\uabSo che cos'\ue8 ma non so come usarlo!\ubb: Un caso di aprassia ideativa

Despite a mass of research on the cognitive basis for tool use, the term Ideational Apraxia is still controversial, and it is used to refer to a number of different types of deficits and describe very different behaviours. We report the case of a right-handed woman (FL) who exhibited selective tool use disturbance in the context of a cognitive profile otherwise preserved. FL shows a striking dissociation between her failure to manipulate objects and her perfect knowledge about their functions and properties. Such dissociation would indicate the existence of two separate systems, one involved in object identification and one in object manipulation: normally, the two systems operate together, however they can be selectively compromised by cerebral lesions. In FL, the automatic selection of action schemas necessary for correct object use appears to be lost, while her ability to identify and recognize their mechanical and functional properties is spared

Development of spoilage microbiota in beef stored in nisin-activated packaging

The aim of this study was to assess the microbial populations causing the spoilage of chilled beef during storage and to evaluate the effect of the use of an antimicrobial packaging for the meat storage. A nisin activated antimicrobial packaging was developed by using a nisin, HCL and EDTA solution and used for the storage of beef cuts at 1°C. The common spoilage related microbial groups were monitored during the storage of beef in activated and non activated plastic bags by using selective media. The use of the antimicrobial packaging caused an overall significant reduction of viable counts of Gram positive bacteria such as carnobacteria, lactic acid bacteria and Brochotrix thermosphacta whose development was inhibited for at least 11 days of storage compared to the control. Moreover, a 1-3 log cycles reduction of enterobacteria was also registered between 22 and 32 days of storage. The microbiota was assessed at species level by using Polymerase chain reaction-denaturing gradient gel electrophoresis (PCR-DGGE) analysis of 16S rRNA gene of DNA extracted directly from meat and from bulk cells from selective media plates and showed that the species occurring within the targeted microbial groups did not change according to storage conditions. In conclusion, the use of the nisin activated packaging reduced the number of spoilage populations but did not affect the species diversity. Improved antimicrobial packaging is needed, possibly coupled with vacuum storage, to possibly achieve a simultaneous inhibition of more spoilage microbial groups and to preserve the microbiological quality of beef during chilled storage

Antibodies against human cytomegalovirus late protein UL94 in the pathogenesis of scleroderma-like skin lesions in chronic graft-versus-host disease

Human cytomegalovirus (hCMV) infection and its reactivation correlate both with the increased risk and with the worsening of graft-versus-host disease (GVHD). Because scleroderma-like skin lesions can occur in chronic GVHD (cGVHD) in allogeneic stem-cell transplant (HCT) patients and hCMV is relevant in the pathogenesis of systemic sclerosis (SSc), we evaluated the possible pathogenetic link between hCMV and skin cGVHD. Plasma from 18 HCT patients was tested for anti-UL94 and/or anti-NAG-2 antibodies, identified in SSc patients, by direct ELISA assays. Both donors and recipients were anti-hCMV IgG positive, without autoimmune diseases. Patients\u2019 purified anti-UL94 and anti- NAG-2 IgG binding to human umbilical endothelial cells (HUVECs) and fibroblasts was performed by FACS analysis and ELISA test. HUVECs apoptosis and fibroblasts proliferation induced by patients\u2019 anti-NAG-2 antibodies were measured by DNA fragmentation and cell viability, respectively. About 11/18 patients developed cGVHD and all of them showed skin involvement, ranging from diffuse SSc-like lesions to limited erythema. Eight of eleven cGVHD patients were positive for anti-UL94 and/or anti-NAG-2 antibodies. Remarkably, 4/5 patients who developed diffuse or limited SSc-like lesions had antibodies directed against both UL94 and NAG-2; their anti-NAG-2 IgG-bound HUVECs and fibroblasts induce both endothelial cell apoptosis and fibroblasts proliferation, similar to that induced by purified anti-UL94 and anti-NAG-2 antibodies obtained from SSc patients. In conclusion, our data suggest a pathogenetic link between hCMV infection and scleroderma-like skin cGVHD in HCT patients through a mechanism of molecular mimicry between UL94 viral protein and NAG-2 molecule, as observed in patients with SSc

Alpha-synuclein seeds in olfactory mucosa and cerebrospinal fluid of patients with dementia with Lewy bodies

In patients with suspected dementia with Lewy bodies, the detection of the disease-associated alpha-synuclein in easily accessible tissues amenable to be collected using minimally invasive procedures remains a major diagnostic challenge. This approach has the potential to take advantage of modern molecular assays for the diagnosis of alpha-synucleinopathy and, in turn, to optimize the recruitment and selection of patients in clinical trials, using drugs directed at counteracting alpha-synuclein aggregation. In this study, we explored the diagnostic accuracy of alpha-synuclein real-time quaking-induced conversion assay by testing olfactory mucosa and CSF in patients with a clinical diagnosis of probable (n=32) or prodromal (n=5) dementia with Lewy bodies or mixed degenerative dementia (dementia with Lewy bodies/Alzheimer's disease) (n=6). Thirty-eight patients with non-alpha-synuclein-related neurodegenerative and non-neurodegenerative disorders, including Alzheimer's disease (n=10), sporadic Creutzfeldt-Jakob disease (n=10), progressive supranuclear palsy (n=8), corticobasal syndrome (n=1), fronto-temporal dementia (n=3) and other neurological conditions (n=6) were also included, as controls. All 81 patients underwent olfactory swabbing while CSF was obtained in 48 participants. At the initial blinded screening of olfactory mucosa samples, 38 out of 81 resulted positive while CSF was positive in 19 samples out of 48 analysed. After unblinding of the results, 27 positive olfactory mucosa were assigned to patients with probable dementia with Lewy bodies, five with prodromal dementia with Lewy bodies and three to patients with mixed dementia, as opposed to three out 38 controls. Corresponding results of CSF testing disclosed 10 out 10 positive samples in patients with probable dementia with Lewy bodies and six out of six with mixed dementia, in addition to three out of 32 for controls. The accuracy among results of real-time quaking-induced conversion assays and clinical diagnoses was 86.4% in the case of olfactory mucosa and 93.8% for CSF. For the first time, we showed that alpha-synuclein real-time quaking-induced conversion assay detects alpha-synuclein aggregates in olfactory mucosa of patients with dementia with Lewy bodies and with mixed dementia. Additionally, we provided preliminary evidence that the combined testing of olfactory mucosa and CSF raised the concordance with clinical diagnosis potentially to 100%. Our results suggest that nasal swabbing might be considered as a first-line screening procedure in patients with a diagnosis of suspected dementia with Lewy bodies followed by CSF analysis, as a confirmatory test, when the result in the olfactory mucosa is incongruent with the initial clinical diagnosis

Predictive factors of Status Epilepticus and its recurrence in patients with adult-onset seizures: A multicenter, long follow-up cohort study

Purpose: Status epilepticus (SE) is associated with high morbidity and mortality. This multicenter retrospective cohort study aims to identify the factors associated with the occurrence of SE and the predictors of its recurrence in patients with adult-onset seizures. Methods: We retrospectively analyzed data of 1115 patients with seizure onset>18 years, observed from 1983 to 2020 in 7 Italian Centers (median follow-up 2.1 years). Data were collected from the databases of the Centers. Patients with SE were consecutively recruited, and patients without SE history were randomly selected in a 2:1 ratio. To assess determinants of SE, different clinical-demographic variables were evaluated and included in univariate and multivariate logistic regression model. Results: Three hundred forty-seven patients had a SE history, whereas the remaining 768 patients had either isolated seizures or epilepsy without SE history. The occurrence of SE was independently associated with increasing age at onset of disease (OR 1.02, 95% CI 1.01--1.03, p<0.001), female sex (OR 1.39, 95% CI 1.05--1.83, p=0.02) and known etiology (OR 3.58, 95% CI 2.61--4.93, p<0.001). SE recurred in 21% of patients with adult-onset SE and recurrence was associated with increasing number of anti-seizure medications taken at last follow-up (OR 1.88, 95% CI 1.31--2.71, p<0.001). Conclusions: In patients with adult-onset seizures, SE occurrence is associated with known etiologies, advanced age , female sex. Patients with recurrent SE are likely to have a refractory epilepsy, deserving careful treat-ment to prevent potentially fatal events