[Letter to the editor] Sustained effects of a neural-based intervention in a refractory case of Tourette syndrome

There are only a few treatments available for Tourette syndrome (TS). These treatments frequently do notwork in patients with moderate to severe TS [1]. Neuroimaging studies show a correlation between tics severity and increased activation over motor pathways, along with reduced activation over the control areas of the cortico-striato-thalamo-cortical circuits [2]. Moreover, the temporal pattern of tic generation suggests that cortical activation especially in the SMA precedes subcortical activation [3]. Following this assumption, here we explored the brain effects of 10-daily sessions of cathodal transcranial Direct Current Stimulation (tDCS) delivered over the pre-SMA in a patient with refractory and severe TS and also assessed whether those changes were long lasting (up to 6 months).

Inferior frontal gyrus white matter abnormalities in obsessive-compulsive disorder

The aim of the present study is to explore obsessive-compulsive disorder (OCD)-related abnormalities in white matter connectivity in OCD for a core region associated with inhibitory control [i.e. inferior frontal gyrus (IFG)]. Fifteen patients with OCD (11 men) and 15 healthy controls (nine men) underwent diffusion tensor imaging scanning to study four diffusivity indexes of white matter integrity [fractional anisotropy, mean diffusivity (MD), axial diffusivity and radial diffusivity (RD)]. The results showed that persons with OCD manifested significantly lower fractional anisotropy levels in the bilateral IFG as well as its parcellations in the pars opercularis, pars triangularis, and pars orbitalis. Significantly higher levels of MD, RD were evident for the OCD group in the IFG as a whole as well as in the bilateral subregions of the pars triangularis and pars opercularis (for MD and RD), the right side of the pars orbitalis (for RD), and the left side of the pars triangularis and right side pars opercularis (for axial diffusivity). Overall, the results suggest significant alterations in structural connectivity, probably associated with myelination and axonal abnormalities in the IFG of OCD patients.

Study to determine the cardiovascular risk ofthe population of Guimarães/Vizela, includingthe prevalence of arterial stiffness and early vascular aging syndrome

Tese de Doutoramento em Medicina.

Redefining the MED13L syndrome

Congenital cardiac and neurodevelopmental deficits have been recently linked to the mediator complex subunit 13-like protein MED13L, a subunit of the CDK8-associated mediator complex that functions in transcriptional regulation through DNA-binding transcription factors and RNA polymerase II. Heterozygous MED13L variants cause transposition of the great arteries and intellectual disability (ID). Here, we report eight patients with predominantly novel MED13L variants who lack such complex congenital heart malformations. Rather, they depict a syndromic form of ID characterized by facial dysmorphism, ID, speech impairment, motor developmental delay with muscular hypotonia and behavioral difficulties. We thereby define a novel syndrome and significantly broaden the clinical spectrum associated with MED13L variants. A prominent feature of the MED13L neurocognitive presentation is profound language impairment, often in combination with articulatory deficits.

Cotard syndrome without depressive symptoms in a schizophrenic patient

Cotard Syndrome without Depressive Symptoms in a Schizophrenic Patient

Removal of ochratoxin A from contaminated white and red wines using oenological fining agents

Mycotoxins are toxic secondary metabolites produced by certain moulds, being ochratoxin A (OTA) one of the most relevant. Its chemical structure is a dihydro-isocoumarin connected at the 7-carboxy group to a molecule of L--phenylalanine via an amide bond. OTA contamination of wines might be a risk to consumer health, thus requiring treatments to achieve acceptable standards for human consumption [1]. According to the Regulation No. 1881/2006 of the European Commission, the maximum limit for OTA in wine is 2 µg/kg [2]. Therefore, the aim of this work was to know the effect of different fining agents on OTA removal, as well as their impact on white and red wine physicochemical characteristics. To evaluate their efficiency, 11 commercial fining agents (mineral, synthetic, animal and vegetable proteins) were used to get new approaches on OTA removal from white and red wines. Trials were performed in wines artificially supplemented (at a final concentration of 10 µg/L) with OTA. The most effective fining agent in removing OTA (80%) from white wine was a commercial formulation that contains gelatine, bentonite and activated carbon. Removals between 10-30% were obtained with potassium caseinate, yeast cell walls and pea protein. With bentonites, carboxymethylcellulose, polyvinylpolypyrrolidone and chitosan no considerable OTA removal was verified. In red wine, removals between 6-19% were obtained with egg albumin, yeast cell walls, pea protein, isinglass, gelatine, polyvinylpolypyrrolidone and chitosan. The most effective fining agents in removing OTA from red wine were an activated carbon (66%) followed again by the commercial formulation (55%), being activated carbon a well-known adsorbent of mycotoxins. These results may provide useful information for winemakers, namely for the selection of the most appropriate oenological product for OTA removal, reducing wine toxicity and simultaneously enhancing food safety and wine quality.

Analytical evaluation of fining treatments for white wines contaminated by Ochratoxin A

Mycotoxins are toxic secondary metabolites produced by certain molds. Ochratoxin A (OTA) is one of the most relevant. Its chemical structure is a dihydro-isocoumarin connected at the 7-carboxy group to a molecule of L--phenylalanine via an amide bond. OTA in wine is a risk to consumer health [1]. According to the Regulation No. 123/2005 of the European Commission, the maximum limit for OTA in wine is 2 µg/kg [2]. Then, it is important to control its occurrence. So, the aim of this work was to know the effect of different fining agents on OTA removal from white wine.

Food safety in wine: removal of ochratoxin A in contaminated white wine using commercial fining agents

The presence of mycotoxins in foodstuff is a matter of concern for food safety. Mycotoxins are toxic secondary metabolites produced by certain molds, being ochratoxin A (OTA) one of the most relevant. Wines can also be contaminated with these toxicants. Several authors have demonstrated the presence of mycotoxins in wine, especially ochratoxin A (OTA) [1]. Its chemical structure is a dihydro-isocoumarin connected at the 7-carboxy group to a molecule of L--phenylalanine via an amide bond. As these toxicants can never be completely removed from the food chain, many countries have defined levels in food in order to attend health concerns. OTA contamination of wines might be a risk to consumer health, thus requiring treatments to achieve acceptable standards for human consumption [2]. The maximum acceptable level of OTA in wines is 2.0 g/kg according to the Commission regulation No. 1881/2006 [3]. Therefore, the aim of this work was to reduce OTA to safer levels using different fining agents, as well as their impact on white wine physicochemical characteristics. To evaluate their efficiency, 11 commercial fining agents (mineral, synthetic, animal and vegetable proteins) were used to get new approaches on OTA removal from white wine. Trials (including a control without addition of a fining agent) were performed in white wine artificially supplemented with OTA (10 µg/L). OTA analysis were performed after wine fining. Wine was centrifuged at 4000 rpm for 10 min and 1 mL of the supernatant was collected and added of an equal volume of acetonitrile/methanol/acetic acid (78:20:2 v/v/v). Also, the solid fractions obtained after fining, were centrifuged (4000 rpm, 15 min), the resulting supernatant discarded, and the pellet extracted with 1 mL of the above solution and 1 mL of H2O. OTA analysis was performed by HPLC with fluorescence detection according to Abrunhosa and Venâncio [4]. The most effective fining agent in removing OTA (80%) from white wine was a commercial formulation that contains gelatine, bentonite and activated carbon. Removals between 10-30% were obtained with potassium caseinate, yeast cell walls and pea protein. With bentonites, carboxymethylcellulose, polyvinylpolypyrrolidone and chitosan no considerable OTA removal was verified. Following, the effectiveness of seven commercial activated carbons was also evaluated and compared with the commercial formulation that contains gelatine, bentonite and activated carbon. The different activated carbons were applied at the concentration recommended by the manufacturer in order to evaluate their efficiency in reducing OTA levels. Trial and OTA analysis were performed as explained previously. The results showed that in white wine all activated carbons except one reduced 100% of OTA. The commercial formulation that contains gelatine, bentonite and activated carbon (C8) reduced only 73% of OTA concentration. These results may provide useful information for winemakers, namely for the selection of the most appropriate oenological product for OTA removal, reducing wine toxicity and simultaneously enhancing food safety and wine quality.

Vascular disease modeling using induced pluripotent stem cells: Focus in Hutchinson-Gilford Progeria Syndrome

Transparency document related to this article can be found online at http://dx.doi.org/10.1016/j.bbrc.2015.10.014