Demyelinating neuropathy as the initial presentation of familial E200K Creutzfeldt-Jakob disease in two patients.

Objective: To describe peripheral neuropathy associated with familial Creutzfeldt-Jakob disease.

Methods: We report two unrelated patients with genetic Creutzfeldt-Jakob disease with demyelinating peripheral neuropathy as initial presentation, with a comprehensive clinical, electrophysiological and neuropathological description.

Results: Both patients exhibited gait disturbance and paresthesia.

The use of differing verb types in the oral narratives of school-age children.

The aim of this project was to examine age-related use of action, metacognitive, and metalinguistic verbs because factors related to verb transparency are thought to impact word learning. Performance on fictional, oral narratives elicited using a single-episode picture was evaluated for 84 children with typical language (TL) and 38 age-matched children with a Developmental Language Disorder (DLD) ranging in age from 5;1 to 14;4 years of age. Narrative samples were transcribed and coded for occurrences of action verbs (AV), metacognitive verbs (MCV), and metalinguistic verbs (MLV).

Competitive adsorption mechanisms of Cd(II), Cu(II) and Pb(II) on bioinspired mesoporous silica revealed by complementary adsorption/isothermal titration calorimetry studies.

This study presents the adsorption properties of a bioinspired grafted mesoporous silica material and the competitive effects between Cd(II) or Cu(II) and Pb(II) during the adsorption process. Glutathione, a natural antioxidant known for its metal binding properties, has been successfully grafted to SBA-15 mesoporous silica and the optimum adsorption parameters were determined. This original and multidisciplinary approach combines classical adsorption studies with thermodynamic investigations to understand the adsorption behavior of Cd(II), Cu(II) and Pb(II) on this material.

Prospective 25-year surveillance of prion diseases in France, 1992 to 2016: a slow waning of epidemics and an increase in observed sporadic forms.

BackgroundPrion diseases are rare, fatal disorders that have repeatedly raised public health concerns since the early 1990s. An active prion disease surveillance network providing national level data was implemented in France in 1992.AimWe aimed to describe the epidemiology of sporadic, genetic and infectious forms of prion diseases in France since surveillance implementation.