Comorbid argyrophilic grain disease in an 87-year-old male with spinocerebellar ataxia type 31 with dementia: a case report

BACKGROUND: Spinocerebellar ataxia type 31 (SCA31) is not usually associated with dementia, and autopsy in a patient with both conditions is very rare. CASE PRESENTATION: An 87-year-old male patient presented with ataxia and progressive dementia. Genetic testing led to a diagnosis of SCA31. Fifteen...

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Autores principales: Toru, Shuta, Ishida, Shoko, Uchihara, Toshiki, Hirokawa, Katsuiku, Kitagawa, Masanobu, Ishikawa, Kinya
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2020
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7158122/
https://www.ncbi.nlm.nih.gov/pubmed/32293309
http://dx.doi.org/10.1186/s12883-020-01723-2
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author Toru, Shuta
Ishida, Shoko
Uchihara, Toshiki
Hirokawa, Katsuiku
Kitagawa, Masanobu
Ishikawa, Kinya
author_facet Toru, Shuta
Ishida, Shoko
Uchihara, Toshiki
Hirokawa, Katsuiku
Kitagawa, Masanobu
Ishikawa, Kinya
author_sort Toru, Shuta
collection PubMed
description BACKGROUND: Spinocerebellar ataxia type 31 (SCA31) is not usually associated with dementia, and autopsy in a patient with both conditions is very rare. CASE PRESENTATION: An 87-year-old male patient presented with ataxia and progressive dementia. Genetic testing led to a diagnosis of SCA31. Fifteen years after his initial symptoms of hearing loss and difficulty walking, he died of aspiration pneumonia. A pathological analysis showed cerebellar degeneration consistent with SCA31 and abundant argyrophilic grains in the hippocampal formation and amygdala that could explain his dementia. CONCLUSIONS: This is the first autopsy report on comorbid argyrophilic grain disease with SCA31.
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spelling pubmed-71581222020-04-21 Comorbid argyrophilic grain disease in an 87-year-old male with spinocerebellar ataxia type 31 with dementia: a case report Toru, Shuta Ishida, Shoko Uchihara, Toshiki Hirokawa, Katsuiku Kitagawa, Masanobu Ishikawa, Kinya BMC Neurol Case Report BACKGROUND: Spinocerebellar ataxia type 31 (SCA31) is not usually associated with dementia, and autopsy in a patient with both conditions is very rare. CASE PRESENTATION: An 87-year-old male patient presented with ataxia and progressive dementia. Genetic testing led to a diagnosis of SCA31. Fifteen years after his initial symptoms of hearing loss and difficulty walking, he died of aspiration pneumonia. A pathological analysis showed cerebellar degeneration consistent with SCA31 and abundant argyrophilic grains in the hippocampal formation and amygdala that could explain his dementia. CONCLUSIONS: This is the first autopsy report on comorbid argyrophilic grain disease with SCA31. BioMed Central 2020-04-15 /pmc/articles/PMC7158122/ /pubmed/32293309 http://dx.doi.org/10.1186/s12883-020-01723-2 Text en © The Author(s) 2020 Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.
spellingShingle Case Report
Toru, Shuta
Ishida, Shoko
Uchihara, Toshiki
Hirokawa, Katsuiku
Kitagawa, Masanobu
Ishikawa, Kinya
Comorbid argyrophilic grain disease in an 87-year-old male with spinocerebellar ataxia type 31 with dementia: a case report
title Comorbid argyrophilic grain disease in an 87-year-old male with spinocerebellar ataxia type 31 with dementia: a case report
title_full Comorbid argyrophilic grain disease in an 87-year-old male with spinocerebellar ataxia type 31 with dementia: a case report
title_fullStr Comorbid argyrophilic grain disease in an 87-year-old male with spinocerebellar ataxia type 31 with dementia: a case report
title_full_unstemmed Comorbid argyrophilic grain disease in an 87-year-old male with spinocerebellar ataxia type 31 with dementia: a case report
title_short Comorbid argyrophilic grain disease in an 87-year-old male with spinocerebellar ataxia type 31 with dementia: a case report
title_sort comorbid argyrophilic grain disease in an 87-year-old male with spinocerebellar ataxia type 31 with dementia: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7158122/
https://www.ncbi.nlm.nih.gov/pubmed/32293309
http://dx.doi.org/10.1186/s12883-020-01723-2
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