Unimpaired Neuropsychological Performance and Enhanced Memory Recall in Patients with Sbma: A Large Sample Comparative Study.

Peculiar cognitive profile of patients with SBMA has been described by fragmented literature. Our retrospective study reports the neuropsychological evaluations of a large cohort of patients in order to contribute towards the understanding of this field. We consider 64 neuropsychological evaluations assessing mnesic, linguistic and executive functions collected from 2013 to 2015 in patients attending at Motor Neuron Disease Centre of University of Padova. The battery consisted in: Digit Span forwards and backwards, Prose Memory test, Phonemic Verbal fluency and Trail making tests. ANCOVA statistics were employed to compare tests scores results with those obtained from a sample of healthy control subjects. Multiple linear regressions were used to study the effect on cognitive performance of CAG-repeat expansion, the degree of androgen insensitivity and their interaction to cognitive performance. Statistical analyses did not reveal altered scores in any neuropsychological tests among those adopted. Interestingly, patients performed significantly better in the Prose Memory test's score. No relevant associations were found with genetic, hormonal or clinical patients' profile. Results inconsistent with previous studies have been interpreted according to the phenomenon of somatic mosaicism. We suggest a testosterone-related and the mood state-dependant perspectives as two possible interpretations of the enhanced performances in the Prose Memory test. Further studies employing more datailed tests batteries are encouraged

New Insights on the high temperature nanostructure evolution of SiOC and B-doped SiBOC Polymer-derived Glasses

SiOC and SiBOC polymer-derived glasses show a complex nanostructure in which nanocrystalline -SiC and sp2 C coexists with nanoclusters of amorphous SiO2 or SiO2-B2O3 and with mixed silicon oxycarbide and boron oxycarbide units. The characterization of the nanostructure is performed with a multiple technique approach on SiOC and SiBOC glasses before and after HF etching. The acid attack dissolves the silica-based nanoclusters and allows indirect information on their size and amount to be obtained. By increasing the pyrolysis temperature from 1200 up to 1500 \ub0C the oxide clusters grow in size and amount in both SiOC and SiBOC glasses. Compared to the B-free SiOC glass, SiBOC is more easily etched and develops higher porosity and larger pore size. Boron has also an important effect on the ordering of the sp2 C phase: it leads to thicker sp2 C nanocrystals. This effect is remarkable because it starts at low temperature (1500 \ub0C) compared to the usual temperature, 1800 \ub0C, reported in the literature for different forms of nanocrystalline carbon. Raman analysis of SiBOC glasses pyrolyzed at 1500 \ub0C clearly shows the presence of the D\u2032 band at 1617 cm-1. From this experimental result it is postulated that B enters into the graphene layers forming BC3 units. Finally, the shift toward lower wavenumbers of the Raman spectra recorded on SiOC and SiBOC glasses after etching seems to reveal a high compressive stress acting along the basal sp2 C planes of the graphite nanocrystals

Skeletal muscle satellite cells in amyotrophic lateral sclerosis

OBJECTIVES: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease involving progressive muscular paralysis reflecting degeneration of motor neurons. Skeletal muscle tissue seems to play a significant role in ALS pathogenesis. Here, the role of satellite cells (SCs) in ALS muscle atrophy is investigated. METHODS: We isolated SCs from ALS human muscle biopsies and we analyzed their ability to grow and expand in vitro. Ultrastructural and immunophenotypical features were analyzed. Quantitative real-time RT-QPCR and western blot (WB) analyses were performed to evaluate MRFs and MyH1 expression. RESULTS: ALS SCs showed a high proliferative potential, but their capacity to proceed through the myogenic program and form myotubes seems altered compared to controls (Ctrls). We observed that differentiating ALS SCs showed some specific features, but they displayed an altered morphology, with a large number of vacuoles. RT-QPCR and WB showed lower Myf-4 and MyH1 compared to Ctrls. CONCLUSIONS: Our data suggest that the capacity of ALS SCs to proceed through the myogenic program seems to be altered: SCs seem to lose their ability to regenerate and restore mature myofiber

Synthesis and characterization of photo luminescent Si-nanocrystals embedded in SiO2 using polymer pyrolysis

Polymer pyrolysis to nanoceramics is employed for the production of photo-luminescent thin film samples, consisting of Si-nanocrystals dispersed in silica matrix. Thin films were deposited on Si wafer by spin-coating technique from a triethoxysilane-derived sol\u2013gel solution. This is a fast process and a low cost technique. Samples were annealed at different temperatures between 600 \ub0C and 1300 \ub0C, under N2 gas atmosphere. Structural evolution and luminescence from Si-ncs vs. temperature were investigated by FT-IR absorption spectroscopy and photo-luminescence. Absorption spectrum of As-Prep sample shows several vibrational bands due to Si\u2013H, C\u2013Hx, and Si\u2013O\u2013Si structural units, without any appreciable luminescence. Silica phase segregation and intense PL (790 nm) due to Si-ncs were observed on annealing at TN1000 \ub0C. \ua9 2007 Elsevier B.V. All rights reserve