Insight into Elderly ALS Patients in the Emilia Romagna Region: Epidemiological and Clinical Features of Late-Onset ALS in a Prospective, Population-Based Study

Few studies have focused on elderly (>80 years) amyotrophic lateral sclerosis (ALS) patients, who represent a fragile subgroup generally not included in clinical trials and often neglected because they are more difficult to diagnose and manage. We analyzed the clinical and genetic features of very late-onset ALS patients through a prospective, population-based study in the Emilia Romagna Region of Italy. From 2009 to 2019, 222 (13.76%) out of 1613 patients in incident cases were over 80 years old at diagnosis, with a female predominance (F:M = 1.18). Elderly ALS patients represented 12.02% of patients before 2015 and 15.91% from 2015 onwards (p = 0.024). This group presented with bulbar onset in 38.29% of cases and had worse clinical conditions at diagnosis compared to younger patients, with a lower average BMI (23.12 vs. 24.57 Kg/m2), a higher progression rate (1.43 vs. 0.95 points/month), and a shorter length of survival (a median of 20.77 vs. 36 months). For this subgroup, genetic analyses have seldom been carried out (25% vs. 39.11%) and are generally negative. Finally, elderly patients underwent less frequent nutritional- and respiratory-supporting procedures, and multidisciplinary teams were less involved at follow-up, except for specialist palliative care. The genotypic and phenotypic features of elderly ALS patients could help identify the different environmental and genetic risk factors that determine the age at which disease onset occurs. Since multidisciplinary management can improve a patient’s prognosis, it should be more extensively applied to this fragile group of patients

Le grandi cerimonie nei media. Caratteri, riprese e messaggi di Expo 2015

Indagine sulla copertura mediale dell'Esposizione Universali di Milano 2015

Epidemiological, Clinical and Genetic Features of ALS in the Last Decade: A Prospective Population-Based Study in the Emilia Romagna Region of Italy

: Increased incidence rates of amyotrophic lateral sclerosis (ALS) have been recently reported across various Western countries, although geographic and temporal variations in terms of incidence, clinical features and genetics are not fully elucidated. This study aimed to describe demographic, clinical feature and genotype-phenotype correlations of ALS cases over the last decade in the Emilia Romagna Region (ERR). From 2009 to 2019, our prospective population-based registry of ALS in the ERR of Northern Italy recorded 1613 patients receiving a diagnosis of ALS. The age- and sex-adjusted incidence rate was 3.13/100,000 population (M/F ratio: 1.21). The mean age at onset was 67.01 years; women, bulbar and respiratory phenotypes were associated with an older age, while C9orf72-mutated patients were generally younger. After peaking at 70-75 years, incidence rates, among women only, showed a bimodal distribution with a second slight increase after reaching 90 years of age. Familial cases comprised 12%, of which one quarter could be attributed to an ALS-related mutation. More than 70% of C9orf72-expanded patients had a family history of ALS/fronto-temporal dementia (FTD); 22.58% of patients with FTD at diagnosis had C9orf72 expansion (OR 6.34, p = 0.004). In addition to a high ALS incidence suggesting exhaustiveness of case ascertainment, this study highlights interesting phenotype-genotype correlations in the ALS population of ERR

LIBER AMICORUM PER ANTONIO MATACENA

Vi sono professori che per spessore scientifico, capacit\ue0 divulgativa e carisma personale si distinguono da tutti gli altri e divengono \u201cmaestri\u201d, proprio come l\u2019etimo della parola richiederebbe. Per questi \u2013 e solo per questi \u2013 \ue8 consuetudine che nell\u2019ultimo anno accademico di ruolo venga curato un libro collettaneo, per celebrare i contributi scientifici e la figura accademica del maestro. La forma pi\uf9 diffusa e istituzionale \ue8 quella degli Scritti in onore di. Chi conosce Antonio Matacena, tuttavia, sa bene che le soluzioni istituzionali e pi\uf9 rassicuranti mal si adattano alla sua personalit\ue0 e al suo impegno intellettuale, sempre attento alla \u201calterit\ue0\u201d e spesso anticonvenzionale. Abbiamo quindi ritenuto che un liber amicorum \u2013 in grado di ospitare contributi eterogenei e non solo accademici \u2013 fosse la soluzione pi\uf9 efficacie per rappresentare la passione per l\u2019insegnamento e la gioia della condivisione che, insieme all\u2019innovazione scientifica, hanno caratterizzato la vita del nostro Maestro Antonio Matacena. In particolare, questo volume pi\uf9 che essere un liber amicorum, vuole essere un liber ab amicis, ossia una testimonianza dagli amici per il prof. Matacena, per ringraziarlo del ruolo che ha saputo svolgere nella vita di ciascuno, come studioso e come docente