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Histopathological Features of Gerhardt Syndrome in a Patient With Multiple System Atrophy: An Autopsy Case Report

Multiple system atrophy (MSA) is a progressive neurodegenerative disease characterized by autonomic failure, parkinsonism, and cerebellar ataxia. Gerhardt syndrome, which is inspiratory dyspnea with laryngeal stridor associated with dysfunction of the vocal folds, is a frequent and fatal complicatio...

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Autores principales: Nakahara, Keiichi, Takamatsu, Koutaro, Kudo, Noritaka, Ito, Takaaki, Ueda, Mitsuharu
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9669818/
https://www.ncbi.nlm.nih.gov/pubmed/36407156
http://dx.doi.org/10.7759/cureus.30415
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author Nakahara, Keiichi
Takamatsu, Koutaro
Kudo, Noritaka
Ito, Takaaki
Ueda, Mitsuharu
author_facet Nakahara, Keiichi
Takamatsu, Koutaro
Kudo, Noritaka
Ito, Takaaki
Ueda, Mitsuharu
author_sort Nakahara, Keiichi
collection PubMed
description Multiple system atrophy (MSA) is a progressive neurodegenerative disease characterized by autonomic failure, parkinsonism, and cerebellar ataxia. Gerhardt syndrome, which is inspiratory dyspnea with laryngeal stridor associated with dysfunction of the vocal folds, is a frequent and fatal complication of MSA. A 59-year-old man with a six-year history of MSA presented with ataxia and dysarthria. He also had dyspnea and stridor, which had worsened in the last three months, and died from respiratory distress. Autopsy revealed neurogenic group atrophy of the posterior cricoarytenoid (PCA) muscle, which suggested that laryngeal nerve damage caused abductor vocal fold paralysis in addition to cerebellar and brainstem atrophy with glial cytoplasmic inclusions. Our histopathological findings suggest that Gerhardt syndrome may be associated with neurogenic atrophy of the laryngeal abductor muscle (PCA muscle) of the vocal folds.
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spelling pubmed-96698182022-11-17 Histopathological Features of Gerhardt Syndrome in a Patient With Multiple System Atrophy: An Autopsy Case Report Nakahara, Keiichi Takamatsu, Koutaro Kudo, Noritaka Ito, Takaaki Ueda, Mitsuharu Cureus Neurology Multiple system atrophy (MSA) is a progressive neurodegenerative disease characterized by autonomic failure, parkinsonism, and cerebellar ataxia. Gerhardt syndrome, which is inspiratory dyspnea with laryngeal stridor associated with dysfunction of the vocal folds, is a frequent and fatal complication of MSA. A 59-year-old man with a six-year history of MSA presented with ataxia and dysarthria. He also had dyspnea and stridor, which had worsened in the last three months, and died from respiratory distress. Autopsy revealed neurogenic group atrophy of the posterior cricoarytenoid (PCA) muscle, which suggested that laryngeal nerve damage caused abductor vocal fold paralysis in addition to cerebellar and brainstem atrophy with glial cytoplasmic inclusions. Our histopathological findings suggest that Gerhardt syndrome may be associated with neurogenic atrophy of the laryngeal abductor muscle (PCA muscle) of the vocal folds. Cureus 2022-10-18 /pmc/articles/PMC9669818/ /pubmed/36407156 http://dx.doi.org/10.7759/cureus.30415 Text en Copyright © 2022, Nakahara et al. https://creativecommons.org/licenses/by/3.0/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 author and source are credited.
spellingShingle Neurology
Nakahara, Keiichi
Takamatsu, Koutaro
Kudo, Noritaka
Ito, Takaaki
Ueda, Mitsuharu
Histopathological Features of Gerhardt Syndrome in a Patient With Multiple System Atrophy: An Autopsy Case Report
title Histopathological Features of Gerhardt Syndrome in a Patient With Multiple System Atrophy: An Autopsy Case Report
title_full Histopathological Features of Gerhardt Syndrome in a Patient With Multiple System Atrophy: An Autopsy Case Report
title_fullStr Histopathological Features of Gerhardt Syndrome in a Patient With Multiple System Atrophy: An Autopsy Case Report
title_full_unstemmed Histopathological Features of Gerhardt Syndrome in a Patient With Multiple System Atrophy: An Autopsy Case Report
title_short Histopathological Features of Gerhardt Syndrome in a Patient With Multiple System Atrophy: An Autopsy Case Report
title_sort histopathological features of gerhardt syndrome in a patient with multiple system atrophy: an autopsy case report
topic Neurology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9669818/
https://www.ncbi.nlm.nih.gov/pubmed/36407156
http://dx.doi.org/10.7759/cureus.30415
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