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98 results on '"Canosa, A"'

1. High Frequency of Cognitive and Behavioural Impairment in ALS Patients with SOD1 Pathogenic Variants (P5-11.004)

Author

Chio, Adriano, primary, Calvo, Andrea, additional, Moglia, Cristina, additional, Canosa, Antonio, additional, Manera, Umberto, additional, Vasta, Rosario, additional, Grassano, Maurizio, additional, Daviddi, Margherita, additional, Matteoni, Enrico, additional, Brunetti, Maura, additional, Cabras, Sara, additional, Palumbo, Francesca, additional, Mora, Gabriele, additional, and Iazzolino, Barbara, additional

Published
2024
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2. Sex-related Differences in ALS Survival and Progression: Insights from a Population-based Study (P6-11.004)

Author

Grassano, Maurizio, primary, Palumbo, Francesca, additional, Moglia, Cristina, additional, Salamone, Paolina, additional, Fuda, Giuseppe, additional, Vasta, Rosario, additional, Manera, Umberto, additional, Canosa, Antonio, additional, Casale, Federico, additional, Mazzini, Letizia, additional, Calvo, Andrea, additional, and Chio, Adriano, additional

Published
2024
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3. Association of Intermediate HTT CAG Repeats with Increased Risk and Disease Severity in Amyotrophic Lateral Sclerosis (P5-11.003)

Author

Grassano, Maurizio, primary, Canosa, Antonio, additional, Corrado, Lucia, additional, D’Alfonso, Sandra, additional, Manera, Umberto, additional, Vasta, Rosario, additional, Koumantakis, Emanuele, additional, Brodini, Giorgia, additional, Palumbo, Francesca, additional, Moglia, Cristina, additional, Chia, Ruth, additional, Mazzini, Letizia, additional, Calvo, Andrea, additional, Scholz, Sonja, additional, Traynor, Bryan, additional, and Chio, Adriano, additional

Published
2024
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4. Disentangling the Relationship Between Social Cognition, Executive Functions, and Behaviour Changes in Amyotrophic Lateral Sclerosis (S18.009)

Author

Palumbo, Francesca, primary, Iazzolino, Barbara, additional, Callegaro, Stefano, additional, Canosa, Antonio, additional, Vasta, Rosario, additional, Grassano, Maurizio, additional, Matteoni, Enrico, additional, Cabras, Sara, additional, Pellegrino, Giorgio, additional, Salamone, Paolina, additional, Casale, Federico, additional, Fuda, Giuseppe, additional, Moglia, Cristina, additional, Calvo, Andrea, additional, and Chio, Adriano, additional

Published
2024
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5. Cognitive and Behavioral Features of Patients With Amyotrophic Lateral Sclerosis Who Are Carriers of the TARDBP Pathogenic Variant

Author

Moglia, Cristina, primary, Calvo, Andrea, additional, Canosa, Antonio, additional, Manera, Umberto, additional, Vasta, Rosario, additional, Di Pede, Francesca, additional, Daviddi, Margherita, additional, Matteoni, Enrico, additional, Brunetti, Maura, additional, Sbaiz, Luca, additional, Cabras, Sara, additional, Gallone, Salvatore, additional, Grassano, Maurizio, additional, Peotta, Laura, additional, Palumbo, Francesca, additional, Mora, Gabriele, additional, Iazzolino, Barbara, additional, and Chio, Adriano, additional

Published
2024
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8. ALS phenotype is influenced by age, sex, and genetics: A population-based study

Author

Chiò, Adriano, Moglia, Cristina, Canosa, Antonio, Manera, Umberto, DʼOvidio, Fabrizio, Vasta, Rosario, Grassano, Maurizio, Brunetti, Maura, Barberis, Marco, Corrado, Lucia, DʼAlfonso, Sandra, Iazzolino, Barbara, Peotta, Laura, Sarnelli, Maria Francesca, Solara, Valentina, Zucchetti, Jean Pierre, De Marchi, Fabiola, Mazzini, Letizia, Mora, Gabriele, and Calvo, Andrea

Published
2020
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9. Association of Copresence of Pathogenic Variants Related to Amyotrophic Lateral Sclerosis and Prognosis

Author

Chio, Adriano, primary, Moglia, Cristina, additional, Canosa, Antonio, additional, Manera, Umberto, additional, Grassano, Maurizio, additional, Vasta, Rosario, additional, Palumbo, Francesca, additional, Gallone, Salvatore, additional, Brunetti, Maura, additional, Barberis, Marco, additional, De Marchi, Fabiola, additional, Dalgard, Clifton, additional, Chia, Ruth, additional, Mora, Gabriele, additional, Iazzolino, Barbara, additional, Peotta, Laura, additional, Traynor, Bryan J., additional, Corrado, Lucia, additional, dalfonso, sandra, additional, Mazzini, Letizia, additional, and Calvo, Andrea, additional

Published
2023
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10. The role of systemic immunity in ALS: a population-based study (P8-8.006)

Author

Grassano, Maurizio, primary, Manera, Umberto, additional, De Marchi, Fabiola, additional, Matteoni, Enrico, additional, Daviddi, Margherita, additional, Cugnasco, Paolo, additional, Canosa, Antonio, additional, Vasta, Rosario, additional, Mazzini, Letizia, additional, Moglia, Cristina, additional, Calvo, Andrea, additional, and Chio, Adriano, additional

Published
2023
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12. The multistep hypothesis of ALS revisited: The role of genetic mutations

Author

Chiò, Adriano, Mazzini, Letizia, DʼAlfonso, Sandra, Corrado, Lucia, Canosa, Antonio, Moglia, Cristina, Manera, Umberto, Bersano, Enrica, Brunetti, Maura, Barberis, Marco, Veldink, Jan H., van den Berg, Leonard H., Pearce, Neil, Sproviero, William, McLaughlin, Russell, Vajda, Alice, Hardiman, Orla, Rooney, James, Mora, Gabriele, Calvo, Andrea, and Al-Chalabi, Ammar

Published
2018
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13. Unraveling the clinical and genetic spectrum of FUS-ALS (S11.006)

Author

Grassano, Maurizio, primary, Calvo, Andrea, additional, Moglia, Cristina, additional, Canosa, Antonio, additional, Manera, Umberto, additional, Bombaci, Alessandro, additional, Brodini, Giorgia, additional, De Marco, Giovanni, additional, Traynor, Bryan, additional, and Chio, Adriano, additional

Published
2022
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14. GBA Variants Influence Cognitive Status of ALS Patients (S11.004)

Author

Chio, Adriano, primary, Grassano, Maurizio, additional, Moglia, Cristina, additional, Iazzolino, Barbara, additional, Peotta, Laura, additional, Gallone, Salvatore, additional, Brunetti, Maura, additional, Palumbo, Francesca, additional, Cabras, Sara, additional, Manera, Umberto, additional, Vasta, Rosario, additional, Traynor, Bryan, additional, Corrado, Lucia, additional, D‘Alfonso, Sandra, additional, Mazzini, Letizia, additional, Calvo, Andrea, additional, and Canosa, Antonio, additional

Published
2022
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15. Brain Metabolic Changes in Amyotrophic Lateral Sclerosis with SOD1 Mutations (P1-1.Virtual)

Author

Canosa, Antonio, primary, Calvo, Andrea, additional, Moglia, Cristina, additional, Vasta, Rosario, additional, Palumbo, Francesca, additional, Solero, Luca, additional, Di Pede, Francesca, additional, Cabras, Sara, additional, Arena, Vincenzo, additional, Zocco, Grazia, additional, Casale, Federico, additional, Brunetti, Maura, additional, Sbaiz, Luca, additional, Gallone, Salvatore, additional, Grassano, Maurizio, additional, Manera, Umberto, additional, Pagani, Marco, additional, and Chiò, Adriano, additional

Published
2022
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16. Deciphering the Phenotype of ALS Patients with Intermediate ATXN2 PolyQ Expansion (S11.003)

Author

Chio, Adriano, primary, Moglia, Cristina, additional, Canosa, Antonio, additional, Manera, Umberto, additional, Brunetti, Maura, additional, Gallone, Salvatore, additional, Chia, Ruth, additional, Ding, Jinhui, additional, Gibbs, J. Raphael, additional, Dalgard, Clifton, additional, D‘Alfonso, Sandra, additional, Corrado, Lucia, additional, De Marchi, Fabiola, additional, Mazzini, Letizia, additional, Traynor, Bryan, additional, and Calvo, Andrea, additional

Published
2022
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17. The Use of Blood Carbonate (HCO3−) and Base-Excess (SBE) in Predicting Non-Invasive Ventilation (NIV) Adaptation (P1-1.Virtual)

Author

Manera, Umberto, primary, Torrieri, Maria Claudia, additional, Bellocchia, Michela, additional, Ribolla, Fulvia, additional, Canosa, Antonio, additional, Vasta, Rosario, additional, Mora, Gabriele, additional, Mattei, Alessio, additional, Moglia, Cristina, additional, Calvo, Andrea, additional, and Chio, Adriano, additional

Published
2022
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18. Mutational Analysis of Known ALS Genes in an Italian Population-Based Cohort

Author

Maura Brunetti, Sandra D'Alfonso, Luca Sbaiz, Cristina Moglia, Raphael Gibbs, Marco Barberis, Ruth Chia, Rosario Vasta, Umberto Manera, Bryan J. Traynor, Letizia Mazzini, Maurizio Grassano, Adriano Chiò, Antonio Canosa, Andrea Calvo, Jinhui Ding, Clifton L. Dalgard, Lucia Corrado, and Sonja W. Scholz

Subjects
Adult, Male, 0301 basic medicine, DNA Mutational Analysis, Population, Disease, Biology, TARDBP, Article, Cohort Studies, Young Adult, 03 medical and health sciences, 0302 clinical medicine, 80 and over, medicine, Humans, Genetic Predisposition to Disease, Amyotrophic lateral sclerosis, education, Aged, Aged, 80 and over, Genetics, Whole genome sequencing, education.field_of_study, C9orf72 Protein, Amyotrophic Lateral Sclerosis, Case-Control Studies, Female, Italy, Middle Aged, Population Surveillance, Case-control study, medicine.disease, 030104 developmental biology, Cohort, Neurology (clinical), 030217 neurology & neurosurgery, Cohort study
Abstract

ObjectiveTo assess the burden of rare genetic variants and to estimate the contribution of known amyotrophic lateral sclerosis (ALS) genes in an Italian population-based cohort, we performed whole genome sequencing in 959 patients with ALS and 677 matched healthy controls.MethodsWe performed genome sequencing in a population-based cohort (Piemonte and Valle d'Aosta Registry for ALS [PARALS]). A panel of 40 ALS genes was analyzed to identify potential disease-causing genetic variants and to evaluate the gene-wide burden of rare variants among our population.ResultsA total of 959 patients with ALS were compared with 677 healthy controls from the same geographical area. Gene-wide association tests demonstrated a strong association with SOD1, whose rare variants are the second most common cause of disease after C9orf72 expansion. A lower signal was observed for TARDBP, proving that its effect on our cohort is driven by a few known causal variants. We detected rare variants in other known ALS genes that did not surpass statistical significance in gene-wise tests, thus highlighting that their contribution to disease risk in our cohort is limited.ConclusionsWe identified potential disease-causing variants in 11.9% of our patients. We identified the genes most frequently involved in our cohort and confirmed the contribution of rare variants in disease risk. Our results provide further insight into the pathologic mechanism of the disease and demonstrate the importance of genome-wide sequencing as a diagnostic tool.

Published
2020

19. ALS phenotype is influenced by age, sex, and genetics

Author

Jean Pierre Zucchetti, Andrea Calvo, Adriano Chiò, Maurizio Grassano, Rosario Vasta, Laura Peotta, Letizia Mazzini, Antonio Canosa, Maria Francesca Sarnelli, Maura Brunetti, Lucia Corrado, Barbara Iazzolino, Sandra D'Alfonso, Valentina Solara, Gabriele Mora, Cristina Moglia, Umberto Manera, Fabiola De Marchi, Marco Barberis, and Fabrizio D'Ovidio

Subjects
Male, 0301 basic medicine, Motor Disorders, Population, Comorbidity, 03 medical and health sciences, Sex Factors, Superoxide Dismutase-1, 0302 clinical medicine, Humans, Medicine, Dementia, Cognitive Dysfunction, Amyotrophic lateral sclerosis, education, Aged, Genetics, education.field_of_study, C9orf72 Protein, business.industry, Upper motor neuron, Amyotrophic Lateral Sclerosis, Age Factors, Cognition, Middle Aged, medicine.disease, Phenotype, 030104 developmental biology, medicine.anatomical_structure, Italy, Frontotemporal Dementia, Mutation, Cohort, Population study, Female, Neurology (clinical), business, 030217 neurology & neurosurgery
Abstract

ObjectiveTo assess the determinants of amyotrophic lateral sclerosis (ALS) phenotypes in a population-based cohort.MethodsThe study population included 2,839 patients with ALS diagnosed in Piemonte, Italy (1995–2015). Patients were classified according to motor (classic, bulbar, flail arm, flail leg, predominantly upper motor neuron [PUMN], respiratory) and cognitive phenotypes (normal, ALS with cognitive impairment [ALSci], ALS with behavioral impairment [ALSbi], ALSci and ALSbi combined [ALScbi], ALS–frontotemporal dementia [FTD]). Binary logistic regression analysis was adjusted for sex, age, and genetics.ResultsBulbar phenotype correlated with older age (p < 0.0001), women were more affected than men at increasing age (p < 0.0001), classic with younger age (p = 0.029), men were more affected than women at increasing age (p < 0.0001), PUMN with younger age (p < 0.0001), flail arm with male sex (p < 0.0001) and younger age (p = 0.04), flail leg with male sex with increasing age (p = 0.008), and respiratory with male sex (p < 0.0001). C9orf72 expansions correlated with bulbar phenotype (p < 0.0001), and were less frequent in PUMN (p = 0.041); SOD1 mutations correlated with flail leg phenotype (p < 0.0001), and were less frequent in bulbar (p < 0.0001). ALS-FTD correlated with C9orf72 (p < 0.0001) and bulbar phenotype (p = 0.008), ALScbi with PUMN (p = 0.014), and ALSci with older age (p = 0.008).ConclusionsOur data suggest that the spatial–temporal combination of motor and cognitive events leading to the onset and progression of ALS is characterized by a differential susceptibility to the pathologic process of motor and prefrontal cortices and lower motor neurons, and is influenced by age, sex, and gene variants. The identification of those factors that regulate ALS phenotype will allow us to reclassify patients into pathologically homogenous subgroups, responsive to targeted personalized therapies.

Published
2020

22. Metabolic Brain Changes Across Different Levels Of Cognitive Impairment In ALS: A 18F-FDG-PET Study (4129)

Author

Canosa, Antonio, primary, Moglia, Cristina, additional, Manera, Umberto, additional, Vasta, Rosario, additional, Torrieri, Maria Claudia, additional, Arena, Vincenzo, additional, D’Ovidio, Fabrizio, additional, Palumbo, Francesca, additional, Zucchetti, Jean Pierre, additional, Iazzolino, Barbara, additional, Peotta, Laura, additional, Calvo, Andrea, additional, Pagani, Marco, additional, and Chio, Adriano, additional

Published
2021
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23. Differential Neuropsychological Profile of Patients With Amyotrophic Lateral Sclerosis With and Without

Author

Barbara, Iazzolino, Laura, Peotta, Jean Pierre, Zucchetti, Antonio, Canosa, Umberto, Manera, Rosario, Vasta, Maurizio, Grassano, Francesca, Palumbo, Maura, Brunetti, Marco, Barberis, Luca, Sbaiz, Cristina, Moglia, Andrea, Calvo, and Adriano, Chiò

Subjects
Male, C9orf72 Protein, Amyotrophic Lateral Sclerosis, Mutation, Humans, Female, Middle Aged, Neuropsychological Tests, Cognition Disorders
Abstract

To determine whether the neuropsychological profiles of patients with amyotrophic lateral sclerosis (ALS) with (ALSC9+) and without (ALSC9-)The study population includes 741 patients with ALS who were consecutively diagnosed at the Turin ALS expert center in the 2010-2018 period and who underwent both cognitive/behavioral and genetic testing. Patients' neuropsychological patterns were compared (1) at the same degree of cognitive and behavioral deficit according to the revised ALS-Frontotemporal Dementia Consensus Criteria and (2) at the same level of motor impairment according to the King staging system.Despite being about 7 years younger, ALSC9+ patients had significantly lower scores in tests exploring executive function and verbal memory both when classified as cognitively normal and when diagnosed in the intermediate cognitive categories. Considering the clinical perspective, ALSC9+ patients showed significantly lower scores compared to ALSC9- patients at King stage 1 and 3 in almost all the examined neuropsychological domains; at King stage 2, ALSC9+ patients were more severely affected only in the verbal memory domain. Behavioral function was comparably impaired in the 2 cohorts.ALSC9+ patients show a different neuropsychological profile compared to ALSC9- patients, being more impaired in executive functions and verbal memory domains at all King stages. Verbal memory emerged as a particularly vulnerable function in ALSC9+, with worse performances even when patients were still classified as cognitively normal.

Published
2020

24. Differential Neuropsychological Profile of Patients With Amyotrophic Lateral Sclerosis With and Without C9orf72 Mutation

Author

Iazzolino, Barbara, primary, Peotta, Laura, additional, Zucchetti, Jean Pierre, additional, Canosa, Antonio, additional, Manera, Umberto, additional, Vasta, Rosario, additional, Grassano, Maurizio, additional, Palumbo, Francesca, additional, Brunetti, Maura, additional, Barberis, Marco, additional, Sbaiz, Luca, additional, Moglia, Cristina, additional, Calvo, Andrea, additional, and Chiò, Adriano, additional

Published
2020
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28. Cognitive impairment across ALS clinical stages in a population-based cohort

Author

Maria Francesca Sarnelli, Barbara Iazzolino, Jean Pierre Zucchetti, Laura Peotta, Lucia Corrado, Antonio Canosa, Sandra D'Alfonso, Marco Barberis, Umberto Manera, Cristina Moglia, Andrea Calvo, Letizia Mazzini, Maura Brunetti, Gabriele Mora, Enrica Bersano, Rosario Vasta, Adriano Chiò, and Valentina Solara

Subjects
0301 basic medicine, cognition, Male, Pediatrics, medicine.medical_specialty, Cross-sectional study, Population, Neuropsychological Tests, Article, Cohort Studies, 03 medical and health sciences, 0302 clinical medicine, medicine, Dementia, Humans, Cognitive Dysfunction, Prospective Studies, Registries, Amyotrophic lateral sclerosis, education, Prospective cohort study, ALS, cognition, Aged, Aged, 80 and over, education.field_of_study, C9orf72 Protein, business.industry, Amyotrophic Lateral Sclerosis, Cognition, Middle Aged, medicine.disease, Cognitive test, 030104 developmental biology, Cross-Sectional Studies, Population Surveillance, Disease Progression, Female, Neurology (clinical), ALS, business, 030217 neurology & neurosurgery, Cohort study
Abstract

ObjectiveTo assess the association of the degree of severity of motor impairment to that of cognitive impairment in a large cohort of patients with amyotrophic lateral sclerosis (ALS).MethodsThis is a population-based cross-sectional study on patients with ALS incident in Piemonte, Italy, between 2007 and 2015. Cognitive status was classified according to the revised ALS–FTD Consensus Criteria. The King system and the Milano Torino Staging system (MiToS) were used for defining the severity of motor impairment.ResultsOf the 797 patients included in the study, 163 (20.5%) had ALS–frontotemporal dementia (FTD), 38 (4.8%) cognitive and behavioral impairment (ALScbi), 132 (16.6%) cognitive impairment (ALSci), 63 (7.9%) behavioral impairment (ALSbi), 16 (2.0%) nonexecutive impairment, and 385 (48.2%) were cognitively normal. According to King staging, the frequency of cases with ALS-FTD progressively increased from 16.5% in stage 1–44.4% in stage 4; conversely, the frequency of ALSci, ALSbi, and ALScbi increased from King stage 1 to King stage 3 and decreased thereafter. A similar pattern was observed with the MiToS staging. ALS-FTD was more frequent in patients with bulbar involvement at time of cognitive testing. Patients with C9ORF72 expansion (n = 61) showed more severe cognitive impairment with increasing King and MiToS stages.ConclusionOur findings suggest that ALS motor and cognitive components may worsen in parallel, and that cognitive impairment becomes more pronounced when bulbar function is involved. Our data support the hypothesis that ALS pathology disseminates in a regional ordered sequence, through a cortico-efferent spreading model.

Published
2019

29. Correlation of cognitive impairment and KING’s and MiTos staging in ALS: a crosssectional population-based study. (S54.007)

Author

Vasta, Rosario, primary, Pain, Debora, additional, Peotta, Laura, additional, Calvo, Andrea, additional, Moglia, Cristina, additional, Canosa, Antonio, additional, Manera, Umberto, additional, Zucchetti, Jean Pierre, additional, Mora, Gabriele, additional, Bersano, Enrica, additional, Sarnelli, Maria Francesca, additional, Solara, Valentina, additional, Mazzini, Letizia, additional, Iazzolino, Barbara, additional, and Chio, Adriano, additional

Published
2019
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33. Validation of the revised classification of cognitive and behavioral impairment in ALS (P1.4-008)

Author

Vasta, Rosario, primary, Pain, Debora, additional, Peotta, Laura, additional, Calvo, Andrea, additional, Moglia, Cristina, additional, Canosa, Antonio, additional, Manera, Umberto, additional, Ilardi, Antonio, additional, Bombaci, Alessandro, additional, Zucchetti, Jean Pierre, additional, Mora, Gabriele, additional, Iazzolino, Barbara, additional, and Chio, Adriano, additional

Published
2019
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34. The multistep hypothesis of ALS revisited: The role of genetic mutations

Author

Chiò, Adriano, Mazzini, Letizia, D'Alfonso, Sandra, Corrado, Lucia, Canosa, Antonio, Moglia, Cristina, Manera, Umberto, Bersano, Enrica, Brunetti, Maura, Barberis, Marco, Veldink, Jan H, van den Berg, Leonard H, Pearce, Neil, Sproviero, William, Mclaughlin, Russell, Vajda, Alice, Hardiman, Orla, Rooney, James, Mora, Gabriele, Calvo, Andrea, and Al-Chalabi, Ammar

Subjects
Adult, Male, C9orf72 Protein, Incidence, Amyotrophic Lateral Sclerosis, Middle Aged, Article, DNA-Binding Proteins, Superoxide Dismutase-1, Mutation, Humans, RNA-Binding Protein FUS, Female, Genetic Predisposition to Disease, Genetic Testing, Aged, Retrospective Studies
Abstract

OBJECTIVE: Amyotrophic lateral sclerosis (ALS) incidence rates are consistent with the hypothesis that ALS is a multistep process. We tested the hypothesis that carrying a large effect mutation might account for ≥1 steps through the effect of the mutation, thus leaving fewer remaining steps before ALS begins. METHODS: We generated incidence data from an ALS population register in Italy (2007-2015) for which genetic analysis for C9orf72, SOD1, TARDBP, and FUS genes was performed in 82% of incident cases. As confirmation, we used data from ALS cases diagnosed in the Republic of Ireland (2006-2014). We regressed the log of age-specific incidence against the log of age with least-squares regression for the subpopulation carrying disease-associated variation in each separate gene. RESULTS: Of the 1,077 genetically tested cases, 74 (6.9%) carried C9orf72 mutations, 20 (1.9%) had SOD1 mutations, 15 (1.4%) had TARDBP mutations, and 3 (0.3%) carried FUS mutations. In the whole population, there was a linear relationship between log incidence and log age (r2 = 0.98) with a slope estimate of 4.65 (4.37-4.95), consistent with a 6-step process. The analysis for C9orf72-mutated patients confirmed a linear relationship (r2 = 0.94) with a slope estimate of 2.22 (1.74-2.29), suggesting a 3-step process. This estimate was confirmed by data from the Irish ALS register. The slope estimate was consistent with a 2-step process for SOD1 and with a 4-step process for TARDBP. CONCLUSION: The identification of a reduced number of steps in patients with ALS with genetic mutations compared to those without mutations supports the idea of ALS as a multistep process and is an important advance for dissecting the pathogenic process in ALS.

Published
2017

37. Are ALS Motor Phenotypes stochastic? (P5.167)

Author

Chio, Adriano, primary, D’Ovidio, Fabrizio, additional, Mazzini, Letizia, additional, Moglia, Cristina, additional, Manera, Umberto, additional, Bersano, Enrica, additional, Canosa, Antonio, additional, Mora, Gabriele, additional, and Calvo, Andrea, additional

Published
2018
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38. Secular trends of ALS incidence in an Italian population-based register, 1995–2014: evidence for a cohort effect in women (S15.005)

Author

Chio, Adriano, primary, Moglia, Cristina, additional, Canosa, Antonio, additional, Bertuzzo, Davide, additional, Bersano, Enrica, additional, Cammarosano, Stefania, additional, Manera, Umberto, additional, Pisano, Fabrizio, additional, Mazzini, Letizia, additional, Mora, Gabriele, additional, and Calvo, Andrea, additional

Published
2017
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40. Extrapyramidal involvement in ALS: a prospective population-based study (P4.314)

Author

Chio, Adriano, primary, Calvo, Andrea, additional, Cammarosano, Stefania, additional, Dematteis, Francesca, additional, Artusi, Carlo Alberto, additional, Pagani, Marco, additional, Nobili, Flavio, additional, Cistaro, Angela, additional, Balma, Michele, additional, Montanaro, Elisa, additional, Iazzolino, Barbara, additional, Brunetti, Maura, additional, Canosa, Antonio, additional, Lopiano, Leonardo, additional, and Rizzone, Mario, additional

Published
2017
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43. ALS clinical trials: do enrolled patients accurately represent the ALS population?

Author

Chio', Adriano, Canosa, A, Gallo, S, Cammarosano, Stefania, Moglia, Cristina, Fuda, G, Calvo, Andrea, Mora, G, and Mauro, Alessandro

Subjects
Adult, Male, medicine.medical_specialty, Adolescent, Population, Disease-Free Survival, Cohort Studies, 03 medical and health sciences, Young Adult, 0302 clinical medicine, Internal medicine, Medicine, Humans, Multicenter Studies as Topic, Young adult, Amyotrophic lateral sclerosis, 10. No inequality, education, 030304 developmental biology, Aged, Randomized Controlled Trials as Topic, Retrospective Studies, 0303 health sciences, education.field_of_study, business.industry, Patient Selection, Amyotrophic Lateral Sclerosis, Retrospective cohort study, Middle Aged, medicine.disease, Confidence interval, 3. Good health, Clinical trial, Logistic Models, Italy, Cohort, Female, Neurology (clinical), business, 030217 neurology & neurosurgery, Cohort study
Abstract

To assess the effect of eligibility criteria in amyotrophic lateral sclerosis (ALS) clinical trials on the representativeness of the enrolled population.Patients enrolled in 8 placebo-controlled clinical trials in our ALS center from 2003 to 2008 were compared 1) to the patients included a prospective epidemiologic register (Piemonte and Valle d'Aosta register for ALS, PARALS) in the same period and 2) the subset of PARALS patients who met the usual criteria for inclusion in clinical trials (PARALS-ct) (definite, probable, probable laboratory-supported ALS; age between 18 and 75 years; disease duration36 months; vital capacity at diagnosis ≥70%; score ≥3 at the items swallowing and respiratory insufficiency at the Amyotrophic Lateral Sclerosis Functional Rating Scale-revised scale; riluzole therapy).A total of 164 patients were enrolled in 8 different clinical trials. The PARALS cohort included 813 patients, of whom 539 (66.3%) met the entry criteria for clinical trials. Patients enrolled in clinical trials were different from both epidemiologic cohorts, since they were younger, had a longer diagnostic delay, and were more likely to have a spinal onset, and to be men. Tracheostomy-free survival was significantly longer in the group of patients enrolled in clinical trials (median survival time, trial patients, 3.9 years [95% confidence interval (CI) 3.4-4.4]; PARALS, 2.6 [2.4-2.8]; PARALS-ct, 2.9 [2.7-3.1]).Patients enrolled in clinical trials do not satisfactorily represent the ALS population; consequently, the findings of ALS trials lack of external validity (generalizability). Efforts should be made to improve patients' recruitment in trials, particularly enrolling incident rather than prevalent cases.

Published
2011

44. 18F-FDG-PET correlates of cognitive impairment in ALS

Author

Canosa, Antonio, primary, Pagani, Marco, additional, Cistaro, Angelina, additional, Montuschi, Anna, additional, Iazzolino, Barbara, additional, Fania, Piercarlo, additional, Cammarosano, Stefania, additional, Ilardi, Antonio, additional, Moglia, Cristina, additional, Calvo, Andrea, additional, and Chiò, Adriano, additional

Published
2015
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46. Are arterial hypertension and diabetes modifiers of ALS phenotype and outcome? A population-based study (P6.101)

Author

Calvo, Andrea, primary, Moglia, Cristina, additional, Canosa, Antonio, additional, Bertuzzo, Davide, additional, Galmozzi, Francesco, additional, Cugnasco, Paolo, additional, Clerico, Marinella, additional, De Mercanti, Stefania, additional, Bersano, Enrica, additional, Cammarosano, Stefania, additional, Ilardi, Antonio, additional, Manera, Umberto, additional, Sideri, Riccardo, additional, Marinou, Kalliopi, additional, Bottacchi, Edo, additional, Pisano, Fabrizio, additional, Cantello, Roberto, additional, Mazzini, Letizia, additional, Mora, Gabriele, additional, and Chio, Adriano, additional

Published
2015
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47. ATXN2 PolyQ Intermediate Repeat Modifies ALS Phenotype and Survival (I8-3B)

Author

Chio, Adriano, primary, Calvo, Andrea, additional, Moglia, Cristina, additional, Canosa, Antonio, additional, Restagno, Gabriella, additional, Brunetti, Maura, additional, Barberis, Marco, additional, Conte, Amelia, additional, Marangi, Giuseppe, additional, Lattante, Serena, additional, Zollino, Marcella, additional, Sabatelli, Mario, additional, Corrado, Lucia, additional, Mora, Gabriele, additional, Mazzini, Letizia, additional, and D'Alfonso, Sandra, additional

Published
2015
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48. ATXN2 PolyQ Intermediate Repeat Modifies ALS Phenotype and Survival (P2.005)

Author

Chio, Adriano, primary, Calvo, Andrea, additional, Moglia, Cristina, additional, Canosa, Antonio, additional, Restagno, Gabriella, additional, Brunetti, Maura, additional, Barberis, Marco, additional, Conte, Amelia, additional, Marangi, Giuseppe, additional, Lattante, Serena, additional, Zollino, Marcella, additional, Sabatelli, Mario, additional, Corrado, Lucia, additional, Mora, Gabriele, additional, Mazzini, Letizia, additional, and D'Alfonso, Sandra, additional

Published
2015
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50. Cognitive Reserve in ALS with Comorbid Frontotemporal Dementia (FTD): A Population-Based Study (P5.081)

Author

Canosa, Antonio, primary, Montuschi, Anna, additional, Iazzolino, Barbara, additional, Calvo, Andrea, additional, Moglia, Cristina, additional, Bertuzzo, Davide, additional, Lopiano, Leonardo, additional, Restagno, Gabriella, additional, Brunetti, Maura, additional, Ossola, Irene, additional, Lo Presti, Anna, additional, Cammarosano, Stefania, additional, and Chio, Adriano, additional

Published
2014
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