【博士論文】学術データベース

博士論文 / Clinicopathological characteristics of patients with amyotrophic lateral sclerosis resulting in a totally locked-in state (communication stage V) 完全閉じ込め状態(communication stage V)に至った筋萎縮性側索硬化症例の臨床病理学的特徴

著者

書誌事項

タイトル

Clinicopathological characteristics of patients with amyotrophic lateral sclerosis resulting in a totally locked-in state (communication stage V)

タイトル別名

完全閉じ込め状態(communication stage V)に至った筋萎縮性側索硬化症例の臨床病理学的特徴

著者名

Hayashi Kentaro

著者名

林健太郎

学位授与大学

新潟大学 (大学ID:0035) (CAT機関ID:KI000356)

取得学位

博士(医学)

学位授与番号

甲第4350号

学位授与年月日

2017-09-20

注記・抄録

学位の種類: 博士(医学). 報告番号: 甲第4350号. 学位記番号: 新大院博(医)甲第763号. 学位授与年月日: 平成29年9月20日

Acta Neuropathologica Communications. 2016, 4, 107.

In the present study, we performed a comprehensive analysis to clarify the clinicopathological characteristics of patients with amyotrophic lateral sclerosis (ALS) that had progressed to result in a totally locked-in state (communication Stage V), in which all voluntary movements are lost and communication is impossible. In 11 patients, six had phosphorylated TAR DNA-binding protein 43 (pTDP-43)-immunoreactive (ir) neuronal cytoplasmic inclusions (NCI), two had fused in sarcoma (FUS)-ir NCI, and three had copper/zinc superoxide dismutase (SOD1)-ir NCI. The time from ALS onset to the need for tracheostomy invasive ventilation was less than 24 months in ten patients. Regardless of accumulated protein, all the patients showed common lesions in the pallido–nigro–luysian system, brainstem reticular formation, and cerebellar efferent system, in addition to motor neurons. In patients with pTDP-43-ir NCI, patients with NCI in the hippocampal dentate granule neurons (DG) showed a neuronal loss in the cerebral cortex, and patients without NCI in DG showed a preserved cerebral cortex. By contrast, in patients with FUS-ir NCI, patients with NCI in DG showed a preserved cerebral cortex and patients without NCI in DG showed marked cerebral degeneration. The cerebral cortex of patients with SOD1-ir NCI was preserved. Together, these findings suggest that lesions of the cerebrum are probably not necessary for progression to Stage V. In conclusion, patients with ALS that had progressed to result in communication Stage V showed rapidly-progressed symptoms, and their common lesions could cause the manifestations of communication Stage V.

元資料の権利情報 : (C) 2016 The Author(s). 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.

キーワード

Amyotrophic lateral sclerosis, Communication Stage V, Totally locked-in state, Pallido-nigro-luysian system, Brainstem reticular formation, Cerebral cortical degeneration

各種コード

NII論文ID(NAID)

500001044156

NII著者ID(NRID)
  • 8000001153887
  • 8000001153888
DOI (出版社)

10.1186/s40478-016-0379-3

DOI

info:doi/10.1186/s40478-016-0379-3

本文言語コード

eng

データ提供元

機関リポジトリ / NDLデジタルコレクション

外部リンク

DOI

博士論文 / 新潟大学 / 医学

博士論文 / 新潟大学

博士論文 / 医学

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