| Item type |
文献 / Documents(1) |
| 公開日 |
2020-03-26 |
| アクセス権 |
|
|
アクセス権 |
open access |
| 資源タイプ |
|
|
資源タイプ識別子 |
http://purl.org/coar/resource_type/c_6501 |
|
資源タイプ |
journal article |
| 出版社版DOI |
|
|
|
識別子タイプ |
DOI |
|
|
関連識別子 |
https://doi.org/10.1186/s12883-019-1402-7 |
|
|
言語 |
ja |
|
|
関連名称 |
10.1186/s12883-019-1402-7 |
| 出版タイプ |
|
|
出版タイプ |
VoR |
|
出版タイプResource |
http://purl.org/coar/version/c_970fb48d4fbd8a85 |
| タイトル |
|
|
タイトル |
Co-morbidity of progressive supranuclear palsy and amyotrophic lateral sclerosis : a clinical-pathological case report |
|
言語 |
en |
| 著者 |
藤田, 浩司
マツバラ, トモヤス
宮本, 亮介
スミクラ, ヒロユキ
タケウチ, トシアキ
マルヤマ, ケイコ
瓦井, 俊孝
野寺, 裕之
ウダカ, フカシ
クメ, コウダイ
森野, 豊之
カワカミ, ヒデシ
ハセガワ, マサト
梶, 龍兒
ムラヤマ, シゲオ
和泉, 唯信
|
| 抄録 |
|
|
内容記述タイプ |
Abstract |
|
内容記述 |
Background: The coexistence of distinct neurodegenerative diseases in single cases has recently attracted greater attention. The phenotypic co-occurrence of progressive supranuclear palsy (PSP) and amyotrophic lateral sclerosis (ALS) has been documented in several cases. That said, the clinicopathological comorbidity of these two diseases has not been demonstrated.
Case presentation: A 77-year-old man presented with gait disturbance for 2 years, consistent with PSP with progressive gait freezing. At 79 years old, he developed muscle weakness compatible with ALS. The disease duration was 5 years after the onset of PSP and 5months after the onset of ALS. Neuropathological findings demonstrated the coexistence of PSP and ALS. Immunohistochemical examination confirmed 4-repeat tauopathy, including globose-type neurofibrillary tangles, tufted astrocytes, and oligodendroglial coiled bodies as well as TAR DNA-binding protein 43 kDa pathology in association with upper and lower motor neuron degeneration. Immunoblotting showed hyperphosphorylated full-length 4-repeat tau bands (64 and 68 kDa) and C-terminal fragments (33 kDa), supporting the diagnosis of PSP and excluding other parkinsonian disorders, such as corticobasal degeneration. Genetic studies showed no abnormalities in genes currently known to be related to ALS or PSP.
Conclusions: Our case demonstrates the clinicopathological comorbidity of PSP and ALS in a sporadic patient. The possibility of multiple proteinopathies should be considered when distinct symptoms develop during the disease course. |
|
言語 |
en |
| キーワード |
|
|
言語 |
en |
|
主題Scheme |
Other |
|
主題 |
Amyotrophic lateral sclerosis |
| キーワード |
|
|
言語 |
en |
|
主題Scheme |
Other |
|
主題 |
Progressive supranuclear palsy |
| キーワード |
|
|
言語 |
en |
|
主題Scheme |
Other |
|
主題 |
TAR DNA-binding protein 43 kDa (TDP-43) |
| キーワード |
|
|
言語 |
en |
|
主題Scheme |
Other |
|
主題 |
Tau |
| キーワード |
|
|
言語 |
en |
|
主題Scheme |
Other |
|
主題 |
Copathology |
| 書誌情報 |
en : BMC Neurology
巻 19,
p. 168,
発行日 2019-07-18
|
| 収録物ID |
|
|
収録物識別子タイプ |
ISSN |
|
収録物識別子 |
14712377 |
| 収録物ID |
|
|
収録物識別子タイプ |
NCID |
|
収録物識別子 |
AA12035325 |
| 出版者 |
|
|
出版者 |
Springer Nature |
|
言語 |
en |
| 出版者 |
|
|
出版者 |
BioMed Central |
|
言語 |
en |
| 権利情報 |
|
|
言語 |
en |
|
権利情報 |
© The Author(s). 2019 Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. 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. |
| EID |
|
|
識別子 |
356565 |
|
識別子タイプ |
URI |
| 言語 |
|
|
言語 |
eng |
BMCNeuro_19_168.pdf (1.54 MB)
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