Quantitative MRI can detect subclinical disease progression in muscular dystrophy

Oculopharyngeal muscular dystrophy (OPMD) is a rare autosomal dominant muscular dystrophy with late onset and slow progression. The aim of this study was to compare different methods of quantitative MRI in the follow-up of OPMD to semiquantitative evaluation of MRI images and to functional parameters. We examined 8patients with genetically confirmed OPMD and 5healthy volunteers twice at an interval of 13months. Motor function measurements (MFM) were assessed. Imaging at 1.5T (Siemens Magnetom Avanto) comprised two axial slice groups at the largest diameter of thigh and calf and included T1w TSE, 2-point Dixon for muscular fat fraction (MFF) and a multi-contrast TSE sequence to calculate quantitative T2 values. T1 images were analyzed using Fischer's semiquantitative 5-point (0-4) scale. MFM and visual scores showed no significant difference over the study period. Overall T2 values increased in patients over the study period from 49.4 to 51.6ms, MFF increased from 19.2 to 20.7%. Neither T2 values nor MFF increased in controls. Changes in T2 correlated with the time interval between examinations (r 2=0.42). In this small pilot trial, it was shown that quantitative muscle MRI can detect subclinical changes in patients with OPMD. Quantitative MRI might, therefore, be a useful tool for monitoring disease progression in future therapeutic trial

Muscular involvement assessed by MRI correlates to motor function measurement values in oculopharyngeal muscular dystrophy

Oculopharyngeal muscular dystrophy (OPMD) is a progressive skeletal muscle dystrophy characterized by ptosis, dysphagia, and upper and lower extremity weakness. We examined eight genetically confirmed OPMD patients to detect a MRI pattern and correlate muscle involvement, with validated clinical evaluation methods. Physical assessment was performed using the Motor Function Measurement (MFM) scale. We imaged the lower extremities on a 1.5T scanner. Fatty replacement was graded on a 4-point visual scale. We found prominent affection of the adductor and hamstring muscles in the thigh, and soleus and gastrocnemius muscles in the lower leg. The MFM assessment showed relative mild clinical impairment, mostly affecting standing and transfers, while distal motor capacity was hardly affected. We observed a high (negative) correlation between the validated clinical scores and our visual imaging scores suggesting that quantitative and more objective muscle MRI might serve as outcome measure for clinical trials in muscular dystrophie

Quantitative MRI and loss of free ambulation in Duchenne muscular dystrophy

The purpose of this ethics approved trial was to correlate quantitative MRI with functional abilities in both ambulant and non-ambulant Duchenne muscular dystrophy (DMD). Twenty patients with genetically confirmed DMD were recruited. Physical assessment was performed using the motor function measurement (MFM) scale. Axial 3T MRI scans of the thighs were acquired using T1-weighted in- and opposed-phase images (TR=20ms, TE1=2.45ms, TE2=3.68ms, flip angle=15°) to calculate the relative fat fraction according to the two-point Dixon method in the knee extensors, flexors, and adductor muscles. The average MFM was 65.3% and correlated negatively to age (r 2=0.60). Overall mean fat fraction correlated positively to age (r 2=0.51-0.64). An average of 5% increase in mean fat fraction per year was calculated. Mean fat fraction of the quadriceps showed a high negative correlation (r 2=0.93) to the D1 (standing position and transfers) component of the MFM. A cutoff for mean fat fraction of 50% predicted loss of ambulation with a sensitivity of 100% and a specificity of 91%. Therefore, quantitative muscle MRI seems to be a promising endpoint for short clinical trials evaluating the effect of newer treatments on the time of loss of ambulation in DM

Blood vessels of Glioblastoma multiforme : Electron-microscopic and immuno-cytochemic investigation of changes in perivascular extracellular matrix and endothelial cells

Ziel dieser Studie war die Untersuchung von Veränderungen der Gefäße des Glioblastoma multiforme, die in Zusammenhang mit dem Zusammenbruch der Blut-Hirn-Schranke stehen. Bei 15 Patienten mit einem Glioblastom, sowie bei 2 Kontrollpatienten mit anderer Pathologie wurden Gewebeproben aus dem Tumor sowie dem peritumoralen Gewebe entnommen und mittels Elektronenmikroskopie sowie immunfloureszenzmikroskopisch auf Kollagene, Fibronektin, Laminin, Tenascin, Agrin, MMP-2, MMP-9 und den Blut-Hirn-Schranken Marker Glut-1 untersucht. Die Zahl der endothelialen Vesikel war deutlich erhöht. Auch fanden sich Lücken in der Endothelschicht. Dagegen fanden sich keine Fenestrae in den Endothelzellen. Die Markierungen für Agrin um die Tumorgefäße waren erniedrigt, diejenigen für Tenascin um die Tumorgefäße deutlich vermehrt - letzteres vor allem in Zonen fokaler Nekrose am ehesten aufgrund erhöhter Expression von VEGF und PDGF-ß. Dabei fand sich eine deutliche Korrelation der Tenascin-Expression zu derjenigen für MMP-9, welches durch dieselben Wachstumsfaktoren induziert wird. Die Markierungen für Agrin und Tenascin verhielten sich gegenläufig zueinander. Wenn beide in der Extrazellularmatrix nachweisbar waren, fand sich eine deutliche räumliche Separation, mit Tenascin in den tumoralen Anteilen und Agrin in den endothelialen Anteilen der Basalmembran. Die essentiellen Bestandteile der Basalmembran- Fibronektin, Kollagen IV und Laminin- waren in allen Gefäßen nachweisen, zusätzlich fand sich eine deutliche Markierung für Kollagen I. Glut-1 war in zahlreichen Gefäßen innerhalb der Tumoren nachzuweisen, was für eine intakte Blut-Hirn-Schranke dieser Gefäße spricht. Dabei waren diese Gefäße nicht für MMP-9 positiv, während MMP-9 positive Gefäße keine Markierung für Glut-1 aufwiesen. Insgesamt zeigten sich deutliche Veränderungen der Extrazellularmatrix pathologischer Gefäße in Glioblastomen, wobei eine Korrelation zum Zusammenbruch der Blut-Hirn-Schranke besteht.The aim of this study was the investigation of changes of the vessels of Glioblastoma multiforme in relation to the break-down of the blood-brain-barrier. In 15 patients with Glioblastoma multiforme and two control-subjects with different pathology, specimen of the tumor and peritumoral tissue were removed during surgery. The specimen were examined with electron-microscopy as well as with immuno-flourescence-microscopy for collagene, fibronectin, laminin, tenascin, agrin, MMP-2, MMP-9 and the blood-brain-barrier marker Glut-1. The number of endothelial vesicles was increased. While there were gaps in the endothelial layer, there were no fenestrations of endothelial cells. Around tumor vessels labeling for agrin was decreased, labeling for tenascin was increased compared to control tissue, the latter especially in areas of focal necrosis, probably due to higher expression of VEGF and PDGF-ß. There was an obvious correlation of tenacin to MMP-9 labeling, witch can be explained by both proteins being induced by the same growth factors. Labeling for agrin and tenascin was exclusive. If both were present in the extracellular matrix of one vessel, there was a clear spatial separation, with tenascin in the tumoral part and agrin in the endothelial part of the basal lamina. The essential parts of the basal lamina- fibronectin, collagene IV and laminin- were detected around all vessels. In addition there was strong labeling for collagene I. Glut-1 was visible in multiple tumoral vessels, indicating an intact blood-brain-barrier. These vessels were negative for MMP-9, while MMP-9 positive vessels showed no labeling for Glut-1. In conclusion there were clearly visible changes in the extracellular matrix of pathologic vessels in glioblasoma multiforme, with a correlation to the break-down of the blood-brain-barrier

Intra-arterial catheter guided steroid administration for the treatment of steroid-refractory intestinal GvHD

Acute gastrointestinal GvHD (GI-aGvHD) refractory to first-line treatment with systemic corticosteroids is resulting in death in the majority of patients. We prospectively assessed the feasibility and efficacy of regional intra-arterial steroid treatment in adult patients with severe (≥ grade III) GI-aGvHD not responding to first-line treatment.; Patients with more than +++ GI-aGvHD not responding to intravenous methylprednisolone at a dose of 2mg/kg/day were eligible for inclusion. Catheter guided intra-arterial steroid administration (IASA) was performed into the superior and inferior mesenteric artery.; 12 consecutive patients with steroid-refractory grade III GI-aGvHD received IASA as second-line treatment. 83% of patients had gastrointestinal response including four patients (33%) with complete response at 28 days after IASA. 5/12 patients were alive at a median time of 531 days.; Regional treatment of severe GVHD with IASA treatment seems to be a safe and effective second-line treatment for steroid-refractory GI-aGvHD in adult patients

Quantification of fat infiltration in oculopharyngeal muscular dystrophy : comparison of three MR imaging methods

To analyze and compare three quantitative MRI methods to determine the degree of muscle involvement in oculopharyngeal muscular dystrophy (OPMD)

"I was seen by a radiologist, but unfortunately I can’t remember the name and I still have questions. What should I do?" : Radiologists should give thoughts to improve service professionalism and patient esteem

BackgroundThe aim of the study is to investigate how well patients remember the radiologist’s name after a radiological examination, and whether giving the patient a business card improves the patient’s perception of the radiologist’s professionalism and esteem.MethodsIn this prospective and randomized two-centre study, a total of 141 patients with BI-RADS 1 and 2 scores were included. After screening examination comprising mammography and ultrasound by a radiologist, 71 patients received a business card (group 1), while 70 received no business card (group 2). Following the examination, patients were questioned about their experiences.ResultsThe patients in group 1 could remember the name of the radiologist in 85% of cases. The patients in group 2, in contrast, could only remember the name in 7% of cases (p All questions were analysed with a Cochran-Mantel-Haenszel (CMH) test that took study centre as stratification into account. In some cases, two categories were collapsed to avoid zero cell counts.ConclusionsUsing business cards significantly increased the recall of the radiologist’s name and could be an important tool in improving the relationships between patients and radiologists and enhancing service professionalism.Trial registrationWe have a general approval from our ethics committee. The patients have given their consent to this study.publishe