Waste Combustion Releases Anthropogenic Nanomaterials in Indigenous Arctic Communities.

Arctic autochthonous communities and the environment face unprecedented challenges due to climate change and anthropogenic activities. One less-explored aspect of these challenges is the release and distribution of anthropogenic nanomaterials in autochthonous communities. This study pioneers a comprehensive investigation into the nature and dispersion of anthropogenic nanomaterials within Arctic Autochthonous communities, originating from their traditional waste-burning practices.

The expanding clinical and genetic spectrum of DYNC1H1-related disorders.

Intracellular trafficking involves an intricate machinery of motor complexes including the dynein complex to shuttle cargo for autophagolysosomal degradation. Deficiency in dynein axonemal chains as well as cytoplasmic light and intermediate chains have been linked with ciliary dyskinesia and skeletal dysplasia. The cytoplasmic dynein 1 heavy chain protein (DYNC1H1) serves as a core complex for retrograde trafficking in neuronal axons.

West Syndrome Is an Exceptional Presentation of Pyridoxine- and Pyridoxal Phosphate-Dependent Epilepsy: Data From a French Cohort and Review of the Literature.

To characterize the electro-clinical presentation of patients with pyridoxine-dependent epilepsy (PDE) and pyridoxal phosphate (PLP)-dependent epilepsy in order to determine whether some of them could be diagnosed as West syndrome, i. e., West syndrome that starts after the age of 2 months without other types of seizures (focal seizures for instance) before the onset of epileptic spasms.

Real-life use of videos in pediatric epilepsy consultations.

Paroxysmal events are usually not directly observed by physicians. The diagnosis remains challenging and relies mostly on the description of witnesses. The effectiveness of videos for seizure diagnosis has been validated by several studies, but their place in clinical practice is not yet clear.

KCNT1 epilepsy with migrating focal seizures shows a temporal sequence with poor outcome, high mortality and SUDEP.

Epilepsy of infancy with migrating focal seizures was first described in 1995. Fifteen years later, KCNT1 gene mutations were identified as the major disease-causing gene of this disease. Currently, the data on epilepsy of infancy with migrating focal seizures associated with KCNT1 mutations are heterogeneous and many questions remain unanswered including the prognosis and the long-term outcome especially regarding epilepsy, neurological and developmental status and the presence of microcephaly.

Opsoclonus in a child with neuroborreliosis: Case report and review of the literature.

Opsoclonus consists of massive erratic rapid eye jerks. They may occur in isolation or in association with myoclonus and ataxia, i.e.

Flax stems: from a specific architecture to an instructive model for bioinspired composite structures.

The present paper proposes to carefully study and describe the reinforcement mechanisms within a flax stem, which is an exceptional natural model of composite structure. Thanks to accurate microscopic investigations, with both optical and SEM method, we finely depicted the flax stem architecture, which can be view as a composite structure with an outer protection, a unidirectional ply on the periphery and a porous core; each component has a specific function, such as mechanical reinforcement for the unidirectional ply and the porous core. The significant mechanical role of fibres was underlined, as well as their local organisation in cohesive bundles, obtained because of an intrusive growth and evidenced in this work through nanomechanical AFM measurement and 3D reconstruction.