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Dyke-Davidoff-Masson Syndrome (DDMS) is a rare neurological disease with an unknown incidence. The manifestations of DDMS are variable, while typical symptoms are seizures, hemiparesis, and mental retardation. Here, we present a case involving a 19-year-old male patient who presents with headaches, mood changes, and a history of seizures during childhood. Based on the neuroimages, a diagnosis of DDMS was established. The application of sertraline hydrochloride as a therapeutic intervention has alleviated the symptoms. This case report illustrates the importance of understanding the clinical features of DDMS based on imaging.
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The presence of heavy metals in soil has gained considerable attention due to their potential risks to ecosystems and human health. In this study, a thorough soil investigation was performed in the hilly region of central Hainan, which was formerly regarded as an area with the highest ecological environmental quality. A total of 7094 soil samples were systematically collected with high density over a large area. Simultaneously, a detailed investigation was conducted on the surrounding environment of each sampling point, including environmental factors such as soil, land use and crop types. The soil samples were analysed for heavy metals, pH, organic matter, and other parameters. The soil heavy metal pollution level, ecological risk and health risk were evaluated using the geo-accumulation index and the potential ecological risk index. The findings showed that the average contents of the heavy metals As, Cd, Cr, Cu, Hg, Ni, Pb and Zn in the soil were 1.68, 0.042, 24.2, 6.49, 0.0319, 7.06, 29.6 and 49.8 mg·kg-1 respectively. Except for Hg, the mean values of the other heavy metals were either lower than or similar to the background values of Hainan. Also, only a few localised areas showed contamination by heavy metals. The primary sources of heavy metals, identified by a positive matrix factorisation model, could be categorised into four types: natural sources related to the soil formation process from acidic intrusive rocks (such as granite); natural sources primarily influenced by atmospheric deposition; anthropogenic sources associated with agricultural activities; and natural sources related to the soil formation process from middle-mafic intrusive rocks and black shales. The correlation analysis and variance analysis findings suggested that the content of heavy metals in the soil was primarily associated with the parent rock. The study area generally had low heavy metal levels and was not significantly polluted. However, agricultural activities still affected the enrichment of heavy metals. Therefore, it is imperative to remain vigilant about the ecological risks linked to soil heavy metals while continuing land development and expanding agricultural activities in the future. These findings indicate that conducting high-density soil surveys can enhance our understanding of regional soil heavy metals and enable reliable recommendations for agricultural planning. Whether in areas with low pollution risk or potential pollution risk, it is recommended that high-density soil surveys be conducted provide scientific guidance for further agricultural development.
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Monitoramento Ambiental , Metais Pesados , Poluentes do Solo , Solo , Metais Pesados/análise , China , Poluentes do Solo/análise , Medição de Risco , Solo/química , HumanosRESUMO
Neurological diseases are a major cause of the global burden of disease. Although the mechanisms of the occurrence and development of neurological diseases are not fully clear, most of them are associated with cells mediating neuroinflammation. Yet medications and other therapeutic options to improve treatment are still very limited. Single-cell RNA sequencing (scRNA-seq), as a delightfully potent breakthrough technology, not only identifies various cell types and response states but also uncovers cell-specific gene expression changes, gene regulatory networks, intercellular communication, and cellular movement trajectories, among others, in different cell types. In this review, we describe the technology of scRNA-seq in detail and discuss and summarize the application of scRNA-seq in exploring neurological diseases, elaborating the corresponding specific mechanisms of the diseases as well as providing a reliable basis for new therapeutic approaches. Finally, we affirm that scRNA-seq promotes the development of the neuroscience field and enables us to have a deeper cellular understanding of neurological diseases in the future, which provides strong support for the treatment of neurological diseases and the improvement of patients' prognosis.
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Electrochemical CO2 reduction reaction (CO2RR) in acid can solve alkalinity issues while highly corrosive and reductive acidic electrolytes usually cause catalyst degradation. Inhibiting catalyst degradation is crucial for the stability of acidic CO2RR. Here, we reveal the microenvironment changes of dynamic Bi-based catalysts and develop a pulse chronoamperometry (CA) strategy to improve the stability of acidic CO2RR. In situ fluorescence mappings show that the local pH changes from neutral to acid, and the in situ Raman spectra reveal the dynamic evolution of interfacial water structures in the microenvironment. We propose that the surface charge properties of dynamic catalysts affect the competitive adsorption of K+ and protons, thereby causing the differences in local pH and CO2RR intermediate adsorption. We also develop a pulse CA strategy to reactivate catalysts, and the stability of acidic CO2RR is improved by 2 orders of magnitude for 100 h operation, which is higher than most reports on the stability of acidic CO2RR. This work gives insights on how microenvironment changes affecting the stability of acidic CO2RR, and provides guidance for designing stable catalysts in acidic electrolytes.
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The carbon dioxide reduction reaction (CO2RR) driven by electricity can transform CO2 into high-value multi-carbon (C2+) products. Copper (Cu)-based catalysts are efficient but suffer from low C2+ selectivity at high current densities. Here La(OH)3 in Cu catalyst is introduced to modify its electronic structure towards efficient CO2RR to C2+ products at ampere-level current densities. The La(OH)3/Cu catalyst has a remarkable C2+ Faradaic efficiency (FEC2+) of 71.2% which is 2.2 times that of the pure Cu catalyst at a current density of 1,000 mA cm-2 and keeps stable for 8 h. In situ spectroscopy and density functional theory calculations both show that La(OH)3 modifies the electronic structure of Cu. This modification favors *CO adsorption, subsequent hydrogenation, *COâ*COH coupling, and consequently increases C2+ selectivity. This work provides a guidance on facilitating C2+ product formation, and suppressing hydrogen evolution by La(OH)3 modification, enabling efficient CO2RR at ampere-level current densities.
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BACKGROUND: Biliary atresia (BA) is a rare fatal liver disease in children, and the aim of this study was to develop a method to diagnose BA early. METHODS: We determined serum levels of matrix metalloproteinase-7 (MMP-7), the results of 13 liver tests, and the levels of 20 bile acids, and integrated computational models were constructed to diagnose BA. RESULTS: Our findings demonstrated that MMP-7 expression levels, as well as the results of four liver tests and levels of ten bile acids, were significantly different between 86 BA and 59 non-BA patients (P < 0.05). The computational prediction model revealed that MMP-7 levels alone had a higher predictive accuracy [area under the receiver operating characteristic curve (AUC) = 0.966, 95% confidence interval (CI): 0.942, 0.989] than liver test results and bile acid levels. The AUC was 0.890 (95% CI 0.837, 0.943) for liver test results and 0.825 (95% CI 0.758, 0.892) for bile acid levels. Furthermore, bile levels had a higher contribution to enhancing the predictive accuracy of MMP-7 levels (AUC = 0.976, 95% CI 0.953, 1.000) than liver test results. The AUC was 0.983 (95% CI 0.962, 1.000) for MMP-7 levels combined with liver test results and bile acid levels. In addition, we found that MMP-7 levels were highly correlated with gamma-glutamyl transferase levels and the liver fibrosis score. CONCLUSION: The innovative integrated models based on a large number of indicators provide a noninvasive and cost-effective approach for accurately diagnosing BA in children. Video Abstract (MP4 142103 KB).
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Soil heavy metal distributions exhibit regional heterogeneity due to the complex characteristics of parent materials and soil formation processes, emphasizing the need for appropriate regional standards prior to assessing soil risks. This study focuses on Hainan Island and employs the Multi-purpose Regional Geochemical Survey dataset to establish heavy metal geochemical baseline and background values for soil using an iterative method. Geographical detector analysis reveals that parent materials are the primary factor influencing heavy metal distribution, followed by soil types and land use. Heavy metal geochemical baseline values are established for the island's three environments and administrative regions. Notably, a universal geochemical baseline value cannot adequately represent regional variations in heavy metal distribution, with parent materials playing a crucial role in various scenarios. Locally applicable values based on parent material are the most representative for Hainan Island. This study provides a reference framework for developing region-specific environmental baseline values for soil heavy metal assessments.
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Direct seawater electrolysis is promising for sustainable hydrogen gas (H2) production. However, the chloride ions in seawater lead to side reactions and corrosion, which result in a low efficiency and poor stability of the electrocatalyst and hinder the use of seawater electrolysis technology. Here we report a corrosion-resistant RuMoNi electrocatalyst, in which the in situ-formed molybdate ions on its surface repel chloride ions. The electrocatalyst works stably for over 3000 h at a high current density of 500 mA cm-2 in alkaline seawater electrolytes. Using the RuMoNi catalyst in an anion exchange membrane electrolyzer, we report an energy conversion efficiency of 77.9% and a current density of 1000 mA cm-2 at 1.72 V. The calculated price per gallon of gasoline equivalent (GGE) of the H2 produced is $ 0.85, which is lower than the 2026 technical target of $ 2.0/GGE set by the United Stated Department of Energy, thus, suggesting practicability of the technology.
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Cloretos , Gasolina , Corrosão , Ânions , Membranas , Halogênios , Água do MarRESUMO
Pericytes surrounding endothelial cells in the capillaries are emerging as an attractive cell resource, which can show a large variety of functions in ischemic stroke, including preservation of the blood-brain barrier, regulation of immune function, and support for cerebral vasculature. These functions have been fully elucidated in previous studies. However, in recent years, increasing evidence has shown that pericytes play an important role in neurological recovery after ischemic stroke due to their regenerative function which can be summarized in two aspects according to current discoveries, one is that pericytes are thought to be multipotential themselves, and the other is that pericytes can promote the differentiation of oligodendrocyte progenitor cells (OPCs). Considering the neuroprotective treatment for stroke has not been much progressed in recent years, new therapies targeting pericytes may be a future direction. Here, we will review the beneficial effects of pericytes in ischemic stroke from two directions: the barrier and vascular functions and the regenerative functions of pericytes.
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AVC Isquêmico , Acidente Vascular Cerebral , Humanos , Células Endoteliais , Pericitos/fisiologia , Acidente Vascular Cerebral/terapia , Barreira HematoencefálicaRESUMO
Main group indium materials have been known as promising electrocatalysts for two-electron-involved carbon dioxide reduction to produce formate, which is a key energy vector in many industrial reactions. However, the synthesis of two-dimensional (2D) monometallic nonlayered indium remains a great challenge. Here, we present a facile electrochemical reduction strategy to transform 2D indium coordination polymer into elemental indium nanosheets. In a customized flow cell, the reconstructed metallic indium exhibits a high Faradaic efficiency (FE) of 96.3% for formate with a maximum partial current density exceeding 360 mA cm-2 and negligible degradation after 140 h operation in 1 M KOH solution, outperforming the state-of-the-art indium-based electrocatalysts. Moreover, in and ex situ electrochemical analysis and characterizations demonstrate that the enhanced exposure of active sites and mass/charge transport at the CO2 gas-catalyst-electrolyte triple-phase interface and the restrained electrolyte flooding are contributing to producing and stabilizing carbon dioxide radical anion intermediates, thus leading to superior catalytic performance.
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Background: Infants with Alagille syndrome (ALGS) need to be promptly differentiated from biliary atresia (BA) at an early stage. ALGS is an autosomal, dominant, multisystem disorder with variable phenotypic penetrance caused by heterozygous mutations in JAG1 or NOTCH2, which encode the Notch signaling pathway. Case presentation: We report two cases, both with cholestatic jaundice as the main manifestation, in which BA was excluded and finally diagnosed as ALGS based on characteristic facial features, serological tests, imaging, laparoscopic cholangiography, pathology and genetic findings. Both cases are novel mutant genes on chromosome 20 that have not been reported in the literature. The mutation in patient 1 was a novel heterozygous nonsense mutation (NM_000214 exon20, c.2419G > T, p.E807Ter), which was a spontaneous mutation. Followed up to 1 year and 6 months, the symptoms resolved with ursodeoxycholic acid and cholestyramine, and the jaundice has now subsided. Patient 2 was a novel heterozygous frameshift mutation (NM_000214 exon19, c.2367-2368dupTC, p.P790Lfs*31), which was inherited from his mother. This patient was followed up to 9 months and is currently awaiting liver transplantation. Conclusion: Both cholestatic infants reported combined to exclude BA, avoid Kasai portoenterostomy (KPE), and definitively diagnose ALGS. Broadening the spectrum of JAG1 gene mutations.
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To understand the enrichment factors and pollution levels of heavy metals in agricultural soils in the semi-arid region of Hainan island, 1818 surface soil samples were collected in Gancheng Town and analyzed for their heavy metal contents and physicochemical composition. Correlation analysis was used to determine the heavy metal enrichment factors. The geo-accumulation index (Igeo), comprehensive ecological risk index (RI), and hazard index (HI), as well as carcinogenic risk (CR), were used to assess the degree of pollution and health risk. Positive matrix factorization (PMF) was used to determine the primary sources of pollution and priority sources. The average values of heavy metal contents in the topsoil were 22.7, 0.128, 33.4, 14.5, 0.032, 9.32, 32.5, and 43.3 mg·kg-1 for As, Cd, Cr, Cu, Hg, Ni, Pb, and Zn, respectively. With the exception of Zn, the concentrations of other heavy metals in the topsoil were higher than the soil background values of Hainan, showing different degrees of heavy metal accumulation effect. The Igeo revealed that the major pollutant element in soils was As, followed by Cd and Cu. The RI showed that the proportion of soil samples that were high-risk level or worse was 29.4% of the total number of samples, among which As was the major source of risk. The health risk assessment results indicated that As, Cr, and Ni exposure presented carcinogenic risk for children with high CR values. Based on PMF, four major sources of heavy metals were identified in the study area. Hg was derived mainly from industrial sources, and As was closely associated with agricultural activities. Ni, Cu, Cr, and Zn were related to soil parent materials. Pb and Cd were associated with agricultural activities and traffic emissions. The PMF models combined with correlation analysis were useful for estimating the source apportionment of heavy metals in soils.
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Mercúrio , Metais Pesados , Poluentes do Solo , Cádmio/análise , Carcinógenos/análise , Criança , China , Monitoramento Ambiental/métodos , Humanos , Chumbo/análise , Mercúrio/análise , Metais Pesados/análise , Medição de Risco , Solo/química , Poluentes do Solo/análiseRESUMO
The relationship between gut microbiota and brain function has been studied intensively in recent years, and gut microbiota has been linked to a couple of neurological disorders including stroke. There are multiple studies linking gut microbiota to stroke in the "microbiota-gut-brain" axis. The aryl hydrocarbon receptor (AHR) is an important mediator of acute ischemic damage and can result in subsequent neuroinflammation. AHR can affect these responses by sensing microbiota metabolites especially tryptophan metabolites and is engaged in the regulation of acute ischemic brain injury and chronic neuroinflammation after stroke. As an important regulator in the "microbiota-gut-brain" axis, AHR has the potential to be used as a new therapeutic target for ischemic stroke treatment. In this review, we discuss the research progress on AHR regarding its role in ischemic stroke and prospects to be used as a therapeutic target for ischemic stroke treatment, aiming to provide a potential direction for the development of new treatments for ischemic stroke.
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Isquemia Encefálica , AVC Isquêmico , Microbiota , Acidente Vascular Cerebral , Humanos , Receptores de Hidrocarboneto ArílicoRESUMO
Meconium peritonitis (MP) combined with intestinal atresia (IA) is a rare neonatal condition, and it is even rarer in combination with biliary atresia (BA). We describe a case of an infant who developed short bowel syndrome after partial intestinal resection due to MP and IA, along with a Santullienterostomy. During continuous enteral and parenteral nutrition, the stool color became paler. BA was identified by elevated direct bilirubin (DBIL), gamma-glutamyltransferase (GGT), serum matrix metalloproteinase-7 (MMP-7), and hepatobiliary ultrasound; then, Kasai portoenterostomy (KPE) was performed promptly. The Roux-en-Y limb was adjusted intraoperatively to preserve the maximum length of the small intestine while closing the enterostomy. After the operation, the infant gradually adapted to enteral nutrition, his bilirubin level returned to normal, and his weight gradually caught up to the normal range. Although rare, BA should be suspected when MP is combined with IA and when the stool becomes paler in color in the enterostomy state.
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The accumulation of some harmful elements in plants from intensive production systems pose a serious threat to human health. In this study, seven heavy metals(Cu, Pb, Zn, Cr, Cd, As, and Hg) and their distribution characteristics in the crops, vegetables, and fruits were analyzed alongside single factor evaluation and Nemero index analysis. Combined with dietary recommended consumption data from the Chinese Nutrition Society, the dietary exposure of heavy metals were further analyzed, and a consequent safety risk assessment was conducted. A total of 673 crop, vegetable, and fruit samples were collected from typical intensive production systems in Hainan Province. The results showed that the content of Cu, As, and Hg in the 673 plant samples was below the value of the national food standard. The exceed rates of Pb, Zn, Cr, and Cd were 2.67%, 3.71%, 2.53%, and 3.71%, respectively. The heavy metal comprehensive pollution degree of six species of plants showed the trend of leafy vegetables > tuber crops > non-leafy vegetables > legume crops > fruits > cereals. In particular, Cr in leafy vegetables showed significantly higher hazard quotients(HQ) than that in other types, and exceeded 1, suggesting a high potential health risk via the ingestion of heavy metals through leafy vegetables. The relatively lower hazard index(HI) values of tuber crops, non-leafy vegetables, legume crops, fruits, and cereals suggest that these types of plants are more suitable for intensive production systems.
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Metais Pesados , Poluentes do Solo , China , Grão Comestível/química , Monitoramento Ambiental , Frutas/química , Humanos , Metais Pesados/análise , Medição de Risco , Solo , Poluentes do Solo/análise , VerdurasRESUMO
Committed to improving the utilization efficiency of Pt atoms and accurately controlling the morphology and composition of nanocatalysts to boost the Pt-based catalyst performance has become the focus of research. Herein, the PtRhNi truncated triangular nanoflakes (TA-NFs) catalyst with a unique PtRh-rich surface structure was successfully prepared by an effective one-pot synthetic method based on the galvanic replace reaction. The freestanding 2D nanostructure of PtRhNi TA-NFs, intrinsically possessing much high specific surface area and surface atomic, and the PtRh-rich characteristics of the surface is undoubtedly the most feasible model to simultaneously achieve high atom utilization. Benefiting from this novel structure, the as-obtained PtRhNi TA-NFs nanocatalyst exhibits excellent performance for ORR and MOR, delivering a mass activity of 0.92 A mgpt-1 for ORR, which is 2.03, 1.64, and 6.9-fold higher than that of PtRhNi nanoparticls (NPs), PtNi truncated triangle nanoflakes (TA-NFs) and commercial Pt/C, respectively. In addition, after 20 k cycles ADT test, PtRhNi TA-NFs show only 10 mV negative shift of half-wave potential and retain 70% of initial value of mass activity. Furthermore, a mass activity is 1.28 A mgpt-1 is achieved after applying this unique nanocatalyst for MOR, which is 1.28,1.5, and 2.6 times higher than that of PtRhNi NPs, PtNi TA-NFs and Pt/C, respectively. Impressively, the PtRhNi TA-NFs nanocatalyst shows an ultrahigh stability even after 2 k cycles ADT measurement in acid solution, and the mass activity is only drop 2% of initial value. This work provides a new strategy to synthesis high-performance of bifunction Pt-based electrocatalyst for ORR and MOR fuel cells.
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OBJECTIVE: To summarize the clinical characteristics and treatment of type â ¢-b congenital intestinal atresia (CIA). METHODS: The clinical data of 12 type â ¢-b CIA treated in the Children's Hospital of Zhejiang University School of Medicine from January 2015 to December 2017 were analyzed retrospectively. RESULTS: Of the 12 patients diagnosed as type â ¢-b CIA in operation, treatment was refused during operation by their parents in 2 cases. For one child, only the proximal intestine was partly resected in the first operation, dilatation and dysplasia of the duodenum was diagnosed and total duodenum was resected and sutured in the second operation, as the child had postoperative intestinal obstruction. For one child, due to the long distal normal intestine, distal apple-peel like intestine was partly resected without mesenteric reformation. For the rest 8 children total duodenum resection and mesenteric reformation were performed. During the postoperative follow-up, one case was early rejected for further treatment by parents, one case died from complex congenital heart disease, 5 cases had the complication of short bowel syndrome. All 8 survival children received parenteral nutrition support after operation, 5 of whom received parenteral nutrition support for more than 42 days, and they were followed up for 1-3 years after discharge. The short-time efficacy was satisfactory. CONCLUSIONS: For children with type â ¢-b CIA, the distal apple-peel like intestine should be preserved as much as possible, the mesenteric reformation should be performed and the proximal dilated bowel should be partly resected and sutured. Postoperative nutritional support and early intestinal rehabilitation contribute to the compensation for rest intestines.
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Atresia Intestinal , Intestinos , Criança , Humanos , Atresia Intestinal/complicações , Atresia Intestinal/cirurgia , Atresia Intestinal/terapia , Intestinos/cirurgia , Nutrição Parenteral , Estudos Retrospectivos , Síndrome do Intestino Curto/complicações , Resultado do TratamentoRESUMO
OBJECTIVE: To evaluate the efficacy and safety of transumbilical single-site laparoscopic surgery for congenital duodenal obstruction (CDO) in neonates. METHODS: A retrospective analysis of clinical data of 15 patients with CDO undergoing transumbilical single-site laparoscopic treatment during November 2017 and January 2018 (single-site group), and 20 patients with CDO undergoing conventional three-hole laparoscopic treatment during August 2017 and October 2017 (three-hole group) was performed. All patients were from the Children's Hospital, Zhejiang University School of Medicine. The operation time, time of initial feeding, time of adequate feeding, length of hospital stay after operation and postoperative complications were compared between two groups. RESULTS: The operations were completed in all patients. No patient converted to laparotomy, and no massive hemorrhage was observed during operation. The operation time of single-site group was (90±10) min for patients with duodenal diamond-shaped anastomosis and (81±15) min for patients with Ladd operation, while those of three-hole group were (85±9) min and (72±11) min, respectively. Postoperative initial feeding time of single-site group was (5.0±1.0) d, and that of the three-hole group was (4.8±0.8) d. The adequate feeding time was (9.0±1.2) d in the single-site group, and (9.3±0.8) d in the three-hole group. The length of hospital stay after operation was (11.2±2.5) d in the single-site group, and (11.5±2.8) d in the three-hole group. There was no significant difference in operation time, postoperative initial feeding time, adequate feeding time and length of hospital stay after operation between two groups (all P>0.05). CONCLUSIONS: Transumbilical single-site laparoscopic surgery for CDO in neonates is safe and effective, and the postoperative abdominal scar is more hidden.
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Obstrução Duodenal , Laparoscopia , Criança , Obstrução Duodenal/cirurgia , Humanos , Recém-Nascido , Tempo de Internação , Complicações Pós-Operatórias , Estudos RetrospectivosRESUMO
Angiomyofibroblastoma (AMFB) is a rare, benign, mesenchymal tumor that occurs mainly in the female lower genital tract. We report on a large posterior paravaginal AMFB that presented as a buttock mass, describing the magnetic resonance imaging (MRI) features of the disease. The tumor displays heterogeneous signal intensity on T2-weighted MRI and fast and persistent inhomogeneous enhancement on dynamic gadolinium-enhanced MRI.