Different patterns of spreading direction and motor neurons involvement in a cohort of limb‐onset amyotrophic lateral sclerosis patients from Southern Italy: Potential implication on disease course or progression?
Abstract Background Currently, there is a lack of knowledge concerning where the pathological process starts and how the neurodegeneration spreads during the course of amyotrophic lateral sclerosis (ALS). Aims This study aims to evaluate the spreading direction of the disease and the corresponding c...
| Main Authors: | , , , , , , , |
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| Format: | Article |
| Language: | English |
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Wiley
2023-06-01
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| Series: | Brain and Behavior |
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| Online Access: | https://doi.org/10.1002/brb3.2899 |
| _version_ | 1827924004927700992 |
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| author | Giammarco Milella Stefano Zoccolella Daniele Urso Salvatore Nigro Ludovica Tamburrino Valentina Gnoni Marco Filardi Giancarlo Logroscino |
| author_facet | Giammarco Milella Stefano Zoccolella Daniele Urso Salvatore Nigro Ludovica Tamburrino Valentina Gnoni Marco Filardi Giancarlo Logroscino |
| author_sort | Giammarco Milella |
| collection | DOAJ |
| description | Abstract Background Currently, there is a lack of knowledge concerning where the pathological process starts and how the neurodegeneration spreads during the course of amyotrophic lateral sclerosis (ALS). Aims This study aims to evaluate the spreading direction of the disease and the corresponding clinical characteristics in a cohort of patients with limb‐onset ALS. Patients and methods Consecutive incident ALS patients referring to an ALS tertiary center from Southern Italy, between 2015 and 2021, were recruited in the study. According to the initial directions of spread, patients were dichotomized into horizontal spreading pattern (HSP) or vertical spreading pattern (VSP) groups. Results Among 137 newly diagnosed ALS, 87 presented a spinal onset. Ten patients with pure LMN were not included in the study. All cases reported a clear spread direction. The frequency of HSP and VSP spreading was similar overall (47 vs. 30). The prevalence of HSP was higher (74% vs. 50%) in patients with upper limb‐onset (UL‐ALS), compared to patients with lower limb‐onset (LL‐ALS; p < .05). Conversely, the occurrence of VSP spread was threefold higher in patients with LL‐ALS, compared to UL‐ALS (p < .05). Patients with VSP showed a wider upper motor neuron impairment, whereas the involvement of LMN resulted greater in patients with HSP. Patients with HSP exhibited a greater drop of ALSFRS‐r sub‐score in the region of onset, while VSP showed a slighter but more diffuse reduction of ASLFRS‐r subscore in more body districts beyond the site of onset. Patients with VSP were also characterized by a higher median progression rate and an earlier median bulbar involvement, compared to HSP. Conclusions Our findings suggested investigating the spreading direction of ALS among patients with spinal onset, to better delineate the clinical profiles of patients with ALS, and predict an earlier impairment of bulbar muscle and a more rapid progression of the disease |
| first_indexed | 2024-03-13T05:05:22Z |
| format | Article |
| id | doaj.art-f391a01cad7148cc96949e788259940f |
| institution | Directory Open Access Journal |
| issn | 2162-3279 |
| language | English |
| last_indexed | 2024-03-13T05:05:22Z |
| publishDate | 2023-06-01 |
| publisher | Wiley |
| record_format | Article |
| series | Brain and Behavior |
| spelling | doaj.art-f391a01cad7148cc96949e788259940f2023-06-16T18:11:55ZengWileyBrain and Behavior2162-32792023-06-01136n/an/a10.1002/brb3.2899Different patterns of spreading direction and motor neurons involvement in a cohort of limb‐onset amyotrophic lateral sclerosis patients from Southern Italy: Potential implication on disease course or progression?Giammarco Milella0Stefano Zoccolella1Daniele Urso2Salvatore Nigro3Ludovica Tamburrino4Valentina Gnoni5Marco Filardi6Giancarlo Logroscino7Department of Basic Medicine, Neuroscience and Sense Organs University of Bari Aldo Moro Bari ItalyASL Bari San Paolo Hospital, Neurology Unit Milan ItalyCenter for Neurodegenerative Diseases and the Aging Brain University of Bari Aldo Moro at Pia Fondazione “Card. G. Panico,” Tricase ItalyCenter for Neurodegenerative Diseases and the Aging Brain University of Bari Aldo Moro at Pia Fondazione “Card. G. Panico,” Tricase ItalyCenter for Neurodegenerative Diseases and the Aging Brain University of Bari Aldo Moro at Pia Fondazione “Card. G. Panico,” Tricase ItalyCenter for Neurodegenerative Diseases and the Aging Brain University of Bari Aldo Moro at Pia Fondazione “Card. G. Panico,” Tricase ItalyDepartment of Basic Medicine, Neuroscience and Sense Organs University of Bari Aldo Moro Bari ItalyDepartment of Basic Medicine, Neuroscience and Sense Organs University of Bari Aldo Moro Bari ItalyAbstract Background Currently, there is a lack of knowledge concerning where the pathological process starts and how the neurodegeneration spreads during the course of amyotrophic lateral sclerosis (ALS). Aims This study aims to evaluate the spreading direction of the disease and the corresponding clinical characteristics in a cohort of patients with limb‐onset ALS. Patients and methods Consecutive incident ALS patients referring to an ALS tertiary center from Southern Italy, between 2015 and 2021, were recruited in the study. According to the initial directions of spread, patients were dichotomized into horizontal spreading pattern (HSP) or vertical spreading pattern (VSP) groups. Results Among 137 newly diagnosed ALS, 87 presented a spinal onset. Ten patients with pure LMN were not included in the study. All cases reported a clear spread direction. The frequency of HSP and VSP spreading was similar overall (47 vs. 30). The prevalence of HSP was higher (74% vs. 50%) in patients with upper limb‐onset (UL‐ALS), compared to patients with lower limb‐onset (LL‐ALS; p < .05). Conversely, the occurrence of VSP spread was threefold higher in patients with LL‐ALS, compared to UL‐ALS (p < .05). Patients with VSP showed a wider upper motor neuron impairment, whereas the involvement of LMN resulted greater in patients with HSP. Patients with HSP exhibited a greater drop of ALSFRS‐r sub‐score in the region of onset, while VSP showed a slighter but more diffuse reduction of ASLFRS‐r subscore in more body districts beyond the site of onset. Patients with VSP were also characterized by a higher median progression rate and an earlier median bulbar involvement, compared to HSP. Conclusions Our findings suggested investigating the spreading direction of ALS among patients with spinal onset, to better delineate the clinical profiles of patients with ALS, and predict an earlier impairment of bulbar muscle and a more rapid progression of the diseasehttps://doi.org/10.1002/brb3.2899amyotrophic lateral sclerosisprognosticspreading directions |
| spellingShingle | Giammarco Milella Stefano Zoccolella Daniele Urso Salvatore Nigro Ludovica Tamburrino Valentina Gnoni Marco Filardi Giancarlo Logroscino Different patterns of spreading direction and motor neurons involvement in a cohort of limb‐onset amyotrophic lateral sclerosis patients from Southern Italy: Potential implication on disease course or progression? Brain and Behavior amyotrophic lateral sclerosis prognostic spreading directions |
| title | Different patterns of spreading direction and motor neurons involvement in a cohort of limb‐onset amyotrophic lateral sclerosis patients from Southern Italy: Potential implication on disease course or progression? |
| title_full | Different patterns of spreading direction and motor neurons involvement in a cohort of limb‐onset amyotrophic lateral sclerosis patients from Southern Italy: Potential implication on disease course or progression? |
| title_fullStr | Different patterns of spreading direction and motor neurons involvement in a cohort of limb‐onset amyotrophic lateral sclerosis patients from Southern Italy: Potential implication on disease course or progression? |
| title_full_unstemmed | Different patterns of spreading direction and motor neurons involvement in a cohort of limb‐onset amyotrophic lateral sclerosis patients from Southern Italy: Potential implication on disease course or progression? |
| title_short | Different patterns of spreading direction and motor neurons involvement in a cohort of limb‐onset amyotrophic lateral sclerosis patients from Southern Italy: Potential implication on disease course or progression? |
| title_sort | different patterns of spreading direction and motor neurons involvement in a cohort of limb onset amyotrophic lateral sclerosis patients from southern italy potential implication on disease course or progression |
| topic | amyotrophic lateral sclerosis prognostic spreading directions |
| url | https://doi.org/10.1002/brb3.2899 |
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