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Spreading in ALS: The relative impact of upper and lower motor neuron involvement
OBJECTIVE: To investigate disease spread in amyotrophic lateral sclerosis (ALS), and determine the influence of lower (LMN) and upper motor neuron (UMN) involvement. METHODS: We assessed disease spread in ALS in 1376 consecutively studied patients, from five European centers, applying an agreed prof...
Autores principales: | , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
John Wiley and Sons Inc.
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7359118/ https://www.ncbi.nlm.nih.gov/pubmed/32558369 http://dx.doi.org/10.1002/acn3.51098 |
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author | Gromicho, Marta Figueiral, Manuel Uysal, Hilmi Grosskreutz, Julian Kuzma‐Kozakiewicz, Magdalena Pinto, Susana Petri, Susanne Madeira, Sara Swash, Michael de Carvalho, Mamede |
author_facet | Gromicho, Marta Figueiral, Manuel Uysal, Hilmi Grosskreutz, Julian Kuzma‐Kozakiewicz, Magdalena Pinto, Susana Petri, Susanne Madeira, Sara Swash, Michael de Carvalho, Mamede |
author_sort | Gromicho, Marta |
collection | PubMed |
description | OBJECTIVE: To investigate disease spread in amyotrophic lateral sclerosis (ALS), and determine the influence of lower (LMN) and upper motor neuron (UMN) involvement. METHODS: We assessed disease spread in ALS in 1376 consecutively studied patients, from five European centers, applying an agreed proforma to assess LMN and UMN signs. We defined the pattern of disease onset and progression from predominant UMN or lower motor neuron (LMN) dysfunction in bulbar, upper limbs, lower limbs, and thoracic regions Non‐linear regression analysis was applied to fit the data to a model that described the relation between two random variables, graphically represented by an inverse exponential curve. We analyzed the probability, rate of spread, and both combined (area under the curve). RESULTS: We found that progression was more likely and quicker to or from the region of onset to close spinal regions. When the disease had a limb onset, bulbar motor neurons were more resistant. Furthermore, in the same time frame more patients progressed from bulbar to lower limbs than vice‐versa, whether predominantly UMN or LMN involvement. Patients with initial thoracic involvement had a higher probability for rapid change. The presence of predominant UMN signs was associated with a faster caudal progression. INTERPRETATION: Contiguous progression was leading pattern, and predominant UMN involvement is important in shortening the time for cranial‐caudal spread. Our results can best be fitted to a model of independent LMN and UMN degeneration, with regional progression of LMN degeneration mostly by contiguity. UMN lesion causes an acceleration of rostral‐caudal LMN loss. |
format | Online Article Text |
id | pubmed-7359118 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | John Wiley and Sons Inc. |
record_format | MEDLINE/PubMed |
spelling | pubmed-73591182020-07-17 Spreading in ALS: The relative impact of upper and lower motor neuron involvement Gromicho, Marta Figueiral, Manuel Uysal, Hilmi Grosskreutz, Julian Kuzma‐Kozakiewicz, Magdalena Pinto, Susana Petri, Susanne Madeira, Sara Swash, Michael de Carvalho, Mamede Ann Clin Transl Neurol Research Articles OBJECTIVE: To investigate disease spread in amyotrophic lateral sclerosis (ALS), and determine the influence of lower (LMN) and upper motor neuron (UMN) involvement. METHODS: We assessed disease spread in ALS in 1376 consecutively studied patients, from five European centers, applying an agreed proforma to assess LMN and UMN signs. We defined the pattern of disease onset and progression from predominant UMN or lower motor neuron (LMN) dysfunction in bulbar, upper limbs, lower limbs, and thoracic regions Non‐linear regression analysis was applied to fit the data to a model that described the relation between two random variables, graphically represented by an inverse exponential curve. We analyzed the probability, rate of spread, and both combined (area under the curve). RESULTS: We found that progression was more likely and quicker to or from the region of onset to close spinal regions. When the disease had a limb onset, bulbar motor neurons were more resistant. Furthermore, in the same time frame more patients progressed from bulbar to lower limbs than vice‐versa, whether predominantly UMN or LMN involvement. Patients with initial thoracic involvement had a higher probability for rapid change. The presence of predominant UMN signs was associated with a faster caudal progression. INTERPRETATION: Contiguous progression was leading pattern, and predominant UMN involvement is important in shortening the time for cranial‐caudal spread. Our results can best be fitted to a model of independent LMN and UMN degeneration, with regional progression of LMN degeneration mostly by contiguity. UMN lesion causes an acceleration of rostral‐caudal LMN loss. John Wiley and Sons Inc. 2020-06-18 /pmc/articles/PMC7359118/ /pubmed/32558369 http://dx.doi.org/10.1002/acn3.51098 Text en © 2020 The Authors. Annals of Clinical and Translational Neurology published by Wiley Periodicals LLC on behalf of American Neurological Association This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc-nd/4.0/ License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made. |
spellingShingle | Research Articles Gromicho, Marta Figueiral, Manuel Uysal, Hilmi Grosskreutz, Julian Kuzma‐Kozakiewicz, Magdalena Pinto, Susana Petri, Susanne Madeira, Sara Swash, Michael de Carvalho, Mamede Spreading in ALS: The relative impact of upper and lower motor neuron involvement |
title | Spreading in ALS: The relative impact of upper and lower motor neuron involvement |
title_full | Spreading in ALS: The relative impact of upper and lower motor neuron involvement |
title_fullStr | Spreading in ALS: The relative impact of upper and lower motor neuron involvement |
title_full_unstemmed | Spreading in ALS: The relative impact of upper and lower motor neuron involvement |
title_short | Spreading in ALS: The relative impact of upper and lower motor neuron involvement |
title_sort | spreading in als: the relative impact of upper and lower motor neuron involvement |
topic | Research Articles |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7359118/ https://www.ncbi.nlm.nih.gov/pubmed/32558369 http://dx.doi.org/10.1002/acn3.51098 |
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