Silvia Pradella

Fetal striatal grafting slows motor and cognitive decline of Huntington's disease

Objective To assess the clinical effect of caudate-putaminal transplantation of fetal striatal tissue in Huntingtons disease (HD). Methods We carried out a follow-up study on 10 HD transplanted patients and 16 HD not-transplanted patients. All patients were evaluated with the Unified HD Rating Scale (UHDRS) whose change in motor, cognitive, behavioural and functional capacity total scores were considered as outcome measures. Grafted patients also received morphological and molecular neuroimaging. Results Patients were followed-up from disease onset for a total of 309.3 person-years (minimum 5.3, median 11.2 years, maximum 21.6 years). UHDRS scores have been available since 2004 (median time of 5.7 years since onset, minimum zero, maximum 17.2 years). Median post-transplantation follow-up was 4.3 years, minimum 2.8, maximum 5.1 years. Adjusted post-transplantation motor score deterioration rate was reduced compared to the pretransplantation period, and to that of not-transplanted patients by 0.9 unit/years (95% CI 0.2 to 1.6). Cognitive score deterioration was reduced of 2.7 unit/years (95% CI 0.1 to 5.3). For grafted patients the 2-year post-transplantation [18F]fluorodeoxyglucose positron emission tomography (PET) showed striatal/cortical metabolic increase compared to the presurgical evaluation; 4-year post-transplantation PET values were slightly decreased, but remained higher than preoperatively. [123I]iodobenzamide single photon emission CT demonstrated an increase in striatal D2-receptor density during postgrafting follow-up. Conclusions Grafted patients experienced a milder clinical course with less pronounced motor/cognitive decline and associated brain metabolism improvement. Life-time follow-up may ultimately clarify whether transplantation permanently modifies the natural course of the disease, allowing longer sojourn time at less severe clinical stage, and improvement of overall survival.

Imaging and cognitive reserve studies predict dementia in presymptomatic Alzheimer's disease subjects.

There is strong evidence that Alzheimers disease (AD) pathology starts decades before clinical onset. Cognitive reserve (CR) and brain reserve can be a good predictive model for AD development. Neuroimaging can help in describing cerebral reserves, as well as in detecting AD brain pathology before the onset of clinical dementia. Education and occupation act as proxies for CR and are associated with a lower risk of AD and delayed onset of symptoms. The apolipoprotein E (ApoE)-ε4 allele is a strong risk factor for AD and is associated with lower hippocampal volume even in normal aging. A fluorodeoxyglucose positron emission tomography study of brain metabolism shows different metabolic phenotypes among subjects with different educational levels and ApoE genotypes. More highly educated subjects reach a clinical level when the cerebral areas involved in coping with network disruption are seriously impaired, and the AD-ε4 carriers show more global metabolic brain impairment compared with non-ε4 carriers. Thus, CR can counteract a genetically unfavorable background, suggesting a possible preventive strategy. AD research findings have already produced results, since recent epidemiological studies report a decreasing incidence of AD in the last years.

Effects of gadoxetic acid on quantitative diffusion-weighted imaging of the liver

To prospectively evaluate the effect of gadoxetic acid (Gd‐EOB‐DTPA; Primovist, Bayer‐Schering, Berlin, Germany) on quantitative diffusion‐weighted imaging (DWI) using the Le Bihan IntraVoxel Incoherent Motion model and considering separately the following parameters: slow diffusion coefficient (D), fast diffusion coefficient (D*), perfusion fraction (PF), and apparent diffusion coefficient (ADC).

Predictors of survival in patients with established cirrhosis and hepatocellular carcinoma treated with sorafenib

AIM To investigate in greater detail the efficacy and safety of sorafenib for the treatment of hepatocellular carcinoma (HCC) in patients with established cirrhosis. METHODS From October 2009 to July 2012 patients with an established diagnosis of cirrhosis and HCC treated with sorafenib were consecutively enrolled. According to the Barcelona Clinic Liver Cancer (BCLC) classification, patients were in the advanced stage (BCLC-C) or in the intermediate stage (BCLC-B) but unfit or unresponsive to other therapeutic strategies. Treatment was evaluated performing a 4-phase computed tomography or magnetic resonance imaging scan every 2-3 mo, and analyzed according to the modified Response Evaluation Criteria in Solid Tumors. Sorafenib was administered at 800 mg/d, until radiological progression or occurrence of unacceptable adverse events (AEs). Univariate and multivariate analyses identified predictors of 16-wk clinical benefit and overall survival. RESULTS Forty-four patients were enrolled, 15 had intermediate HCC and 14 a Child-Pugh score of B7. AEs caused treatment interruption in 19 patients (43%), and median treatment duration was shorter in this subset (5 wk vs 19 wk, P < 0.001) and in the BCLC-C subgroup (13 wk vs 40 wk, P = 0.015). No significant differences in the reason for treatment interruption or in treatment duration were found comparing patients in Child-Pugh class A vs B or in patients older or younger than 70 years. After 16 wk of treatment, 18 patients (41%) had stable disease or partial response. Patients with viral infection or BCLC-C were at higher risk of disease progression. ECOG, extrahepatic spread, macrovascular invasion, alpha-fetoprotein or alkaline phosphatase levels at admission were independent predictors of overall survival. CONCLUSION In patients with cirrhosis and HCC treated with sorafenib, AEs are a common cause of early treatment withdrawal. Vascular invasion and extrahepatic spread condition early response to treatment and survival. Baseline biochemical parameters may be helpful to identify patients at higher risk of shorter overall survival.

Liver Lesion Characterization: The Wrong Choice of Contrast Agent Can Mislead the Diagnosis of Hemangioma

AJR 2012; 199:W662 0361–803X/12/1995–W662

Focal Liver Lesions Classification and Characterization: What Value Do DWI and ADC Have?

Objective The aim of this work was to analyze the value of diffusion-weighted imaging (DWI) in the classification/characterization of focal liver lesions (FLLs). Methods Retrospective study, approved by ethical board, of 100 proven FLLs (20 hemangiomas, 20 focal nodular hyperplasia, 20 dysplastic nodules, 20 hepatocellular carcinomas, and 20 metastases) was performed by 1.5-T MR. For each lesion, 2 readers, blinded of medical history, have evaluated 6 sets of images: set A (T1/T2-weighted images), set B (set A + DWI), set C (set B + apparent diffusion coefficient [ADC] map), set D (set A + dynamic and hepatobiliary phases), set E (set D + DWI), set F (set E + ADC map). Results In unenhanced images, the evaluation of the ADC improves the accuracy in classification/characterization (+9%/14%, respectively), whereas in enhanced images the accuracy was increased by DWI (+7%/12%, respectively) and ADC (+13%/19%, respectively). Diffusion-weighted imaging does not improve classification/characterization of hemangiomas, may be useful in focal nodular hyperplasia/dysplastic nodules vs metastases/hepatocellular carcinoma differentiation, and increases the classification/characterization of metastases in both unenhanced and enhanced images. Conclusions Diffusion-weighted imaging may improve classification/characterization of FLLs at unenhanced/enhanced examinations.

Transient Hepatic Parenchymal Enhancement detected at dynamic imaging: A short instruction manual for the clinician

Basic knowledge in the interpretation of hepatic imaging is essential for the clinical hepatologist. In recent years, the availability of dynamic imaging studies of the liver using computed tomography or magnetic resonance has led to appreciate the importance of early changes in arterial perfusion for the interpretation of hepatic lesions. Transient Hepatic Parenchymal Enhancement (THPE) is defined as a normal area of liver parenchyma that enhances after injection of contrast agent during the arterial phase of perfusion. Appearance of this sign is mostly associated with a reduction in portal perfusion or with inflammation, and appears in different morphologic patterns. THPE should not be considered a radiological artefact, and its interpretation is essential to avoid misclassification of hepatic lesions that may have clinical significance, such as hepatocellular carcinoma or hepatic metastases. In this short review we provide essential information on the causes, pathophysiology and morphology of THPE, and discuss the relevance of these findings in a clinical perspective.

Rheolityc thrombectomy in acute myocardial infarction: Effect on microvascular obstruction, infarct size, and left ventricular remodeling

We sought to analyze whether rheolytic thrombectomy (RT) in comparison with manual thrombus aspiration (MTA) may reduce microvascular obstruction (MVO), infarct size (IS), and left ventricular (LV) remodeling in ST‐elevation myocardial infarction (STEMI). Background: Conflicting results have been reported as to whether MTA reduces MVO and IS.

Pancreatic fistula: A proposed percutaneous procedure.

AIM To propose a percutaneous treatment for otherwise intractable pancreatic fistula (PF). METHODS From 2005 to 2011, 12 patients (9 men and 3 women, mean age 59 years, median 63 years, range 33-78 years) underwent radiological treatment for high-output PF associated with peripancreatic fluid collection. The percutaneous procedures were performed after at least 4 wk of unsuccessful conservative treatments. We chose either a one or two step procedure, depending on the size and characteristics of the fistula and the fluid collection (with an arbitrary cut-off of 2 cm). Initially, 2 to 6 pigtail drainages of variable size from 8.3 (8.3-Pig Duan Cook, Bloomington, Indiana, United States) to 14 Fr (Flexima, Boston Scientific, Natick, United States) were positioned inside the collection using a transgastric approach. In a second procedure, after 7-10 d, two or more endoprostheses (cystogastrostomic 8 Fr double-pigtail, Cook, Bloomington, Indiana, United States in 10 patients; covered Niti-S stent, TaeWoong Medical Co, Seoul, South Korea in 2 patients) were placed between the collection and the gastric lumen. In all cases the metal or plastic prostheses were removed within one year after positioning. RESULTS Four out of 12 high-output fistulas fistulas were external while 8/12 were internal. The origin of the fistulous tract was visualised by computer tomography (CT) imaging studies: in 11 patients it was at the body, and in 1 patient at the tail of the pancreas. Single or multiple drainages were positioned under CT guidance. The catheters were left in place for a varying period (0 to 40 d - median 10 and 25(th)-75(th) percentile 0-14). In one case external transgastric drainages were left in place for a prolonged time (40 d) due to the presence of vancomycin-resistant bacteria (Staphylococcus) and fluconazole-resistant fungi (Candida) in the drained fluid. In this latter case systemic and local antibiotic therapy was administered. In both single and two-step techniques, when infection was present, we carried out additional washing with antibiotics to improve the likelihood of the procedures success. In all cases the endoprostheses were left in situ for a few weeks and endoscopically removed after remission of collections, as ascertained by CT scan. Procedural success rate was 100% as the resolution of external PF was achieved in all cases. There were no peri-procedural complications in any of the patients. The minimum follow-up was 18 mo. In two cases the procedure was repeated after 1 year, due to the onset of new fluid collections and the development of pseudocysts. Indeed, this type of endoprosthesis is routinely employed for the treatment of pseudocysts. Endoscopy was adopted both for control of the positioning of the endoprosthesis in the stomach, and for its removal after resolution of the fistula and fluid collection. The resolution of the external fistula was assessed clinically and CT scan was employed to demonstrate the resolution of peripancreatic collections for both the internal and external fistulae. CONCLUSION The percutaneous placement of cistogastrostomic endoprostheses can be used for the treatment of PF that cannot be treated with other procedures.

Cardiac magnetic resonance in patients with mitral valve prolapse: Focus on late gadolinium enhancement and T1 mapping

ObjectivesTo evaluate the incidence of late-gadolinium-enhancement (LGE) in mitral valve prolapse (MVP) (in the absence of other heart/valvular diseases), and its association with the degree of mitral regurgitation (MR) and/or with complex ventricular arrhythmia (ComVA), and to analyse the role of T1 mapping in the evaluation of MVP patients.MethodsWe included all consecutive patients with MVP who underwent during 2015–2016 a comprehensive cardiac magnetic resonance (CMR) examination at 1.5 T. We evaluated the association of LGE with the MR fraction and the presence of ComVA. We compared myocardial T1-native and post-contrast times and extracellular volume (ECV)-values between MVP patients, both with and without LGE, and the control group.ResultsThirty-four patients with MVP were selected (56 ± 14 years old, 59% male). All patients had MR; LGE and ComVA were present in 15 (44%) and 11 (34%) patients, respectively. Significant associations of LGE with both MR severity and ComVA were not found (p=0.72 and 0.79, respectively). T1 mapping confirmed the presence of LGE in all cases. In one patient a thin signal alteration resulted in more evident T1 mapping than LGE. Patients with MVP had higher native T1-values, lower post-contrast T1-values and increased ECV-values compared with controls (p=0.01, 0.01 and 0.00, respectively).ConclusionFocal fibrosis with LGE was found in about half the MVP patients and it was independent of the degree of the valve dysfunction and the presence of ComVA. T1 mapping allows diffuse myocardial wall alterations to be identified, but no significant associations between the MR severity and ComVA and T1/ECV values were found.Key Points• MVP is a common valvulopathy affecting 2–3% of the general population.• MVP has been associated with an increased risk of arrhythmic complications and suddencardiac death.• CMR is a non-invasive imaging method that provides a precise and more accurate assessmentof patients with MVP.