Amyotrophic Lateral Sclerosis (ALS) is associated in about half of the cases with behavioral and cognitive disorders, including impairments in socio-emotional processing, considered as key-features for the diagnosis of the behavioral variant of frontotemporal dementia (bv-FTD). The neurostructural bases of emotional deficits in ALS, however, still remain largely unexplored. Here we aim to asses...

In situ transmission electron microscopy (TEM) has been extensively applied to study radiation effects in a wide variety of materials, such as metals, ceramics and semiconductors and is an indispensable tool in obtaining a fundamental understanding of energetic beam-matter interactions, damage events, and materials' behavior under intense radiation environments. In this article, in situ TEM obs...

OBJECTIVE To investigate grey (GM) and white matter (WM) abnormalities and their effects on cognitive and behavioral deficits in a large, phenotypically and genotypically well-characterized cohort of classic adult (aDM1, age at onset ≥ 20 years) or juvenile (jDM1, age at onset <20 years) patients with myotonic dystrophy type 1 (DM1). METHODS A case-control study including 51 DM1 patients (17 ...

Materials designed for nuclear reactors undergo microstructural changes resulting from a combination of several environmental factors, including neutron irradiation damage, gas accumulation and elevated temperatures. Typical ion beam irradiation experiments designed for simulating a neutron irradiation environment involve irradiating the sample with a single ion beam and subsequent characteriza...