Utility of cardiac magnetic resonance imaging to differentiate cardiac sarcoidosis from arrhythmogenic right ventricular cardiomyopathy

Some patients diagnosed with arrhythmogenic right ventricular cardiomyopathy (ARVC) are eventually found to have cardiac sarcoidosis (CS). Accurate differentiation between these 2 conditions has implications for immunosuppressive therapy and familial screening. We sought to determine whether cardiac magnetic resonance imaging (MRI) could be used to identify the characteristic findings to accurately differentiate between CS and ARVC. Consecutive patients with a diagnostic MRI scan indicating CS and/or ARVC constituted the cohort. All patients diagnosed with CS had histologic confirmation of sarcoidosis, and all patients with ARVC met the diagnostic task force criteria. The cardiac MRI data were retrospectively analyzed to identify possible differentiating characteristics. Of the patients, 40 had CS and 21 had ARVC. Those with CS were older and had more left ventricular scar. The presence of mediastinal lymphadenopathy or left ventricular septal involvement was seen exclusively in the patients with CS (p <0.001). A family history of sudden cardiac death was seen only in the ARVC group (p = 0.012). The right ventricular ejection fraction and ventricular volumes were also significantly different between the 2 groups. In conclusion, patients with CS have significantly different cardiac MRI characteristics than patients with ARVC. The cardiac volume, in addition to the degree and location of cardiac involvement, can be used to distinguish between these 2 disease entities. The presence of mediastinal lymphadenopathy and left ventricular septal scar favors a diagnosis of CS and not ARVC. Consideration of CS should be given if these MRI findings are observed during the evaluation for possible ARVC.     

Increased temporal dispersion of myocardial repolarization in myotonic dystrophy type 1: beyond the cardiac conduction system

Objectives

To assess ventricular dysfunction and ventricular interaction after repair of Tetralogy of Fallot (ToF) employing echocardiography speckle-tracking and cardiac magnetic resonance imaging (CMR).

Background

Severe pulmonary regurgitation and right ventricular (RV) dysfunction are common after repair of ToF and may also affect the shape and function of the left ventricle (LV). Recent studies suggest that LV dysfunction may be of particular prognostic value.

Methods and results

Twenty-one consecutive adults with repaired ToF (15 male, mean age 38 ± 11 years, 7 with severe PR) underwent a comprehensive echocardiographic exam including speckle-tracking analysis, CMR and cardiopulmonary exercise testing. Twenty-one subjects without relevant heart disease served as controls. Echocardiographically measured RV diameters correlated with RV volumes obtained from CMR (r = 0.63; p = 0.006). In addition, a close correlation was found between RV and LV function on CMR (r = 0.74, p = 0.002), speckle-tracking LV and RV peak longitudinal 2D strain (r = 0.66, p = 0.003) and mitral and tricuspid annular plain systolic excursion (r = 0.71, p = 0.0003). While LV ejection fraction was normal in the majority of patients and not different from controls, LV longitudinal strain was significantly reduced in ToF patients (− 16.5 ± 3.3 vs. -20.5 ± 2.7%, p = 0.0001).

Conclusion

Left and right ventricular function both by CMR and speckle-tracking is interrelated in adults with repaired ToF. Despite normal LV ejection fraction, 2D longitudinal strain is significantly reduced in ToF patients, suggesting subclinical LV myocardial damage. Considering the potential prognostic value of LV dysfunction in ToF, this measurement may gain importance and should be included in future outcome studies.     

Role of stem cell factor and granulocyte colony-stimulating factor in remodeling during liver regeneration

Functional pluripotent characteristics have been observed in specific subpopulations of hepatic cells that express some of the known cholangiocyte markers. Although evidence indicates that specific cytokines, granulocyte macrophage colony-stimulating factors (GM-CSFs), and stem cell factors (SCFs) may be candidate treatments for liver injury, the role of these cytokines in intrahepatic biliary epithelium remodeling is unknown. Thus, our aim was to characterize the specific cytokines that regulate the remodeling potentials of cholangiocytes after 70% partial hepatectomy (PH). The expression of the cytokines and their downstream signaling molecules was studied in rats after 70% PH by immunoblotting and in small and large murine cholangiocyte cultures (SMCCs and LMCCs) by immunocytochemistry and real-time polymerase chain reaction (PCR). There was a significant, stable increase in SCF and GM-CSF levels until 7 days after PH. Real-time PCR analysis revealed significant increases of key remodeling molecules, such as S100 calcium-binding protein A4 (S100A4) and miR-181b, after SCF plus GM-CSF administration in SMCCs. SMCCs produced significant amounts of soluble and bound SCFs and GM-CSFs in response to transforming growth factor-beta (TGF-β). When SMCCs were incubated with TGF-β plus anti-SCF+GM-CSF antibodies, there was a significant decrease in S100A4 expression. Furthermore, treatment of SMCCs with SCF+GM-CSF significantly increased matrix metalloproteinases (MMP-2 and MMP-9) messenger RNA as well as miR-181b expression, along with a reduction of metalloproteinase inhibitor 3. Levels of MMP-2, MMP-9, and miR-181b were also up-regulated in rat liver and isolated cholangiocytes after PH. CONCLUSION: Our data suggest that altered expression of SCF+GM-CSF after PH can contribute to biliary remodeling (e.g., post-transplantation) by functional deregulation of the activity of key signaling intermediates involved in cell expansion and multipotent differentiation.     

Brain microstructure of subclinical apathy phenomenology in healthy individuals

Although apathy has been extensively studied in relation to neuropsychiatric disorders, it is still unclear whether, in healthy people, it should be considered as a physiological phenomenon or whether it is a risk factor for progression to clinical disturbances. Here, we investigated subclinical apathy phenomenology and its brain microstructural correlates in healthy individuals. We submitted 72 participants to a comprehensive clinical assessment, a high‐resolution structural MRI and a diffusion tensor imaging scan protocol. Data of individual microstructural (mean diffusivity and fractional anisotropy) variations were processed across genders in relation to the Apathy Rating Scale score. In females, subclinical apathy phenomenology was associated with microstructural variation of the bilateral thalami, the anterior thalamic radiation, the forceps major, and the corona radiate. These are white matter areas mostly connecting the thalami to the frontal and occipital cortices, regions that are known to be implicated in the expression of apathy in clinical samples. No significant relationship with brain microstructure was found in males who showed a positive correlation between subclinical apathy and somatic phenomenology of depression. In conclusion, our results show that in healthy individuals subclinical apathy phenomenology is associated with different mechanisms across genders, and raise the issue about whether brain microstructural changes associated with subclinical apathy in healthy females could be a precocious marker useful in the prediction of progression to more severe apathetic conditions. Hum Brain Mapp 34:3193–3203, 2013. © 2012 Wiley Periodicals, Inc.     

Post Graphemic Impairments Of Writing: The Case Of Micrographia

Micrographia (MG), an acquired impairment of writing characterized by diminution of letter size, coupled with slowing and loss of accuracy, is most frequently observed in the course of degenerative disease of basal ganglia, such as Parkinson’s Disease. Only a few cases of MG without concomitant extrapyramidal signs have been described, mostly following vascular lesion of the left basal ganglia.

In this article we report an isolated pattern of MG following an extensive traumatic cerebral lesion centered in the left basal ganglia. The nature of this impairment is analyzed according to a cognitive model of writing and interpreted as a specific example of peripheral dysgraphia.     

A Comparison between Endodontics and Implantology: An 8‐Year Retrospective Study

Purpose: The aim of this study was to compare endodontic and implant treatments and to evaluate their predictability over an 8‐year period on the basis of an analysis of survival data and a retrospective clinical study. Materials and Methods: A group of 40 partially edentulous patients were selected for this study. Their teeth had been endodontically treated and rehabilitated using gold alloy and ceramic restorations. In these patients, 65 osseointegrated implants were restored with single gold alloy–ceramic crowns and monitored on a yearly basis for 8 years with standardized periapical radiographs, using a polivynilsiloxane occlusal key as a positioner. A total of nine patients who did not attend the yearly follow‐up were excluded from the study. The Melloning and Triplett criteria were used to evaluate the clinical results obtained in the implant sites. The clinical results of the 56 endodontically treated teeth, restored with the fixed prosthesis of 40 patients, were analyzed according to probing depth as well as an assessment of the correct apical and coronal seals. The survival rate was calculated using the Kaplan–Meier method and the statistical significance was calculated using the chi‐square test. Results: During the follow‐up of the endodontically treated elements, seven failures were detected (83.34%) and the success rate of implants inserted in the same patients was equal to 80.8%, with nine implants lost in 8 years. The survival analysis of the elements treated with both therapies was not statistically significant (p = .757) and the confidence interval was between 0.2455 and 2.777. Conclusion: In view of the superimposable results between the two therapies, it should be noted that the endodontically treated teeth could be interested by different pathologies while the restoration of the atrophic edentulous ridge with an implant support is predictable when patients comply with correct oral hygiene and when the occlusal loads are axially distributed in implant‐protected occlusion.