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- Cousyn, Louis, et al.
(författare)
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Brain MRI features and scoring of leukodystrophy in adult-onset Krabbe disease
- 2019
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Ingår i: Neurology. - : Lippincott Williams & Wilkins. - 0028-3878 .- 1526-632X. ; 93:7, s. E647-E652
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Tidskriftsartikel (refereegranskat)abstract
- OBJECTIVE: To perform a systematic analysis and scoring of brain MRI white matter hyperintensities (WMH) in adult-onset Krabbe disease.METHODS: We retrospectively collected basic clinical data and the first available brain MRI from patients with confirmed Krabbe disease with first clinical manifestations beyond 10 years of age. Data were obtained from our reference center for lysosomal diseases (n = 6) and from contacted authors of published articles describing patients with adult-onset Krabbe disease (n = 15). T2-weighted fluid-attenuated inversion recovery images of each patient were analyzed and scored using a radiologic score of WMH in a single center.RESULTS: The corticospinal tract was always affected by WMH (100% of patients), however, with some distinctions along the tract: the precentral gyrus (100%), corona radiata (95%), and posterior internal capsule (81%) were highly abnormal, whereas the mesencephalon (57%), pons (52%), and medulla oblongata (5%) were less affected. WMH were also frequently present in the posterior lateral periventricular white matter (95%), optic radiations (86%), postcentral gyrus (71%), medial lemniscus (62%), and corpus callosum, especially in the isthmus (71%), whereas the genu was always normal. A few patients did not have the classical MRI pattern but extensive hyperintensities (n = 3), or patchy distribution of hyperintensities mimicking an acquired etiology (n = 2), or very subtle hyperintensities of the corticospinal tract (n = 1).CONCLUSIONS: We specified the main locations of WMH, which were observed in the earliest stages of the disease and were also present in patients with atypical MRI pattern, highlighting the importance of radiologic features to guide the diagnosis.
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| 3. |
- Jastrzebski, Miroslaw, et al.
(författare)
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Geochronology, petrogenesis and geodynamic significance of the Visean igneous rocks in the Central Sudetes, northeastern Bohemian Massif
- 2018
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Ingår i: Lithos. - : Elsevier BV. - 0024-4937 .- 1872-6143. ; 316-317, s. 385-405
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Tidskriftsartikel (refereegranskat)abstract
- New sensitive high-resolution ion microprobe (SHRIMP) UPb zircon geochronologic data, whole-rock geochemical and Sr-Nd-Pb isotopic data, and zircon and quartz Ύ18O isotopic data from the Staré Město granitoids (SMG), Jawornik granitoids (JG), and Kłodzko-Złoty Stok granitoids (KZSG) and associated mafic and ultramafic rocks are examined. This study provides new insights into the processes of magma generation, transport and emplacement during the Variscan development of the contact zone of the Saxothuringia, Teplá-Barrandia and Brunovistulia in the Central European portion of the Variscan belt. The results of this study, combined with existing geochemical and isotopic data, imply that the parental magmas of these intrusions share a close affinity and suggest that these intrusions formed in a subduction-related tectonic setting. The SMG, JG and KZSG magmas represent hybrids that formed from the contamination of partial melts from the lower crust and/or subducted sediments with various proportions of enriched mantle-derived melts. These mainly sheeted plutons intruded along the main geological boundaries during the Visean and recorded the vertical and lateral displacements between the major tectono-stratigraphic units (microplates) in the NE Bohemian Massif. In the Central Sudetes, these granitoid magmas were first emplaced along the northern continuation of the Moldanubian Zone. The SHRIMP UPb dating of zircons indicates that the SMG intruded the reactivated suture zone between the Brunovistulia and Saxothuringia at 344–341 Ma. Sills of the JG were emplaced between c. 347 and c. 334 Ma. The composite Kłodzko-Złoty Stok Pluton, which includes the KZSG and accompanying mafic enclaves and pyroxenite and lamprophyre dykes, was mainly emplaced at 340–333 Ma. The magmas of the KZSG possibly facilitated the final amalgamation of the Sudetic counterparts of the Teplá-Barrandia and Saxothuringia microplates during the orogenic uplift of the latter. Petrologic and oxygen isotopic data further indicate partial post-magmatic hydrothermal and/or alteration processes. Our new data further stress the connection of magma transfer and active shear zones, which could manifest as crustal-scale magma-ascent conduits.
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