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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 trials

Purpose Methods and Materials Results Conclusion References Personal Information, Purpose: Muscular MRI is regularly used in neuromuscular disorders as a diagnostic and prognostic tool, due to the high resolution of soft-tissue compared to other imaging methods [1]. Quantification of muscular fat content was one of the earliest...

Purpose Methods and Materials Results Conclusion References Personal Information, Purpose: Hematopoietic stem cell transplantation (HSCT) is the standard treatment for high risk leukemia and lymphoma. Acute graft-versus-host disease (aGvHD) is the single most important cause for morbidity and mortality post HSCT. AGvHD is caused by donor...

Purpose Methods and Materials Results Conclusion References Personal Information, Purpose: Nerve injuries affect several hundred thousand people each year in Europe with serious economic implications for both the individual patient and society. Despite limited improvements in surgical technique, traumatic nerve injuries represent a major...

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.