Psammomatoid juvenile ossifying fibroma: an analysis of 2 cases affecting the mandible with review of the literature

Juvenile ossifying fibroma (JOF) is a rare fibro-osseous neoplasm, defined as a variant of the ossifying fibroma that arises within the craniofacial bones. Two subgroups, juvenile psammomatoid ossifying fibroma (PsJOF) and juvenile trabecular ossifying fibroma, have been delineated by their histology. PsJOF occurs predominantly in the sinonasal and orbital bones. This work reports on 2 cases of extensive PsJOF in the body of the right mandible as well as reviews the literature regarding the radiographic and histologic features, treatment, and prognosis of PsJOF of the jaws.     

Severe Thrombotic Events Associated with Dengue Fever,Brazil

Dengue fever has been a major problem in hospital settings in Brazil for the past 15 years. The main concern has been the severe forms, i.e., dengue hemorrhagic fever and dengue shock syndrome. Hemorrhagic events of different degrees have also been a major concern. We report five cases of large vein thrombotic events associated with the acute phase of dengue fever, including a previously non-reported case of mesenteric vein thrombosis. Complications such as these could have been overlooked in the diagnosis of dengue fever, given that the major concern is the hemorrhagic event.Dengue fever frequently affects Brazil, where thousands of cases have been diagnosed annually and all four dengue virus serotypes (DENV-1 through DENV-4) have been reported, sometimes concurrently in the same region.^1,2 Hemorrhagic events of different degrees have often been described in dengue, but thrombotic events have not been extensively reported, despite the wide range of increased procoagulant activity during illness.^1,3–8 During a recent outbreak, several cases of thrombotic events affected large veins in dengue fever patients. We report cases of unusual and overlooked complication of dengue fever.From January 2011 through March 2011 during a local outbreak of dengue fever caused by DENV-1 and DENV-2 according to local health officials, five patients were given diagnoses by imaging techniques (pulmonary computed tomography angiography, cholangio-magnetic resonance imaging, and Doppler ultrasound of lower extremities) as having large vessel thrombosis. These patients were part of 92 serologically confirmed (by immunochromatographic strip test or IgM antigen-capture enzyme-linked immunosorbent assay) patients with dengue (60% women and 40% men, median age = 39 years, age range = 10–99 years) admitted to Monte Sinai Hospital (200 beds) in Juiz de Fora, (population = 500,000), Minas Gerais, Brazil. This study was approved by the Ethical Committee of Monte Sinai Hospital.Twenty-three patients (25%) were classified as having dengue shock syndrome or dengue hemorrhagic fever and 4 deaths were recorded (case-fatality rate = 4.3%). These thrombotic patients represented 5.4% of all dengue inpatients. All thrombotic events were identified within the first five days of illness, and all patients had symptoms compatible with the reported thrombotic event at hospital admission. No thrombotic events were identified among the remaining patients during hospitalization, and none of the patients them received drugs for thrombosis prophylaxis, which is contraindicated in dengue.The demographic and clinical characteristics of the five patients are summarized in Figure 1), which was associated with jaundice and severe sepsis by Escherichia coli demonstrated by blood culture. Known risk factors for thrombotic events such as smoking, use of oral contraceptives, and being overweight were absent in all patients and none had had any similar diagnosis or symptoms.Open in a separate windowFigure 1.T1-weighted cholangio-magnetic resonance image with contrast of dengue patient 5 showing the superior mesenteric vein (SMV) with a large thrombus (arrow) occupying the vein lumen. SMA = superior mesenteric artery; IVC = inferior vena cava; Ao = aorta.

Table 1

Demographic, clinical, and laboratory characteristics of five dengue fever patients with thrombotic events, Brazil^*

Morphometry of corpus callosum in Williams syndrome: shape as an index of neural development

Brain abnormalities in Williams syndrome (WS) have been consistently reported, despite few studies have devoted attention to connectivity between different brain regions in WS. In this study, we evaluated corpus callosum (CC) morphometry: bending angle, length, thickness and curvature of CC using a new shape analysis method in a group of 17 individuals with WS matched with a typically developing group. We used this multimethod approach because we hypothesized that neurodevelopmental abnormalities might result in both volume changes and structure deformation. Overall, we found reduced absolute CC cross-sectional area and volume in WS (mean CC and subsections). In parallel, we observed group differences regarding CC shape and thickness. Specifically, CC of WS is morphologically different, characterized by a larger bending angle and being more curved in the posterior part. Moreover, although CC in WS is shorter, a larger relative thickness of CC was found in all callosal sections. Finally, groups differed regarding the association between CC measures, age, white matter volume and cognitive performance. In conclusions, abnormal patterns of CC morphology and shape may be implicated in WS cognitive and behavioural phenotype.     

Training charter in epilepsy surgery added competence

The present Training Charter in Epilepsy Surgery Added Competence constitutes the third stage of a program initiated by the European Society for Stereotactic and Functional Neurosurgery (ESSFN) and substantiated in close collaboration with the Union Européennedes Médecins Spécialists (UEMS) and the European Association of Neurosurgical Societies (EANS). This program aims to raise the standards of clinical practice by guiding education and quality control concepts. The particular sections of this Charter include: definitions and standards of added competence training, relations of the Epilepsy Unit with the Neurosurgical Department, duration of epilepsy surgery fellowship, institution and training program director requirements, operative totals for epilepsy surgery, educational program, individual requirements, and evaluation and qualification of the trainees. The specification of all these requirements is expected to improve harmonisation and quality of epilepsy surgery practice across Europe, and enhance the clinical activity and the scientific productivity of existing neurosurgical centres.     

Methylglyoxal alters glucose metabolism and increases AGEs content in C6 glioma cells

Methylglyoxal is a dicarbonyl compound that is physiologically produced by enzymatic and non-enzymatic reactions. It can lead to cytotoxicity, which is mainly related to Advanced Glycation End Products (AGEs) formation. Methylglyoxal and AGEs are involved in the pathogenesis of Neurodegenerative Diseases (ND) and, in these situations, can cause the impairment of energetic metabolism. Astroglial cells play critical roles in brain metabolism and the appropriate functioning of astrocytes is essential for the survival and function of neurons. However, there are only a few studies evaluating the effect of methylglyoxal on astroglial cells. The aim of this study was to evaluate the effect of methylglyoxal exposure, over short (1 and 3?h) and long term (24?h) periods, on glucose, glycine and lactate metabolism in C6 glioma cells, as well as investigate the glyoxalase system and AGEs formation. Glucose uptake and glucose oxidation to CO₂ increased in 1?h and the conversion of glucose to lipids increased at 3?h. In addition, glycine oxidation to CO₂ and conversion of glycine to lipids increased at 1?h, whereas the incorporation of glycine in proteins decreased at 1 and 3?h. Methylglyoxal decreased glyoxalase I and II activities and increased AGEs content within 24?h. Lactate oxidation and lactate levels were not modified by methylglyoxal exposure. These data provide evidence that methylglyoxal may impair glucose metabolism and can affect glyoxalase activity. In periods of increased methylglyoxal exposure, such alterations could be exacerbated, leading to further increases in intracellular methylglyoxal and AGEs, and therefore triggering and/or worsening ND.