TY - JOUR A1 - Porubsky, Stefan A1 - Popovic, Zoran V. A1 - Badve, Sunil A1 - Banz, Yara A1 - Berezowska, Sabina A1 - Borchert, Dietmar A1 - Brüggemann, Monika A1 - Gaiser, Timo A1 - Graeter, Thomas A1 - Hollaus, Peter A1 - Huettl, Katrin S. A1 - Kotrova, Michaela A1 - Kreft, Andreas A1 - Kugler, Christian A1 - Lötscher, Fabian A1 - Möller, Burkhard A1 - Ott, German A1 - Preissler, Gerhard A1 - Roessner, Eric A1 - Rosenwald, Andreas A1 - Ströbel, Philipp A1 - Marx, Alexander T1 - Thymic hyperplasia with lymphoepithelial sialadenitis (LESA)-like features: strong association with lymphomas and non-myasthenic autoimmune diseases JF - Cancers N2 - Thymic hyperplasia (TH) with lymphoepithelial sialadenitis (LESA)-like features (LESA-like TH) has been described as a tumor-like, benign proliferation of thymic epithelial cells and lymphoid follicles. We aimed to determine the frequency of lymphoma and autoimmunity in LESA-like TH and performed retrospective analysis of cases with LESA-like TH and/or thymic MALT-lymphoma. Among 36 patients (21 males) with LESA-like TH (age 52 years, 32–80; lesion diameter 7.0 cm, 1–14.5; median, range), five (14%) showed associated lymphomas, including four (11%) thymic MALT lymphomas and one (3%) diffuse large B-cell lymphoma. One additional case showed a clonal B-cell-receptor rearrangement without evidence of lymphoma. Twelve (33%) patients (7 women) suffered from partially overlapping autoimmune diseases: systemic lupus erythematosus (n = 4, 11%), rheumatoid arthritis (n = 3, 8%), myasthenia gravis (n = 2, 6%), asthma (n = 2, 6%), scleroderma, Sjögren syndrome, pure red cell aplasia, Grave’s disease and anti-IgLON5 syndrome (each n = 1, 3%). Among 11 primary thymic MALT lymphomas, remnants of LESA-like TH were found in two cases (18%). In summary, LESA-like TH shows a striking association with autoimmunity and predisposes to lymphomas. Thus, a hematologic and rheumatologic workup should become standard in patients diagnosed with LESA-like TH. Radiologists and clinicians should be aware of LESA-like TH as a differential diagnosis for mediastinal mass lesions in patients with autoimmune diseases. KW - autoimmune disease KW - imaging KW - LESA KW - lymphoma KW - myasthenia KW - pathology KW - surgery KW - thymus KW - thymic epithelial tumor KW - thymitis Y1 - 2021 U6 - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-223049 SN - 2072-6694 VL - 13 IS - 2 ER - TY - JOUR A1 - Rottlaender, Andrea A1 - Kuerten, Stefanie T1 - Stepchild or prodigy? Neuroprotection in multiple sclerosis (MS) research JF - International Journal of Molecular Sciences N2 - Multiple sclerosis (MS) is an autoimmune disorder of the central nervous system (CNS) and characterized by the infiltration of immune cells, demyelination and axonal loss. Loss of axons and nerve fiber pathology are widely accepted as correlates of neurological disability. Hence, it is surprising that the development of neuroprotective therapies has been neglected for a long time. A reason for this could be the diversity of the underlying mechanisms, complex changes in nerve fiber pathology and the absence of biomarkers and tools to quantify neuroregenerative processes. Present therapeutic strategies are aimed at modulating or suppressing the immune response, but do not primarily attenuate axonal pathology. Yet, target-oriented neuroprotective strategies are essential for the treatment of MS, especially as severe damage of nerve fibers mostly occurs in the course of disease progression and cannot be impeded by immune modulatory drugs. This review shall depict the need for neuroprotective strategies and elucidate difficulties and opportunities. KW - experimental autoimmune encephalomyelitis KW - white matter KW - lesions KW - remyelination KW - multiple sclerosis KW - regeneration KW - neuroprotection KW - degeneration KW - axonal damage KW - neurodegeneration KW - pathology KW - sodium channels KW - axonal injury KW - central nervous system Y1 - 2015 U6 - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-148416 VL - 16 ER - TY - THES A1 - Fischer, Monika T1 - Nicht operierte Sagittalnahtsynostosen im Verlauf T1 - Radiographic Changes in Non-Operated Sagittal Suture Synostosis N2 - Zur Darstellung des Verlaufs bei nicht operierten Kindern mit Sagittalsynostose wurden 155 Röntgenbilder von 52 Patienten im Alter zwischen 15 Tagen und 9 Jahren untersucht. Die Schädelnähte wurden hinsichtlich der Darstellbarkeit, Begrenzung, Zähnelung und Aktivität beurteilt. Weiterhin wurden acht Strecken und vier Winkel gemessen, daraus zwei Indizes berechnet. Die Sagittalnaht war bei mehr als der Hälfte der Aufnahmen im ersten Lebensjahr partiell bzw. vollständig darstellbar. Die Lambdanaht war ab dem zweiten Lebensmonat immer, die Coronarnaht bis auf wenige Ausnahmen darstellbar. Die Zähnelung der Nähte entwickelte sich altersentsprechend. Der Anteil der Nähte, die keine erhöhte Aktivität aufwiesen, sank im Verlauf von 94% auf 38%. Bei den Messstrecken und Winkeln wurden die Ergebnisse aus der Literatur weitgehend bestätigt. Der Basiswinkel war im untersuchten Patientenkollektiv signifikant erhöht. Der Höhenindex näherte sich im Verlauf der Altersnorm an, wohingegen sich die Parameter innere Schädelbreite und Breiten-Längen-Index signifikant von der Altersnorm entfernten. Der Skaphozephalus wächst sich nicht aus, aber einzelne Merkmale, wie die parietale Wölbung,nähern sich wieder etwas der Norm an. Sichere Hinweise für ein Übergreifen der Synostose auf andere Nähte wurden nicht gefunden. Im weiteren wurden digitale und konventionelle Röntgenaufnahmen von 33 Patienten mit Kraniostenosen verglichen. Untersucht wurde die Beurteilbarkeit hinsichtlich Schärfe und Kontrast. Der Zeitabstand zwischen konventioneller und digitaler Röntgenaufnahme lag im Mittel bei 24 Monaten. Die Vorteile des digitalen Röntgens hinsichtlich der Beurteilbarkeit konnten deutlich gezeigt werden. Somit ist das optimierte digitale Röntgensystem dem konventionellen vorzuziehen. N2 - Purpose: To characterize the spontaneous clinical course of isolated sagittal synostosis based on planar skull radiography. Materials and Methods: In this retrospective analysis we evaluated a total of 155 radiographs of 55 children 2 weeks to 9 years old. The sagittal, coronal and lambdoid sutures were evaluated on the basis of pairs of ap and lateral radiographs. The sutures were examined with respect to their boundary, activity, and conspicuity to be visualized (based on a 3-grade score system). Six selected points on the skull X-ray defined eight measured distances, three angles, and a width-length index. To document changes over time, the measurements were correlated to normal values. In addition, a correlation between suture activity and selected parameters was evaluated. Results: The sagittal suture could be continuously or partially depicted in more then half of all radiographs taken during the first year of life. The measured distances and angles were concordant with results from the literature. With increasing age, the width-length index deviated from standard values while other parameters approximated the norm. Conclusion: In the case of children younger than twelve months, the sagittal suture appears radiologically open in many cases despite clear-cut scaphocephaly. Definite signs of progressive plurisutural fusion were not found in this series. The dolichocephalic deformity remained unchanged while some signs of scaphocephalic appearance actually improved. Key words KW - Kraniostenose KW - Konservative Therapie KW - Radiologie KW - Vergleich konservative digitale Röntgentechnik KW - pathology KW - skull KW - radiography Y1 - 2007 U6 - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-24310 ER -