Explore the vast range of titles available.

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

Wolfram syndrome (WS) is a recessive multisystem disorder defined by the association of diabetes mellitus and optic atrophy, reminiscent of mitochondrial diseases. The role played by mitochondria remains elusive, with contradictory results on the occurrence of mitochondrial dysfunction. We evaluated 13 recessive WS patients by deep clinical phenotyping, including optical coherence tomography (OCT), serum lactic acid at rest and after standardized exercise, brain Magnetic Resonance Imaging, and brain and muscle Magnetic Resonance Spectroscopy (MRS). Finally, we investigated mitochondrial bioenergetics, network morphology, and calcium handling in patient-derived fibroblasts. Our results do not support a primary mitochondrial dysfunction in WS patients, as suggested by MRS studies, OCT pattern of retinal nerve fiber layer loss, and, in fibroblasts, by mitochondrial bioenergetics and network morphology results. However, we clearly found calcium mishandling between endoplasmic reticulum (ER) and mitochondria, which, under specific metabolic conditions of increased energy requirements and in selected tissue or cell types, may turn into a secondary mitochondrial dysfunction. Critically, we showed that Wolframin (WFS1) protein is enriched at mitochondrial-associated ER membranes and that in patient-derived fibroblasts WFS1 protein is completely absent. These findings support a loss-of-function pathogenic mechanism for missense mutations in WFS1 , ultimately leading to defective calcium influx within mitochondria.

Studies of functional neurosurgery and electroencephalography in Parkinson's disease have demonstrated abnormally synchronous activity between basal ganglia and motor cortex. Functional neuroimaging studies investigated brain dysfunction during motor task or resting state and primarily have shown altered patterns of activation and connectivity for motor areas. L-dopa administration relatively normalized these functional alterations. The aim of this pilot study was to examine the effects of L-dopa administration on functional connectivity in early-stage PD, as revealed by simultaneous recording of functional magnetic resonance imaging (fMRI) and electroencephalographic (EEG) data. Six patients with diagnosis of probable PD underwent EEG-fMRI acquisitions (1.5 T MR scanner and 64-channel cap) before and immediately after the intake of L-dopa. Regions of interest in the primary motor and sensorimotor regions were used for resting state fMRI analysis. From the EEG data, weighted partial directed coherence was computed in the inverse space after the removal of gradient and cardioballistic artifacts. fMRI results showed that the intake of L-dopa increased functional connectivity within the sensorimotor network, and between motor areas and both attention and default mode networks. EEG connectivity among regions of the motor network did not change significantly, while regions of the default mode network showed a strong tendency to increase their outflow toward the rest of the brain. This pilot study provided a first insight into the potentiality of simultaneous EEG-fMRI acquisitions in PD patients, showing for both techniques the analogous direction of increased connectivity after L-dopa intake, mainly involving motor, dorsal attention and default mode networks.

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

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

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

An amendment to this paper has been published and can be accessed via a link at the top of the paper.An amendment to this paper has been published and can be accessed via a link at the top of the paper.

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

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

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