30 research outputs found

    Association of CSF and PET markers of neurodegeneration with electroclinical progression in Lafora disease

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    PurposeTo evaluate the electro-clinical features in association with laboratory and instrumental correlates of neurodegeneration to detect the progression of Lafora disease (LD).MethodsWe investigated the electro-clinical longitudinal data and CSF Aβ42, p-tau181 and t-tauAg, amyloid, and 18F-FDG PET of five unrelated LD families.ResultsThree progressive electro-clinical stages were identified. The early phase was characterized by rare, generalized tonic-clonic and focal visual seizures, followed by the occurrence of myoclonus after a period ranging from 2 to 12 months. The intermediate stage, usually occurring 2 years after the onset of epilepsy, is characterized by a worsening of epilepsy and myoclonus associated with progressive dementia and cerebellar signs. Finally, the late stage, evolving after a mean period of 7 ± 1.41 years from the onset of the disease, was characterized by gait ataxia resulting in bedriddenness, severe dementia, daily/pluri-daily myoclonus, drug-resistant epilepsy, clusters of seizures or status epilepticus, and medical complications. Amyloid (CSF Aβ42, amyloid PET) and neurodegenerative (CSF p-tau181 and t-tauAg, FDG-PET) biomarkers indicate a pattern of cognitive impairment of the non-Alzheimer's disease type. A total of 80% of the LD patients showed more severe hypometabolism in the second FDG-PET scan compared to the first scan performed in a lower phase; the lateral temporal lobe and the thalamus hypometabolism were associated with the presence of intermediate or late phase.ConclusionsThree electroclinical and 18F-FDG PET evolutive stages are useful biomarkers for the progression of LD and could help to evaluate the efficacy of new disease-modifying treatments. The combination of traditional CSF biomarkers improves the diagnostic accuracy of cognitive decline in LD patients, indicating a cognitive impairment of the non-Alzheimer's disease type

    3D to 2D reorganization of silver–thiol nanostructures, triggered by solvent vapor annealing

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    Metal–organic composites are of great interest for a wide range of applications. The control of their struc-ture remains a challenge, one of the problems being a complex interplay of covalent and supramolecularinteractions. This paper describes the self-assembly, thermal stability and phase transitions of orderedstructures of silver atoms and thiol molecules spanning from the molecular to the mesoscopic scale.Building blocks of molecularly defined clusters formed from 44 silver atoms, each particle coated by amonolayer of 30 thiol ligands, are used as ideal building blocks. By changing solvent and temperature it ispossible to tune the self-assembled 3D crystals of pristine nanoparticles or, conversely, 2D layered structures, with alternated stacks of Ag atoms and thiol monolayers. The study investigates morphological,chemical and structural stability of these materials between 25 and 300 °Cin situandex situat the nano-scale by combining optical and electronic spectroscopic and scattering techniques, scanning probemicroscopies and density-functional theory (DFT) calculations. The proposed wet-chemistry approach isrelatively cheap, easy to implement, and scalable, allowing the fabricated materials with tuned propertiesusing the same building blocks

    SARS-CoV-2 serology after COVID-19 in multiple sclerosis: An international cohort study

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    COVID-19 Severity in Multiple Sclerosis: Putting Data Into Context

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    Background and objectives: It is unclear how multiple sclerosis (MS) affects the severity of COVID-19. The aim of this study is to compare COVID-19-related outcomes collected in an Italian cohort of patients with MS with the outcomes expected in the age- and sex-matched Italian population. Methods: Hospitalization, intensive care unit (ICU) admission, and death after COVID-19 diagnosis of 1,362 patients with MS were compared with the age- and sex-matched Italian population in a retrospective observational case-cohort study with population-based control. The observed vs the expected events were compared in the whole MS cohort and in different subgroups (higher risk: Expanded Disability Status Scale [EDSS] score > 3 or at least 1 comorbidity, lower risk: EDSS score ≤ 3 and no comorbidities) by the χ2 test, and the risk excess was quantified by risk ratios (RRs). Results: The risk of severe events was about twice the risk in the age- and sex-matched Italian population: RR = 2.12 for hospitalization (p < 0.001), RR = 2.19 for ICU admission (p < 0.001), and RR = 2.43 for death (p < 0.001). The excess of risk was confined to the higher-risk group (n = 553). In lower-risk patients (n = 809), the rate of events was close to that of the Italian age- and sex-matched population (RR = 1.12 for hospitalization, RR = 1.52 for ICU admission, and RR = 1.19 for death). In the lower-risk group, an increased hospitalization risk was detected in patients on anti-CD20 (RR = 3.03, p = 0.005), whereas a decrease was detected in patients on interferon (0 observed vs 4 expected events, p = 0.04). Discussion: Overall, the MS cohort had a risk of severe events that is twice the risk than the age- and sex-matched Italian population. This excess of risk is mainly explained by the EDSS score and comorbidities, whereas a residual increase of hospitalization risk was observed in patients on anti-CD20 therapies and a decrease in people on interferon

    DMTs and Covid-19 severity in MS: a pooled analysis from Italy and France

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    We evaluated the effect of DMTs on Covid-19 severity in patients with MS, with a pooled-analysis of two large cohorts from Italy and France. The association of baseline characteristics and DMTs with Covid-19 severity was assessed by multivariate ordinal-logistic models and pooled by a fixed-effect meta-analysis. 1066 patients with MS from Italy and 721 from France were included. In the multivariate model, anti-CD20 therapies were significantly associated (OR = 2.05, 95%CI = 1.39–3.02, p < 0.001) with Covid-19 severity, whereas interferon indicated a decreased risk (OR = 0.42, 95%CI = 0.18–0.99, p = 0.047). This pooled-analysis confirms an increased risk of severe Covid-19 in patients on anti-CD20 therapies and supports the protective role of interferon

    Tract metastasis from previously unknown malignant pleural mesothelioma: a case report.

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    A case of malignant pleural mesothelioma discovered because of a chest wall metastasis which developed over a previous pleural drainage site is presented. Imaging findings at sonography, contrast enhanced computed tomography and fluorodeoxyglucose - positron emission tomography are shown

    14C Tephrochronology with Different Fractions of Paleosol Humic Matter at Procida Island, Italy

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    From the 13th International Radiocarbon Conference held in Dubrovnik, Yugoslavia, June 20-25, 1988.14C apparent ages along with delta-13C values for three different fractions of humic matter (HM) isolated from a suite of four paleosols (PAs) interbedded within tephra are reported. The dated HM fractions were: 1) HA, the easily released humic acids; 2) HAtot, the bulk of humic acids; 3) RES, the insoluble HM. The 14C sequence dated from 13,000-25,000 BP, in agreement with stratigraphy and previous data. Age differences up to 2540 +/- 430 were statistically significant among fractions; their order of magnitude being independent from C content and depth. All the PAs showed a common pattern of among-fraction age variation, 14C agingltrending from RES to HA101 through HA. As the HM fractions exhibited quite comparable delta-13C values (delta-13C = -25.4 +/- 0.2 per mil), it is inferred that the primitive organic matter (OM) input to PAs was dominantly supplied by vegetation of C-3 photosynthetic pathway which underwent complete decomposition during diagenesis.This material was digitized as part of a cooperative project between Radiocarbon and the University of Arizona Libraries.The Radiocarbon archives are made available by Radiocarbon and the University of Arizona Libraries. Contact [email protected] for further information.Migrated from OJS platform February 202

    Perioperative pain management in cardiac surgery: a systematic review

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    Every year, more than 1.5 million patients, who undergo cardiac surgery worldwide, are exposed to a series of factors that can trigger acute postoperative pain associated with hemodynamic instability, respiratory complications, and psychological disorders. Through an evaluation of literature data about postoperative pain in cardiac surgery we define unmet needs and potential objectives for future research on this often underestimated problem

    68Ga-DOTANOC PET/CT clinical impact in patients with neuroendocrine tumors

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    Several authors reported the superiority of Ga-68-DOTANOC PET/CT to conventional imaging (CI) for the assessment of neuroendocrine tumors (NET). However, the detection of a higher number of lesions is not necessarily followed by a modification of disease stage or therapeutic approach. The aim of this study was to assess the impact of Ga-68-DOTANOC PET/CT on the clinical management of NET patients. Methods: The study included 90 patients with pathologic confirmation of NET, CT performed within a month of Ga-68-DOTANOC PET/CT, and a follow-up period of at least 1 y. PET/CT results were compared with CI results. As a standard of reference to finally evaluate PET results, clinical and imaging follow-up data were used. To assess the clinical impact of PET findings, all referring physicians were contacted after PET and asked about how patients were managed. Stage or therapy modifications were independently recorded, and the overall impact was evaluated patient by patient if PET results either affected therapy or caused a change in disease stage. Results: Considering PET/CT and CI concordant cases (47/90 [52.2%]), PET findings affected the therapeutic management in 17 of 47 (36.2%) patients. Although PET did not result in modification of disease stage, Ga-68-DOTANOC detected a higher lesion number in most patients. PET/CT and CI findings were discordant in 42 of 90 (46.7%) patients: PET resulted in a modification of stage in 12 patients (28.6%) and affected the treatment plan in 32 patients (76.2%). PET and CT were both equivocal in 1 patient (1/90). Considering all cases, Ga-68-DOTANOC PET/CT affected either stage or therapy in 50 of 90 (55.5%) patients. The most frequent impact on management (27 patients) was the initiation or continuance of peptide receptor radionuclide therapy, followed by the initiation or continuance of somatostatin analog medical treatment (7 patients) and referral to surgery (6 patients). PET prevented unnecessary surgery in 6 patients and excluded from treatment with somatostatin analogs 2 patients with NET lesions that did not express somatostatin receptors. Less frequent impacts on management included the initiation of radiotherapy (1 patient), further diagnostic investigation (1 patient), and liver transplantation (1 patient). Conclusion: Ga-68-DOTANOC PET/CT either affected stage or caused a therapy modification in more than half the patients, thus confirming the clinical role of PET in the management of NET
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