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Wild-type transthyretin amyloidosis (ATTRwt amyloidosis) is a progressive disease resulting from the accumulation of wild-type transthyretin (TTR) amyloid fibrils, and is diagnosed primarily in males. This analysis examined sex differences in patients with ATTRwt amyloidosis from the Transthyretin Amyloidosis Outcomes Survey (THAOS).THAOS is an ongoing, global, longitudinal, observational survey of patients with transthyretin amyloidosis, including both inherited and wild-type disease, and asymptomatic carriers of TTR mutations. THAOS data were analyzed to identify potential differences in demographic and clinical characteristics between males and females with ATTRwt amyloidosis (data cutoff: August 1, 2021).Of 1386 patients with ATTRwt amyloidosis, 84 (6%) were female and 1302 (94%) were male. Females had a higher median age at enrollment (80 vs. 78 years; p = 0.002) and symptom onset (75 vs. 73 years; p = 0.045) than males. Mean left ventricular (LV) ejection fraction was higher (53% vs. 48%; p = 0.001) and mean LV diastolic diameter lower (42 vs. 46 mm; p 0.001) in females versus males, but sex was not identified as a predictor of LV mean wall thickness adjusted for height (beta coefficient - 0.22; p = 0.460) or a predominantly cardiac phenotype (odds ratio 1.60; p = 0.191). Modified polyneuropathy disability scores differed between groups (p 0.001), with a larger proportion of scores ≥ IIIa among females (23% vs. 7%).Females with ATTRwt amyloidosis in THAOS tended to present at a later age and showed signs of less severe cardiac impairment and more severe walking impairment.ClinicalTrials.gov: NCT00628745.

Analisi critica di una decisione di legittimità (Cass., sez. V, 23 luglio 2020, n. 25838) che ha riguardato le modalità di partecipazione a distanza all’udienza di riesame in un processo relativo all'accertamento di fatti di mafia. Oltre che sull'eventuale esistenza di invalidità processuali il contributo si sofferma sui criteri valutativi utilizzati in questa peculiare tipologia di accertamenti.

Dementia with Lewy bodies (DLB) causes elevated outlays for the National Health Systems due to high institutionalization rate and patients' reduced quality of life and high mortality. Furthermore, DLB is often misdiagnosed as Alzheimer's disease. These data motivate harmonized multicenter longitudinal cohort studies to improve clinical management and therapy monitoring. The Italian DLB study group of the Italian Neurological Society for dementia (SINdem) developed and emailed a semi-structured questionnaire to 572 national dementia centers (from primary to tertiary) to prepare an Italian large longitudinal cohort. The questionnaire surveyed: (1) prevalence and incidence of DLB; (2) clinical assessment; (3) relevance and availability of diagnostic tools; (4) pharmacological management of cognitive, motor, and behavioural disturbances; (5) causes of hospitalization, with specific focus on delirium and its treatment. Overall, 135 centers (23.6 %) contributed to the survey. Overall, 5624 patients with DLB are currently followed by the 135 centers in a year (2042 of them are new patients). The percentage of DLB patients was lower (27 ± 8 %) than that of Alzheimer's disease and frontotemporal dementia (56 ± 27 %) patients. The majority of the centers (91 %) considered the clinical and neuropsychological assessments as the most relevant procedure for a DLB diagnosis. Nonetheless, most of the centers has availability of magnetic resonance imaging (MRI; 95 %), electroencephalography (EEG; 93 %), and FP-CIT single photon emission-computerized tomography (SPECT; 75 %) scan for clinical applications. It will be, therefore, possible to recruit a large harmonized Italian cohort of DLB patients for future cross-sectional and longitudinal multicenter studies.

Conservation Science in Cultural Heritage, Vol 17 (2017)

Different aspects of episodic long-term, short-term and implicit long-term memory were investigated in subjects who strictly fulfilled the criteria for the amnestic form of Mild Cognitive Impairment (a-MCI). Results showed normal short-term memory abilities in these subjects, while each of the episodic long-term memory indices explored showed poorer results in a-MCI subjects with respect to normal controls. Although some episodic memory functions were relatively well preserved, others appeared to have deteriorated to a level comparable to that of mild AD patients. The finding of an extensive impairment of all memory functions depending on hippocampal structures in a population with a high risk of developing dementia is strongly supportive of the hypothesis that a pure amnesic syndrome characterizes the preclinical phase of AD. Copyright © Taylor & Francis Ltd.

In the prospect of improved disease management and future clinical trials in Frontotemporal Dementia, it is desirable to share common diagnostic procedures. To this aim, the Italian FTD Network, under the aegis of the Italian Neurological Society for Dementia, has been established. Currently, 85 Italian Centers involved in dementia care are part of the network. Each Center completed a questionnaire on the local clinical procedures, focused on (1) clinical assessment, (2) use of neuroimaging and genetics; (3) support for patients and caregivers; (4) an opinion about the prevalence of FTD. The analyses of the results documented a comprehensive clinical and instrumental approach to FTD patients and their caregivers in Italy, with about 1,000 newly diagnosed cases per year and 2,500 patients currently followed by the participating Centers. In analogy to other European FTD consortia, future aims will be devoted to collect data on epidemiology of FTD and its subtypes and to provide harmonization of procedures among Centers.

contenuti del volume “La gestione del rischio nel settore dei beni culturali: musei, biblioteche, archivi”, secondo della Collana “La Formazione e la Ricerca nel settore dei Beni Culturali e Ambientali”, sono riconducibili preliminarmente ad un excursus storico sulla situazione normativa in materia di sicurezza dei beni culturali in Italia e a livello internazionale con la conseguente trattazione di procedure di valutazione e prevenzione, linee guida e sistemi di sicurezza, in particolare, negli ambienti confinati. Si rivolge, quindi, l’attenzione alle varie tipologie di rischio e alle corrispondenti metodologie scientifiche in un confronto di percorsi metodologici e monitoraggi stabiliti anche da Organizzazioni Internazionali, affrontando alcuni casi di studio che, nell’ambito del Laboratorio Diagnostico per i Beni Culturali del Dipartimento di Beni Culturali dell’Alma Mater Studiorum Università di Bologna, sono stati oggetto di studio e applicazione.

Interpretazione delle nuove disposizioni contenute nel d.l. 23 maggio 2008, conv. nella l. 14 luglio 2008, n.90.

Summary This study, addresses the specific subject relating to the formulation of the economic value of art works in connection with their attribution and authentication. Through this process, which also includes regulatory issues, it follows that the current problem in the field of forgery and, in general inauthentic works, is precisely that of ascertaining, by means of scientific methods, whether a work of art is authentic or not. With this aim in mind, the following research project has been proposed and aims to develop the right methodological path to use to achieve this aim. It includes a cognitive phase involving aesthetic, stylistic, iconographic, and historical analysis and concludes with a technical-experimental phase in which diagnostic-analytical and computer technologies are employed. On completion of the research, combined the diagnostic and analytical results, as well as the development of software allowing the style of a particular artist to be identified, will provide a valuable contribution to the various and complex issues relating to the attribution and authentication of art works. Riassunto Lo studio è rivolto alla specifica tematica relativa alla formulazione del valore economico delle opere d’arte collegato alla loro attribuzione ed autenticazione. Attraverso questo percorso, che include anche gli aspetti normativi, ne deriva che la problematica attuale, in materia di falsi e, in genere, di opere non autentiche, è proprio quella di accertare mediante metodi scientifici l’autenticità o non di un’opera d’arte. A tal riguardo di seguito viene proposto un progetto di ricerca che si prefigge di mettere a punto il corretto e completo percorso metodologico, che comprende la fase conoscitiva relativa all’analisi di carattere estetico, stilistico, iconografico, storico, e si completa con la fase tecnico-sperimentale mediante l’utilizzo di tecnologie diagnostico-analitiche ed informatiche. Alla conclusione della ricerca, la combinazione delle risultanze diagnostico-analitiche, nonché la messa a punto di un software che permetta di identificare lo stile di un determinato artista, forniranno un valido contributo in riferimento alle diverse e complesse problematiche relative alla attribuzione e autenticazione delle opere d’arte. Résumé Cette étude s’adresse au thème spécifique de la formulation de la valeur économique des oeuvres d’art en lien avec leur attribution et authentification. Ce parcours, qui se penche aussi sur les questions réglementaires, fait ressortir qu’en matière de faux et en général, d’oeuvres non authentiques, l’enjeu consiste aujourd’hui à vérifier, par des méthodes scientifiques, l’authenticité ou la non-authenticité d’une oeuvre d’art. À ce propos, le projet de recherche qui est proposé vise à mettre au point un parcours méthodologique correct et complet, articulé en une phase de connaissance de l’oeuvre, par son analyse esthétique, stylistique, iconographique, historique. Celle-ci est suivie d’une phase technique et expérimentale, à l’aide de techniques diagnostiques, analytiques et informatiques. À la fin de la recherche, l’ensemble des résultats diagnostiques et analytiques et la mise au point d’un logiciel permettant d’identifier le style d’un artiste donné apporteront une aide précieuse, eu égard des problématiques multiples et complexes qui se posent dans le cadre de l’attribution et de l’authentification des oeuvres d’art. Zusammenfassung Diese Studie befasst sich spezifisch mit der Thematik der Bestimmung des wirtschaftlichen Werts von Kunstwerken in Verbindung mit deren Zuordnung und Authentifizierung. Aus diesen Betrachtungen, die auch den Aspekt der gesetzlichen Vorschriften berücksichtigen, ergibt sich, dass die heutige Problematik der Fälschungen und generell der nicht authentischen Werke gerade darin liegt, anhand wissenschaftlicher Methoden die Echtheit oder Nicht-Echtheit eines Kunstwerks zu ermitteln. Diesbezüglich wird im Weiteren ein Forschungsprojekt vorgestellt, das sich das Ziel gesteckt hat, eine korrekte und vollständige Methode auszuarbeiten, die die kognitive Phase der Analysen ästhetischer, stilistischer, ikonographischer, historischer Art umfasst, und mit der technisch-experimentellen Phase abschließt, wobei diagnostisch-analytische sowie datentechnische Technologien herangezogen werden. Bei Abschluss der Forschungsarbeit sollen die Kombination der diagnostisch-analytischen Ergebnisse und der Erstellung einer Software, die es gestattet, den Stil eines bestimmten Künstlers zu erkennen, einen wesentlichen Beitrag zur Lösung der verschiedenen und komplexen Problematiken im Rahmen der Zuordnung und Authentifizierung von Kunstwerken leisten. Resumen El estudio se centra en la cuestión específica de la formulación del valor económico de las obras de arte en relación con su atribución y autenticación. Siguiendo el hilo argumental del texto, que también incluye aspectos normativos, se deduce que en referencia a las falsificaciones y, en general, a las obras no auténticas, hoy día el principal problema está en la verificación, aplicando métodos científicos, de la autenticidad o no de una obra de arte. A continuación se describe un proyecto de investigación a este respecto que se propone establecer una metodología correcta y completa, articulada en dos fases: una de conocimiento, fundada en un análisis estético, estilístico, iconográfico e histórico; y otra técnico-experimental, basada en el uso de tecnología diagnóstico-analítica e informática. Al final de la investigación, la combinación de los resultados diagnóstico-analíticos, sumada a la creación de un programa informático que permita identificar el estilo de un determinado artista, contribuirá de modo relevante a arrojar luz sobre los distintos y complejos problemas relativos a la atribución y autenticación de las obras de arte. 简介 研究主题围绕着艺术作品的经济价值换算公式，联系作品归属及真伪鉴定展 开。通过这一过程，附带法律法规方面参考，针对仿制、伪造，从中引申出现 实问题，即通过科学途径鉴定艺术作品的真伪。 接下来是一项分析研究计划，需要预先确定其使用的研究方法正确全面，其中 需包含关于美学特色、风格特色、肖像学特色、历史特色研究的认知性分析步 骤，以及通过使用诊断-分析技术和信息技术的技术-实验性分析步骤。 在研究的结论中，诊断-分析结论的融合，与分析特定艺术家风格软件的加 入，针对艺术作品归属及真伪鉴定这个庞杂的问题，提供一个准确可信的结论。 Резюме Эта работа посвящена вопросам определения экономической ценности произ-ведений искусства в зависимости от установления их авторства и подлинности. Из этого подхода, затрагивающего также и нормативные аспекты, следует, что самый актуальный вопрос в сфере подделок и репродукций произведений заклю-чается именно в определении того, подлинно или нет произведение искусства, применяя для этого научные методы. В этих целях предложен проект исследований, направленный на разработку правильного и комплексного методологического подхода, включающего позна-вательный этап с эстетическим, стилистическим, иконографическим и историче-ским анализом, и завершающийся технико-экспериментальным этапом с приме-нением аналитическо-диагностических и информационных технологий. По итогам исследовательской работы сочетание аналитическо-диагностиче-ских результатов, а также и разработка программного обеспечения, позволяю-щего идентифицировать стиль определенного художника, предоставят дейст-венную помощь при решении многочисленных и сложных проблем, связанных с установлением авторства и подлинности произведений искусства., Conservation Science in Cultural Heritage, Vol 14 (2014)

The early differentiation of Alzheimer’s disease (AD) from frontotemporal dementia (FTD) may be difficult. The Tower of London (ToL), thought to assess executive functions such as planning and visuo-spatial working memory, could help in this purpose. Twentytwo Dementia Centers consecutively recruited patients with early FTD or AD. ToL performances of these groups were analyzed using both the conventional statistical approaches and the Artificial Neural Networks (ANNs) modelling. Ninety-four non aphasic FTD and 160 AD patients were recruited. ToL Accuracy Score (AS) significantly (p < 0.05) The use of hidden information contained in the different items of ToL and the non linear processing of the data through ANNs allows a high discrimination between FTD and AD in individual patients. However, the discriminant validity of AS checked by ROC curve analysis, yielded no significant results in terms of sensitivity and specificity (AUC 0.63). The performances of the 12 Success Subscores (SS) together with age, gender and schooling years were entered into advanced ANNs developed by Semeion Institute. The best ANNs were selected and submitted to ROC curves. The nonlinear model was able to discriminate FTD from AD with an average AUC for 7 independent trials of 0.82. The use of hidden information contained in the different items of ToL and the non linear processing of the data through ANNs allows a high discrimination between FTD and AD in individual patients.

BACKGROUND/OBJECTIVES: Subjects affected by mild cognitive impairment (MCI) may improve during the observation period. This is the first study investigating qualitative features of memory deficits in subjects affected by reversible MCI [reversible cognitive impairment (RCI)]. METHODS: Baseline cognitive and memory performances of 18 subjects affected by amnestic MCI who had normalized cognitive performances at follow-ups were compared with those of 76 amnestic MCI subjects who still showed impaired cognitive performances at the 24-month follow-up (MCI) and with those of a group of 87 matched control subjects (normal controls). RESULTS: Compared with normal controls the memory deficit in the MCI group affected all aspects of explicit long-term memory functioning; in the RCI group, instead, the memory deficit only affected the free recall of verbal material, particularly when the encoding could be improved by the use of semantic strategies. CONCLUSIONS: These results are consistent with the view that the memory deficit in the MCI group is due to a very early degenerative pathology; in the RCI group, instead, a transitory reduction of processing resources, resulting a poor encoding of incoming material, is likely at the origin of the reversible memory disorder.

Background: Different rates and cognitive predictors of conversion to dementia have been reported in subjects with different kinds of mild cognitive impairment (MCI). Methods: A prospective, 24-month follow-up study, involving 269 subjects who strictly fulfilled criteria for the amnestic MCI. Results: Conversion rate to dementia was 21.4% per year. Seventy-nine out of the 83 individuals who developed dementia were affected by probable Alzheimer’s disease (AD). Among others, at the 24-month follow-up 24.1% were still affected by amnestic MCI, 13.3% had changed their neuropsychological profile of impairment and 17.2% were cognitively normalised. Compared to subjects who did not convert to AD, those who did convert showed poorer immediate and delayed recall and recognition of verbal and visual material at baseline as well as reduced executive abilities. A combination of age, Clinical Dementia Rating boxes and scores on delayed recall and recognition of verbal and visual material accurately identified 86% of the subjects who developed AD. Conclusions: Elderly subjects affected by an isolated memory disorder have a high probability of developing AD. The ability of verbal and visual measures to predict incipient dementia of memory impairment may be increased by the simultaneous assessment of individual features, such as age or rate of functional impairment.

This study aimed at investigating the clinical usefulness of the Mental Deterioration Battery (MDB) in the neuropsychological diagnosis and characterization of the dementia syndrome. In this paper, we report: (a) normative data for various test scores derived from the analysis of performance of 340 normal subjects living in urban areas; (b) an evaluation of the reliability of the single tests and of the battery as a whole in differentiating normal subjects from patients affected by cognitive deterioration derived from the analysis of performance of 130 normal subjects living in rural areas and 134 patients affected by probable Alzheimer's dementia; (c) a cluster analysis of performances of the 340 normal subjects in the standardization group to evaluate possible criteria of homogeneity according to which the various MDB scores tend to aggregate; (d) an analysis of performance profiles of 183 patients with right monohemispheric focal lesions, 159 patients with left unilateral lesions with aphasia and 131 left-lesioned nonaphasic patients to evaluate the specificity of the single tests of the battery in documenting a selective impairment of one of the two cerebral hemispheres. Results confirm the reliability of the MBD in discriminating between normal and demented patients and provide indications for use of the battery in differentiating qualitative patterns of cognitive impairment.