods Magnetic resonance imaging (MRI) data were collected in 134 subjects (58 FEP patients; 76 healthy controls [HC]). A novel automated hippocampal segmentation algorithm was used to segment the hippocampal subfields, based on an atlas constructed from ultra-high resolution imaging on ex vivo hippocampal tissue. The general linear model was used to investigate volume differences between FEP patients and HC, with age, gender and total intracranial volume as covariates. Results We found significantly lower volumes of bilateral CA1, CA4, and granule cell layer (GCL), and of left CA3, and left molecular layer (ML) in FEP patients compared to HC. Only the volumes of the left hippocampus and its subfields were significantly lower in FEP than HC at the False Discovery Rate (FDR) of 0.1. No correlation was found between hippocampal subfield volume and duration of illness, age of onset, duration of medication, and Positive and Negative Syndrome Scale (PANSS). Conclusion We report abnormally low volumes of left hippocampal subfields in patients with FEP, sustaining its role as a putative neural marker of psychosis onset.

Because the visual cortices are contra-laterally organized, inter-hemispheric transfer tasks have been used to behaviorally probe how information briefly presented to one hemisphere of the visual cortex is integrated with responses resulting from the ipsi- or contra-lateral motor cortex. By forcing rapid information exchange across diverse regions, these tasks robustly activate not only gray matter regions, but also white matter tracts. It is likely that the response hand itself (dominant or non-dominant) modulates gray and white matter activations during within and inter-hemispheric transfer. Yet the role of uni-manual responses and/or right hand dominance in modulating brain activations during such basic tasks is unclear. Here we investigated how uni-manual responses with either hand modulated activations during a basic visuo-motor task (the established Poffenberger paradigm) alternating between inter- and within-hemispheric transfer conditions. In a large sample of strongly right-handed adults (n=49), we used a factorial combination of transfer condition [Inter vs. Within] and response hand [Dominant(Right) vs. Non-Dominant (Left)] to discover fMRI-based activations in gray matter, and in narrowly defined white matter tracts. These tracts were identified using a priori probabilistic white matter atlases. Uni-manual responses with the right hand strongly modulated activations in gray matter, and notably in white matter. Furthermore, when responding with the left hand, activations during inter-hemispheric transfer were strongly predicted by the degree of right-hand dominance, with increased right-handedness predicting decreased fMRI activation. Finally, increasing age within the middle-aged sample was associated with a decrease in activations. These results provide novel evidence of complex relationships between uni-manual responses in right-handed subjects, and activations during within- and inter-hemispheric transfer. The results suggest that the organization of the motor system exerts sophisticated functional effects. Moreover, our evidence of activation in white matter tracts is consistent with prior studies, confirming fMRI-detectable white matter activations which are systematically modulated by experimental condition.

Vertebral compression fractures (VFs) are observed in 30-50 % of patients affected by steroid-induced osteoporosis, with consequentially severe back pain and functional limitation. An alternative treatment to medical therapy for pain caused by recent VFs is percutaneous vertebroplasty (PVP). Patients were treated by PVP after careful selection, based on the presence of persistent pain not resolved by standard medical therapy, correlation between pain and level of the VF, and neuroradiological features. We performed PVP in 4 patients with generalized MG associated with recent steroid-induced symptomatic VFs. Relief from pain was very rapid, usually within 24 h, and retained at a 3-month evaluation. No severe complication or MG worsening were observed in the post-operative period. Although clinical indication for PVP is still controversial, in our experience PVP is a useful and safe tool to be considered in the management of recent steroid-induced symptomatic VFs in selected MG patients.

Background and purposeThe 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 methodsThe 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.ResultsFifty patients with 50 unruptured target IAs were enrolled. Mean aneurysm diameter was 8.82±6.15 mm. Thirty-eight aneurysms (76%) were small (ConclusionsThe 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 registrationNCT02390037.

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 (χ2 = 14.3; p = 0.001), ICP > 200 mmH2O (χ2 = 12.7; p = 0.002) and history of abuse or neglect (χ2 = 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.

Spinal dural arteriovenous fistulas (SDAVFs) are acquired diseases that represent the majority of all arteriovenous spinal shunts, leading to progressive and disabling myelopathy. Treatment is focused on accurately disconnecting the fistula point. We present our experience with the double-cone-beam CT fusion technique successfully applied to evaluate treatment results in a series of SDAVFs. Between November 2011 and December 2015 we performed double-DynaCT acquisition (pre- and post-embolisation) in 12 cases of SDAVF. A successful DynaCT fusion technique was only achieved in the group of patients with pre- and post-treatment images acquired at the same time as the treatment session, under general anaesthesia (4/12). DynaCT performed on different days proved to be inadequate for the automated fusion technique because of changes in the body position (8/12). A pre-treatment flat-panel cone-beam CT with contrast, at the time of diagnostic angiography, can be very helpful to detect the correct level of the fistula and the relationship between the fistula and the surrounding structures. In case of the endovascular approach, additional post-treatment native acquisition merged with the pre-treatment acquisition (double-cone-beam CT fusion technique) permits to immediately evaluate the distribution of the glue cast and to confirm the success of the procedure. • SDAVF treatment must be aimed to occlude the fistula point shunt. • Native post-operative cone-beam CT permits high-spatial-resolution imaging of the embolic cast. • The automated double-cone-beam CT fusion technique (pre/post) accurately demonstrates intravascular glue distribution after embolisation. • Patient movements should be avoided to obtain good technical results.

Direct exposure and cannulation of the superior ophthalmic vein (SOV) provides an alternative access to reach the cavernous sinus for carotid-cavernous fistula (CCF) embolization, when classic transvenous routes through the inferior petrosal sinus (IPS) or facial vein are not feasible. We have used indocyanine green (ICG)-videoangiography to study intraoperatively the flow inside the SOV in two cases of indirect CCF. In this paper, we report the operative technique and the result of ICG videoangiographic-guided cannulation of the SOV for endovascular treatment of CCF. Two male patients, of 59 and 66 years of age respectively, presented at our Institution with right decreased visual acuity, persistent binocular diplopia and painful ophthalmoplegia, chemosis and proptosis, due to right unilateral indirect CCF fistula. The endovascular transvenous approach failed in one case due to thrombosis of the inferior petrosal sinus (IPS) and the extreme tortuosity of the angular vein. In the other case, it was considered unfeasible due to an unfavourable vascular angioarchitecture. For this reason, an ICG videoangiographic-guided cannulation of the SOV, followed by endovascular obliteration of the CCF, was performed. CCF was cured in both cases with this approach. No additional neurological deficits and no complications due to SOV cannulation were registered during the hospital stay. There were no fistula recurrences during the mean follow-up of 18 months. ICG-videoangiography is a simple, fast and cost-effective technique that can be reliably applied in SOV cannulation for subsequent indirect CCF embolization.

BACKGROUND AND PURPOSE: Diffusion tensor imaging (DTI) offers a unique window on the connectivity changes, extending beyond the basal ganglia, which accompany the cognitive symptoms of Parkinson disease (PD). The primary purpose of this study was to assess the microstructural damage to cerebral white matter occurring in idiopathic PD. MATERIALS AND METHODS: Our sample included patients with PD without dementia (n = 10; Hoehn and Yahr stages I and II; Unified Parkinson Disease Rating Scale, 20.5 ± 8.3; and Mini-Mental State Examination, 28.3 ± 1.5) and age-matched healthy control subjects (n = 10). DTI was performed on a 1.5T scanner, and mean diffusivity (MD) and fractional anisotropy (FA) maps were obtained. Regions of interest (ROIs) were drawn on the major fiber bundles as well as on gray matter nuclei. RESULTS: In patients, the MD was increased at borderline significance in the substantia nigra but was unaltered in the thalamus, globus pallidus, putamen, and in the head of the caudate nucleus. The FA and MD were unaltered in the corticospinal tract in the midbrain and at the level of the internal capsule, and in the splenium of the corpus callosum. By contrast, the MD was increased and the FA was decreased in the genu of the corpus callosum and in the superior longitudinal fasciculus; in the cingulum, only the MD was altered. The observed changes were not significantly lateralized. CONCLUSIONS: Widespread microstructural damage to frontal and parietal white matter occurs already in the early stages of PD.

OBJECTIVE: Radical resection of meningiomas invading the superior sagittal sinus (SSS) presents several hazards. Some surgeons consider SSS invasion a contraindication for complete resection, and others advocate total resection with venous reconstruction. There is a lack of published large series to provide definitive guidelines for the surgical treatment of these complex cases. We report our 15-year experience with surgery of parasagittal meningiomas invading the SSS. METHODS: Between 1986 and 2001, 108 patients (73 women, 35 men; age range, 22–83 yr; mean age, 56.2 yr) underwent surgery at the Neurological Institute “C. Besta” of Milan for tumors invading the SSS. Parasagittal meningiomas not invading the SSS were excluded from this series. RESULTS: Simpson Grade I to II removal was achieved in 100 patients. Thirty patients with meningiomas totally occluding the SSS had complete resection of the encased portion of the sinus. Histological examination revealed 86 benign (79.6%), 16 atypical (14.8%), and 4 malignant (3.7%) meningiomas along with 2 hemangiopericytomas. There were two perioperative deaths. Serious complications included brain swelling (nine patients; 8.3%) and postoperative hematoma (two patients; 1.85%). Follow-up ranged from 19 to 223 months (mean, 79.5 mo). One patient was lost to follow-up. Tumors recurred in 15 patients (13.9%). After multivariate analysis, histological type, tumor size, and Simpson grade were confirmed as significant independent prognostic factors for recurrence. CONCLUSION: On the basis of our results, we conclude that if the sinus is partially invaded, it can be opened to obtain as complete a resection as possible and to attempt to preserve the patency of the sinus. If the sinus is obstructed, the portion of the sinus involved can be resected completely. In both situations, extreme care is vital to preservation of cortical veins, which may offer important collateral drainage. With our approach, good results are achieved and it is not necessary to reconstruct the sinus.

Assessment of cerebrovascular reactivity (CVR) is essential in cerebrovascular diseases, as exhausted CVR may enhance the risk of cerebral ischemic events. Transcranial Doppler (TCD) with a vasodilatory stimulus is currently used for CVR evaluation. Scanty data are available for Quantitative Magnetic Resonance Angiography (QMRA), which supplies higher spatial resolution and quantitative cerebral blood flow values. Aims of our pilot study were: (a) to assess safety and feasibility of CO2 administration during QMRA, (b) evaluation of CVR under QMRA compared to TCD, and (c) quantitative evaluation of blood flow from the major intracranial arterial vessels both at rest and after CO2. CVR during 5% CO2 air breathing was measured with TCD as a reference method and compared with QMRA. Fifteen healthy subjects (age 60.47 ± 2.24; male 11/15) were evaluated at rest and during CO2 challenge. Feasibility and safety of QMRA under CO2 were ensured in all subjects. CVR from middle cerebral artery territory was not statistically different between TCD and MRI (p0.05). Mean arterial pressure (MAP) and heart rate (HR) increased during QMRA and TCD (MAP p=0.007 and p=0.001; HR p=0.043 and p=0.068, respectively). Blood flow values from all intracranial vessels increased after CO2 inhalation (p0.001). CO2 administration during QMRA sessions is safe and feasible. Good correlation in terms of CVR was obtained comparing TCD and QMRA. Blood flow values significantly increased from all intracranial arterial vessels after CO2. Studies regarding CVR in physiopathological conditions might consider the utilization of QMRA both in routine clinical settings and in research projects.

We present the MRI findings in five patients with congenital muscular dystrophy (CMD) and merosin (laminin alpha2) deficiency, which was total in one and partial in four. In one patient with partial merosin deficiency, MRI was normal. The other four patients had supratentorial white matter abnormalities. In three, T2-weighted images revealed subcortical, deep lobar and periventricular high signal in white matter, while in the other there were only small peritrigonal areas of increased signal. On T1-weighted images, there was slightly low signal. Cortical abnormalities were absent. None of these changes were accompanied by symptoms or signs of central nervous system involvement. White matter abnormalities in a patient with CMD should prompt investigation of merosin.

Waung et al.1 described a case of Hirayama disease (HD) and concluded that CT venography may be helpful in evaluating patients in whom cervical MRI in flexion fails to demonstrate the typical features to clarify the pathogenesis of HD. Recently, the focus on the pathogenesis of HD seems to be shifting from arterial insufficiency to possible venous stagnation with impairment of spinal cord microcirculation. The hypothetic pathogenetic mechanisms of HD related to venous engorgement (VE) have been examined recently by Elsheikh et al.2 and by our group.3

INTRODUCTION: We report the experiences of 25 Italian centers, analyzing intra- and periprocedural complications of endovascular treatment of intracranial aneurysms using Silk (Balt Extrusion, Montmorency, France) and pipeline embolization devices (EV3 Inc, Irvine California). METHODS: Two hundred seventy-three patients with 295 cerebral aneurysms, enrolled in 25 centers in Italy and treated with the new flow-diverter devices, were evaluated; 142 patients were treated with Silk and 130 with pipeline (in one case, both devices were used). In 14 (5.2 \%) cases devices were used with coils. Aneurysm size was >15 mm in 46.9 \%, 5-15 mm in 42.2 \%, and

The goal of this study was to compare the rate of cerebral microembolization during carotid artery stenting (CAS) with proximal versus distal cerebral protection in patients with high-risk, lipid-rich plaque.Cerebral protection with filters partially reduces the cerebral embolization rate during CAS. Proximal protection has been introduced to further decrease embolization risk.Fifty-three consecutive patients with carotid artery stenosis and lipid-rich plaque were randomized to undergo CAS with proximal protection (MO.MA system, n = 26) or distal protection with a filter (FilterWire EZ, n = 27). Microembolic signals (MES) were assessed by using transcranial Doppler during: 1) lesion wiring; 2) pre-dilation; 3) stent crossing; 4) stent deployment; 5) stent dilation; and 6) device retrieval/deflation. Diffusion-weighted magnetic resonance imaging was conducted before CAS, after 48 h, and after 30 days.Patients in the MO.MA group had higher percentage diameter stenosis (89 ± 6% vs. 86 ± 5%, p = 0.027) and rate of ulcerated plaque (35% vs. 7.4%; p = 0.019). Compared with use of the FilterWire EZ, MO.MA significantly reduced mean MES counts (p0.0001) during lesion crossing (mean 18 [interquartile range (IQR): 11 to 30] vs. 2 [IQR: 0 to 4]), stent crossing (23 [IQR: 11 to 34] vs. 0 [IQR: 0 to 1]), stent deployment (30 [IQR: 9 to 35] vs. 0 [IQR: 0 to 1]), stent dilation (16 [IQR: 8 to 30] vs. 0 [IQR: 0 to 1]), and total MES (93 [IQR: 59 to 136] vs. 16 [IQR: 7 to 36]). The number of patients with MES was higher with the FilterWire EZ versus MO.MA in phases 3 to 5 (100% vs. 27%; p0.0001). By multivariate analysis, the type of brain protection was the only independent predictor of total MES number. No significant difference was found in the number of patients with new post-CAS embolic lesion in the MO.MA group (2 of 14, 14%) as compared with the FilterWire EZ group (9 of 21, 42.8%).In patients with high-risk, lipid-rich plaque undergoing CAS, MO.MA led to significantly lower microembolization as assessed by using MES counts.

Purpose To evaluate a retrievable inferior vena cava (IVC) filter in combination with low-intensity oral anticoagulation for prevention of pulmonary embolism (PE) in patients with malignancy complicated by thromboembolic disease. Materials and Methods From October 2005 to December 2009, 107 Bard G2 filters were placed in 106 patients. Forty-eight patients had deep vein thrombosis (DVT) alone, 53 had PE with DVT, and five had PE with no evidence of DVT. After an initial period of anticoagulation with heparin, low-intensity oral anticoagulant therapy to achieve a target International Normalized Ratio of 1.5–2.0 was instituted. Follow-up computed tomography to evaluate the pulmonary circulation, IVC, and lower limbs was performed at 3 and 6 months. Results PE recurred in three of 58 patients (5.2%). None of the 48 patients with DVT alone developed PE, nor was there any recurrence of DVT. The filter was removed in 14 patients (13.2%). No complications occurred during the retrieval procedure. A total of 16 complications occurred in seven patients: one migration (0.9%); four cases of vena cava thrombosis (3.7%), three of which were associated with recurrent PE (2.8%); one filter fracture (0.9%); and one IVC penetration (0.9%). Filter tilting greater than 15° occurred in six patients (5.7%) and was associated with other complications in five (4.7%). Conclusions In patients with malignancies complicated by venous thromboembolic disease, an IVC filter together with low-intensity anticoagulation may be a possible treatment strategy for PE prophylaxis. Controlled studies are warranted.

The authors demonstrate the feasibility of a new procedure to create intracranial interrupted microvascular anastomosis. Self-closing nitinol surgical clips were used for a pericallosal artery-pericallosal artery side-to-side bypass in a 52-year-old man harboring an unruptured large aneurysm located on the right A2 segment. The outflow artery was found to arise from the dome of the aneurysm, which was considered unsuitable for stand-alone clip ligation or coil occlusion. After bypass patency was intraoperatively confirmed using near-infrared indocyanine green videoangiography, the aneurysm and feeding artery were embolized with coils and safely occluded. Both postoperative courses were uneventful. The patient was discharged neurologically intact on the 5th postembolization day. Postprocedure angiography demonstrated no ipsilateral aneurysm filling and excellent bilateral distal outflow from the left anterior cerebral artery.

Antitumor and immunosuppressant treatment-related neurotoxicity can determine nonspecific clinical syndromes. Exclusion of other possible causes, among which tumor progression, appearance of paraneoplastic disease, renal or hepatic failure, diabetes or hypertension, is relevant. We report clinical and neuroradiological features in five patients with neurotoxic syndromes due to chemotherapy/radiotherapy or immunosuppression in the context of neoplastic disease/organ transplantation. Acute neurological syndrome developed in three patients after methotrexate (MTX), cyclosporine A, and L-asparaginase therapy, respectively. MRI showed posterior reversible encephalopathy in two cases and venous thrombosis with intraparenchymal hematoma in the third patient. Late onset clinical syndrome occurred in the last two patients, treated with MTX or radiation therapy for breast cancer metastasis and pituitary adenoma. Neuroimaging showed brain diffuse abnormalities. Patients affected by tumors suffer from increased risk for treatment-related toxicities. Appearance or worsening of neurological signs and symptoms challenge the clinician to discriminate between CNS involvement by the tumor, toxicity of drugs, parane-oplastic disease and infections. MRI has a key role in differential diagnosis. Close interaction between the neurologist, the oncologist and the neuroradiologist leads to the optimal management of patients.

The clinical phenotype of multiple acyl-CoA dehydrogenase deficiency in infancy is characterized by recurrent episodes of hypoketotic hypoglycemia and lipid storage myopathy. Brain damage has been described only as a consequence of severe and protracted hypoglycemia. We describe a child who experienced normal physical and psychomotor development until the age of 3 years, who then developed progressive intention tremors, dysarthria, ataxia, and spastic tetraparesis. Episodes of acute metabolic distress were never observed. Magnetic resonance imaging disclosed abnormal signals within the white matter of the brain and cerebellum, suggesting leukodystrophy. Gas chromatography/mass spectrometry analysis revealed abnormally high levels of glutaric acid, dicarboxylic acids, and glycine derivatives in urine. Riboflavin therapy was initiated at 4 years of age, when the patient had already lost control of trunk and head posture. Consistent improvement rapidly occurred after riboflavin supplementation. Glutaric aciduria type II may cause brain damage, in spite of the absence of acute metabolic distress, and should be considered in the differential diagnosis of leukodystrophies.

OBJECTIVE AND IMPORTANCE: Shunt catheter migration is a potential complication of cerebrospinal fluid shunting procedures. We report an unusual case of proximal shunt migration into the sphenoid sinus. To our knowledge, there have been no previous reports of shunt migration through the bony structures of the cranial base. CLINICAL PRESENTATION: A 41-year-old man who had had a cyst-to-peritoneum shunt placed 21 years earlier for a temporal lobe arachnoid cyst presented with cerebrospinal fluid rhinorrhea. Neuroradiological imaging revealed migration of the shunt catheter through the medial wall of the middle temporal fossa into the sphenoid sinus. INTERVENTION: The patient underwent shunt removal and repair of the dural defect. Intraoperatively, the proximal catheter tip was found in the sphenoid sinus with dural and bony erosion. The patient made an uneventful recovery. CONCLUSION: We present a unique long-term complication associated with intracranial shunt catheters. We hypothesize that excessive proximal catheter length and chronic cerebrospinal fluid pulsations contributed to migration of the catheter into the sphenoid sinus.

Rasmussen's encephalitis (RE) is a rare, progressive, chronic encephalitis characterised by drug-resistant epilepsy, progressive hemiparesis and mental impairment. It typically involves only one cerebral hemisphere, which becomes atrophic. We present neuroradiological findings in 13 children with RE. MRI was performed in all patients, fluorodeoxyglucose positron-emission tomography (PET) in three, Tc-99m hexamethylpropylenamine oxime single-photon emission computed tomography (SPECT) in two and proton MR spectroscopy ((1)HMRS) in two. MRI showed progression of the hemisphere atrophy, always prevalent in the region primarily involved (13 patients), spread of the abnormal signal in white matter (11) and cortex (10) and progression of atrophy of the head of the caudate nucleus (nine). Associated secondary changes were: atrophy of the contralateral cerebellar hemisphere (in four patients), the ipsilateral hippocampus (in five) and the brain stem (in five). The earliest CT and MRI abnormalities, seen between 1 day and 4 months after the first seizure (in 12 patients examined, nine of whom had MRI) in one cerebral hemisphere included: high signal on T2-weighted images in the cortex (seven patients) and white matter (nine), cortical atrophy usually involving the frontoinsular region, with mild or severe enlargement of the lateral ventricle (eight) and moderate atrophy of the head of the caudate nucleus (seven). Cortical swelling in the early stage of the disease was recognisable only in two patients. PET revealed hypometabolism, SPECT decreased perfusion, and (1)HMRS reduction of N-acetylaspartate in the affected hemisphere. PET and SPECT were usually performed in the late stages and did not provide specific findings. MRI thus demonstrates the progression of RE and may suggest the diagnosis in the early stages, often before the appearance of neurological deficits. Early diagnosis of RE may be crucial for selecting patients for aggressive medical therapy or major surgical interventions such as hemispherectomy.

Object. The aim of this study was to test the feasibility, safety, and efficacy of a new endovascular method for the treatment of giant intracranial aneurysms. This new method consists of combining a metallic stent with a liquid polymer; the stent is first placed across the neck of the aneurysm to reconstruct a tubular arterial lumen, followed by obliteration of the fundus of the aneurysm with an ethyl vinyl alcohol polymer. During its injection, the liquid polymer is contained within the aneurysm by temporarily inflating an occlusion balloon in the parent artery. Methods. Eleven patients harboring a giant aneurysm were successfully treated using this procedure. All aneurysms were excluded from the circulation, with preservation of the parent artery. In nine of the 11 patients, the 6-month follow-up angiogram demonstrated no recanalization of the aneurysm. In one patient who had a giant and partially clotted internal carotid artery bifurcation aneurysm, the follow-up angiogram demonstrated minimal recanalization. The complications in this series of patients included one death and one case of transient hemiparesis caused by watershed ischemia. Conclusions. The initial anatomical results and the clinical outcome in this small series of patients are very encouraging. The mortality and morbidity rates associated with this new endovascular treatment are superior to those associated with surgical clipping of giant aneurysms.

A clinical and magnetic resonance imaging (MRI) study on a selected group of 11 children, with a diagnosis of neurogenic arthrogryposis multiplex congenita (AMC) based on clinical, electromyographic, and muscle biopsy findings, is presented to determine the extent of central nervous system involvement in AMC. Family history, pregnancy, perinatal problems, other abnormalities, and epileptic seizures were reviewed. Neurologic examination, electroencephalography, intellectual assessment, and MRI study both of spinal cord and brain were performed. The clinical and laboratory findings disclosed evidence of spinal cord lesions with involvement of anterior horn cell function in all patients, and impairment of cerebral function in 5 patients. MRI revealed spinal cord atrophy in 3 patients, diffuse atrophy in 2 patients, and involved thoraco-lumbar segments in 1 patient. Cranial MRI studies demonstrated features of developmental brain abnormalities in 3 patients, cortical frontal atrophy in 2, and was normal in 4. In neurogenic AMC patients, MRI examination of the spinal cord and brain may help to clarify the pathogenesis of the disease and is helpful for prognostic and therapeutic purposes.