A Case Report of Sandhoff Disease

Sandhoff disease is a rare and severe lysosomal storage disorder representing 7% of GM2 gangliosidoses. Bilateral thalamic involvement has been suggested as a diagnostic marker of Sandhoff disease. A case of an 18-month-old infant admitted for psychomotor regression and drug resistant myoclonic epilepsy is presented. Cerebral CT scan showed bilateral and symmetrical thalamic hyperdensity. MRI revealed that the thalamus was hyperintense on T1-weighted images and hypointense on T2-weighted images with a hypersignal T2 of the white matter. Enzymatic assays objectified a deficiency of both hexosaminidases A and B confirming the diagnosis of Sandhoff disease

Interaction of a flame front with its self-generated flow in an enclosure: The “tulip flame” phenomenon

International audienc

Interaction of a flame front with its self-generated flow in an enclosure: The “tulip flame” phenomenon

International audienc

Characterization and modelling of spring-in effect on Z-shape composite part

International audienceIn this study, a simple finite-element model is developed to predict the spring-in of Z-section composite parts made of carbon fiber/ epoxy resin manufactured with resin transfer molding process. The different mechanisms occurring during manufacturing and effective in the formation of residual stresses and shape distortions are implemented. They are mainly the thermal effect and the chemical shrinkage of the resin during the cross-linking polymerization reaction. A dedicated experimental procedure allowing separation of strains to cure shrinkage and thermal expansion is presented to characterize the resin cure shrinkage. Cure kinetic properties of the resin are determined with differential scanning calorimetry measurements. The spring-in angles predicted by the finite element analysis are compared to the angles measured on the Z-section specimens of various thicknesses