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Frontal and Cerebellar Atrophy Supports FTSD-ALS Clinical Continuum
BACKGROUND: Frontotemporal Spectrum Disorder (FTSD) and Amyotrophic Lateral Sclerosis (ALS) are neurodegenerative diseases often considered as a continuum from clinical, epidemiologic, and genetic perspectives. We used localized brain volume alterations to evaluate common and specific features of FT...
Autores principales: | , , , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Frontiers Media S.A.
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7726473/ https://www.ncbi.nlm.nih.gov/pubmed/33324193 http://dx.doi.org/10.3389/fnagi.2020.593526 |
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author | Pizzarotti, Beatrice Palesi, Fulvia Vitali, Paolo Castellazzi, Gloria Anzalone, Nicoletta Alvisi, Elena Martinelli, Daniele Bernini, Sara Cotta Ramusino, Matteo Ceroni, Mauro Micieli, Giuseppe Sinforiani, Elena D’Angelo, Egidio Costa, Alfredo Gandini Wheeler-Kingshott, Claudia A. M. |
author_facet | Pizzarotti, Beatrice Palesi, Fulvia Vitali, Paolo Castellazzi, Gloria Anzalone, Nicoletta Alvisi, Elena Martinelli, Daniele Bernini, Sara Cotta Ramusino, Matteo Ceroni, Mauro Micieli, Giuseppe Sinforiani, Elena D’Angelo, Egidio Costa, Alfredo Gandini Wheeler-Kingshott, Claudia A. M. |
author_sort | Pizzarotti, Beatrice |
collection | PubMed |
description | BACKGROUND: Frontotemporal Spectrum Disorder (FTSD) and Amyotrophic Lateral Sclerosis (ALS) are neurodegenerative diseases often considered as a continuum from clinical, epidemiologic, and genetic perspectives. We used localized brain volume alterations to evaluate common and specific features of FTSD, FTSD-ALS, and ALS patients to further understand this clinical continuum. METHODS: We used voxel-based morphometry on structural magnetic resonance images to localize volume alterations in group comparisons: patients (20 FTSD, seven FTSD-ALS, and 18 ALS) versus healthy controls (39 CTR), and patient groups between themselves. We used mean whole-brain cortical thickness ([Formula: see text]) to assess whether its correlations with local brain volume could propose mechanistic explanations of the heterogeneous clinical presentations. We also assessed whether volume reduction can explain cognitive impairment, measured with frontal assessment battery, verbal fluency, and semantic fluency. RESULTS: Common (mainly frontal) and specific areas with reduced volume were detected between FTSD, FTSD-ALS, and ALS patients, confirming suggestions of a clinical continuum, while at the same time defining morphological specificities for each clinical group (e.g., a difference of cerebral and cerebellar involvement between FTSD and ALS). [Formula: see text] values suggested extensive network disruption in the pathological process, with indications of a correlation between cerebral and cerebellar volumes and [Formula: see text] in ALS. The analysis of the neuropsychological scores indeed pointed toward an important role for the cerebellum, along with fronto-temporal areas, in explaining impairment of executive, and linguistic functions. CONCLUSION: We identified common elements that explain the FTSD-ALS clinical continuum, while also identifying specificities of each group, partially explained by different cerebral and cerebellar involvement. |
format | Online Article Text |
id | pubmed-7726473 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
spelling | pubmed-77264732020-12-14 Frontal and Cerebellar Atrophy Supports FTSD-ALS Clinical Continuum Pizzarotti, Beatrice Palesi, Fulvia Vitali, Paolo Castellazzi, Gloria Anzalone, Nicoletta Alvisi, Elena Martinelli, Daniele Bernini, Sara Cotta Ramusino, Matteo Ceroni, Mauro Micieli, Giuseppe Sinforiani, Elena D’Angelo, Egidio Costa, Alfredo Gandini Wheeler-Kingshott, Claudia A. M. Front Aging Neurosci Neuroscience BACKGROUND: Frontotemporal Spectrum Disorder (FTSD) and Amyotrophic Lateral Sclerosis (ALS) are neurodegenerative diseases often considered as a continuum from clinical, epidemiologic, and genetic perspectives. We used localized brain volume alterations to evaluate common and specific features of FTSD, FTSD-ALS, and ALS patients to further understand this clinical continuum. METHODS: We used voxel-based morphometry on structural magnetic resonance images to localize volume alterations in group comparisons: patients (20 FTSD, seven FTSD-ALS, and 18 ALS) versus healthy controls (39 CTR), and patient groups between themselves. We used mean whole-brain cortical thickness ([Formula: see text]) to assess whether its correlations with local brain volume could propose mechanistic explanations of the heterogeneous clinical presentations. We also assessed whether volume reduction can explain cognitive impairment, measured with frontal assessment battery, verbal fluency, and semantic fluency. RESULTS: Common (mainly frontal) and specific areas with reduced volume were detected between FTSD, FTSD-ALS, and ALS patients, confirming suggestions of a clinical continuum, while at the same time defining morphological specificities for each clinical group (e.g., a difference of cerebral and cerebellar involvement between FTSD and ALS). [Formula: see text] values suggested extensive network disruption in the pathological process, with indications of a correlation between cerebral and cerebellar volumes and [Formula: see text] in ALS. The analysis of the neuropsychological scores indeed pointed toward an important role for the cerebellum, along with fronto-temporal areas, in explaining impairment of executive, and linguistic functions. CONCLUSION: We identified common elements that explain the FTSD-ALS clinical continuum, while also identifying specificities of each group, partially explained by different cerebral and cerebellar involvement. Frontiers Media S.A. 2020-11-26 /pmc/articles/PMC7726473/ /pubmed/33324193 http://dx.doi.org/10.3389/fnagi.2020.593526 Text en Copyright © 2020 Pizzarotti, Palesi, Vitali, Castellazzi, Anzalone, Alvisi, Martinelli, Bernini, Cotta Ramusino, Ceroni, Micieli, Sinforiani, D’Angelo, Costa and Gandini Wheeler-Kingshott. http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. |
spellingShingle | Neuroscience Pizzarotti, Beatrice Palesi, Fulvia Vitali, Paolo Castellazzi, Gloria Anzalone, Nicoletta Alvisi, Elena Martinelli, Daniele Bernini, Sara Cotta Ramusino, Matteo Ceroni, Mauro Micieli, Giuseppe Sinforiani, Elena D’Angelo, Egidio Costa, Alfredo Gandini Wheeler-Kingshott, Claudia A. M. Frontal and Cerebellar Atrophy Supports FTSD-ALS Clinical Continuum |
title | Frontal and Cerebellar Atrophy Supports FTSD-ALS Clinical Continuum |
title_full | Frontal and Cerebellar Atrophy Supports FTSD-ALS Clinical Continuum |
title_fullStr | Frontal and Cerebellar Atrophy Supports FTSD-ALS Clinical Continuum |
title_full_unstemmed | Frontal and Cerebellar Atrophy Supports FTSD-ALS Clinical Continuum |
title_short | Frontal and Cerebellar Atrophy Supports FTSD-ALS Clinical Continuum |
title_sort | frontal and cerebellar atrophy supports ftsd-als clinical continuum |
topic | Neuroscience |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7726473/ https://www.ncbi.nlm.nih.gov/pubmed/33324193 http://dx.doi.org/10.3389/fnagi.2020.593526 |
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