A population based study to analyse amyotrophic lateral sclerosis as a multi-step process
Abstract Recent studies suggest that Amyotrophic Lateral Sclerosis (ALS) follows a multistep process. We evaluated this hypothesis in a well-defined ALS population in Palermo, Sicily, almost entirely followed by our ALS Clinical Center. Incident data from the ALS Center (2014–2023) were analyzed, in...
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Nature Portfolio
2025-04-01
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| Series: | Scientific Reports |
| Online Access: | https://doi.org/10.1038/s41598-025-89616-7 |
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| author | Anna D’Amico Roberta Cucunato Giuseppe Salemi Vincenzo La Bella Paolo Aridon |
| author_facet | Anna D’Amico Roberta Cucunato Giuseppe Salemi Vincenzo La Bella Paolo Aridon |
| author_sort | Anna D’Amico |
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| description | Abstract Recent studies suggest that Amyotrophic Lateral Sclerosis (ALS) follows a multistep process. We evaluated this hypothesis in a well-defined ALS population in Palermo, Sicily, almost entirely followed by our ALS Clinical Center. Incident data from the ALS Center (2014–2023) were analyzed, including both sporadic and familial ALS forms of the disease. To evaluate the multistep process, we regressed the natural log of age-specific incidence against the natural log of patient age We identified 216 ALS patients. We obtained a slope of 5 (r2 = 0.93); the 95% CI ranged from 2.51 to 7.60, remaining relatively wide due to the small sample size, with a p-value of 0.008. The slope estimate was consistent with a 6-step process. In the Palermo ALS population, the multistep analysis confirms a process consistent with a 6-step model. This data, obtained in a relatively homogeneous population, further highlights the probability of strict interaction between environmental and genetic variables in the disease. Our data offer insights into the complexity of the mechanisms involved in the pathogenesis of the disease, particularly during its asymptomatic phase. This study supports the hypothesis that a single therapeutic silver bullet would probably be insufficient to arrest or slow the disease’s progression. |
| format | Article |
| id | doaj-art-d35df74643214b15bb1f427cefaa782a |
| institution | OA Journals |
| issn | 2045-2322 |
| language | English |
| publishDate | 2025-04-01 |
| publisher | Nature Portfolio |
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| series | Scientific Reports |
| spelling | doaj-art-d35df74643214b15bb1f427cefaa782a2025-08-20T01:56:01ZengNature PortfolioScientific Reports2045-23222025-04-011511810.1038/s41598-025-89616-7A population based study to analyse amyotrophic lateral sclerosis as a multi-step processAnna D’Amico0Roberta Cucunato1Giuseppe Salemi2Vincenzo La Bella3Paolo Aridon4Department of Biomedicine, Neuroscience and Advanced Diagnostics (BiND), University of PalermoDepartment of Biomedicine, Neuroscience and Advanced Diagnostics (BiND), University of PalermoDepartment of Biomedicine, Neuroscience and Advanced Diagnostics (BiND), University of PalermoDepartment of Biomedicine, Neuroscience and Advanced Diagnostics (BiND), University of PalermoDepartment of Biomedicine, Neuroscience and Advanced Diagnostics (BiND), University of PalermoAbstract Recent studies suggest that Amyotrophic Lateral Sclerosis (ALS) follows a multistep process. We evaluated this hypothesis in a well-defined ALS population in Palermo, Sicily, almost entirely followed by our ALS Clinical Center. Incident data from the ALS Center (2014–2023) were analyzed, including both sporadic and familial ALS forms of the disease. To evaluate the multistep process, we regressed the natural log of age-specific incidence against the natural log of patient age We identified 216 ALS patients. We obtained a slope of 5 (r2 = 0.93); the 95% CI ranged from 2.51 to 7.60, remaining relatively wide due to the small sample size, with a p-value of 0.008. The slope estimate was consistent with a 6-step process. In the Palermo ALS population, the multistep analysis confirms a process consistent with a 6-step model. This data, obtained in a relatively homogeneous population, further highlights the probability of strict interaction between environmental and genetic variables in the disease. Our data offer insights into the complexity of the mechanisms involved in the pathogenesis of the disease, particularly during its asymptomatic phase. This study supports the hypothesis that a single therapeutic silver bullet would probably be insufficient to arrest or slow the disease’s progression.https://doi.org/10.1038/s41598-025-89616-7 |
| spellingShingle | Anna D’Amico Roberta Cucunato Giuseppe Salemi Vincenzo La Bella Paolo Aridon A population based study to analyse amyotrophic lateral sclerosis as a multi-step process Scientific Reports |
| title | A population based study to analyse amyotrophic lateral sclerosis as a multi-step process |
| title_full | A population based study to analyse amyotrophic lateral sclerosis as a multi-step process |
| title_fullStr | A population based study to analyse amyotrophic lateral sclerosis as a multi-step process |
| title_full_unstemmed | A population based study to analyse amyotrophic lateral sclerosis as a multi-step process |
| title_short | A population based study to analyse amyotrophic lateral sclerosis as a multi-step process |
| title_sort | population based study to analyse amyotrophic lateral sclerosis as a multi step process |
| url | https://doi.org/10.1038/s41598-025-89616-7 |
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