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Dystrophic muscle distribution in late-stage muscular dystrophy

There is scant information about the comprehensive distribution of dystrophic muscles in muscular dystrophy. Despite different clinical presentations of muscular dystrophy, a recent multi-center study concluded that phenotypic distribution of dystrophic muscles is independent of clinical phenotype a...

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Autores principales: Pilato, Celeste Michelle, Walker, Melissa Sue, Nguyen, Andrea M., Hanna, McKay Elizabeth, Huang, Scott Lanxing, Lutins, Erika Morgan, Meredith, M. Alex, Haar, Peter Jacob, Thangarajh, Mathula, Richard, Hope Theresa, Chow, Woon Nam
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hospital Universitário da Universidade de São Paulo 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7703009/
https://www.ncbi.nlm.nih.gov/pubmed/33344329
http://dx.doi.org/10.4322/acr.2020.221
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author Pilato, Celeste Michelle
Walker, Melissa Sue
Nguyen, Andrea M.
Hanna, McKay Elizabeth
Huang, Scott Lanxing
Lutins, Erika Morgan
Meredith, M. Alex
Haar, Peter Jacob
Thangarajh, Mathula
Richard, Hope Theresa
Chow, Woon Nam
author_facet Pilato, Celeste Michelle
Walker, Melissa Sue
Nguyen, Andrea M.
Hanna, McKay Elizabeth
Huang, Scott Lanxing
Lutins, Erika Morgan
Meredith, M. Alex
Haar, Peter Jacob
Thangarajh, Mathula
Richard, Hope Theresa
Chow, Woon Nam
author_sort Pilato, Celeste Michelle
collection PubMed
description There is scant information about the comprehensive distribution of dystrophic muscles in muscular dystrophy. Despite different clinical presentations of muscular dystrophy, a recent multi-center study concluded that phenotypic distribution of dystrophic muscles is independent of clinical phenotype and suggested that there is a common pattern of involved muscles. To evaluate this possibility, the present case report used cadaveric dissection to determine the whole-body distribution of fat-infiltrated, dystrophic muscles from a 72-year-old white male cadaver with adult-onset, late-stage muscular dystrophy. Severely dystrophic muscles occupied the pectoral, gluteal and pelvic regions, as well as the arm, thigh and posterior leg. In contrast, muscles of the head, neck, hands and feet largely appeared unaffected. Histopathology and a CT-scan supported these observations. This pattern of dystrophic muscles generally conformed with that described in the multi-center study, and provides prognostic insight for patients and the physicians treating them.
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spelling pubmed-77030092020-12-18 Dystrophic muscle distribution in late-stage muscular dystrophy Pilato, Celeste Michelle Walker, Melissa Sue Nguyen, Andrea M. Hanna, McKay Elizabeth Huang, Scott Lanxing Lutins, Erika Morgan Meredith, M. Alex Haar, Peter Jacob Thangarajh, Mathula Richard, Hope Theresa Chow, Woon Nam Autops Case Rep Original Article There is scant information about the comprehensive distribution of dystrophic muscles in muscular dystrophy. Despite different clinical presentations of muscular dystrophy, a recent multi-center study concluded that phenotypic distribution of dystrophic muscles is independent of clinical phenotype and suggested that there is a common pattern of involved muscles. To evaluate this possibility, the present case report used cadaveric dissection to determine the whole-body distribution of fat-infiltrated, dystrophic muscles from a 72-year-old white male cadaver with adult-onset, late-stage muscular dystrophy. Severely dystrophic muscles occupied the pectoral, gluteal and pelvic regions, as well as the arm, thigh and posterior leg. In contrast, muscles of the head, neck, hands and feet largely appeared unaffected. Histopathology and a CT-scan supported these observations. This pattern of dystrophic muscles generally conformed with that described in the multi-center study, and provides prognostic insight for patients and the physicians treating them. Hospital Universitário da Universidade de São Paulo 2020-11-20 /pmc/articles/PMC7703009/ /pubmed/33344329 http://dx.doi.org/10.4322/acr.2020.221 Text en Copyright: © 2020 The Authors. https://creativecommons.org/licenses/by/4.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Original Article
Pilato, Celeste Michelle
Walker, Melissa Sue
Nguyen, Andrea M.
Hanna, McKay Elizabeth
Huang, Scott Lanxing
Lutins, Erika Morgan
Meredith, M. Alex
Haar, Peter Jacob
Thangarajh, Mathula
Richard, Hope Theresa
Chow, Woon Nam
Dystrophic muscle distribution in late-stage muscular dystrophy
title Dystrophic muscle distribution in late-stage muscular dystrophy
title_full Dystrophic muscle distribution in late-stage muscular dystrophy
title_fullStr Dystrophic muscle distribution in late-stage muscular dystrophy
title_full_unstemmed Dystrophic muscle distribution in late-stage muscular dystrophy
title_short Dystrophic muscle distribution in late-stage muscular dystrophy
title_sort dystrophic muscle distribution in late-stage muscular dystrophy
topic Original Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7703009/
https://www.ncbi.nlm.nih.gov/pubmed/33344329
http://dx.doi.org/10.4322/acr.2020.221
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