ABSTRACT. Objectives. To further define the clinical spectrum of the disease for pediatric and metabolic spe cialists, and to suggest that the general pediatrician and pediatric neurologist consider succinic semialdehyde de hydrogenase (SSADH) deficiency in the differential di agnosis of patients with (idiopathic) mental retardation and emphasize the need for accurate, quantitative or ganic acid analysis in such patients.Patients. The clinical features of 23 patients (20 fam ilies) with SSADH deficiency (4-hydroxybutyric acid uria) are presented. The age at diagnosis ranged from 3
Phases of matter are ubiquitous with everyday examples including solids and liquids. In reduced dimensions, particular phases, such as the 2D hexatic phase and corresponding phase transitions occur. A particularly exciting example of 2D ordered systems are skyrmion lattices, where in contrast to previously studied 2D colloid systems, the skyrmion size and density can be tuned by temperature and magnetic fields. This allows for the system to be driven from a liquid phase to the onset of a hexatic phase as deduced from the analysis of the hexagonal order. Using coarse-grained molecular dynamics simulations of soft disks, the skyrmion interaction potentials are determined, and it is found that the simulations are able to reproduce the phase behavior. This shows that not only the static behavior of skyrmions is qualitatively well described in terms of a simple 2D model system but skyrmion lattices are versatile and tunable 2D model systems that allow for studying phases and phase transitions in reduced dimensions.
The syndrome of ichthyosis follicularis, alopecia, and photophobia (IFAP) is an uncommon neuroichthyosis described in only 10 males so far. We report on a man with congenital ichthyosis and alopecia with apparently normal development in early infancy. Photophobia and generalized myoclonicastatic seizures began during or after the first year of age and were associated with progressive impairment of motor skills and mental abilities. He died at 33 years of age. Neuropathological findings showed an unusual deformation of the temporal lobes and olivocerebellar atrophy. Cytogenetic and molecular studies did not uncover deletions in either Xp22.2 to 3 or in Xq27.3 to qter.
Reservoir computing (RC) has been considered as one of the key computational principles beyond von-Neumann computing. Magnetic skyrmions, topological particle-like spin textures in magnetic films are particularly promising for implementing RC, since they respond strongly nonlinearly to external stimuli and feature inherent multiscale dynamics. However, despite several theoretical proposals that exist for skyrmion reservoir computing, experimental realizations have been elusive until now. Here, we propose and experimentally demonstrate a conceptually new approach to skyrmion RC that leverages the thermally activated diffusive motion of skyrmions. By confining the electrically gated and thermal skyrmion motion, we find that already a single skyrmion in a confined geometry suffices to realize nonlinearly separable functions, which we demonstrate for the XOR gate along with all other Boolean logic gate operations. Besides this universality, the reservoir computing concept ensures low training costs and ultra-low power operation with current densities orders of magnitude smaller than those used in existing spintronic reservoir computing demonstrations. Our proposed concept is robust against device imperfections and can be readily extended by linking multiple confined geometries and/or by including more skyrmions in the reservoir, suggesting high potential for scalable and low-energy reservoir computing.
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