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  1. C100 医学部/医学系研究科
  2. C100b 紀要
  3. Nagoya journal of medical science
  4. 83(3)

A case of sporadic late-onset nemaline myopathy with monoclonal gammopathy of undetermined significance : long-term observation of neurological symptoms after autologous stem-cell transplantation

https://doi.org/10.18999/nagjms.83.3.641
https://doi.org/10.18999/nagjms.83.3.641
928c96d2-7452-4412-85ea-01a839fc1a46
名前 / ファイル ライセンス アクション
19_Ando.pdf 19_Ando.pdf (6.2 MB)
Item type itemtype_ver1(1)
公開日 2021-09-16
タイトル
タイトル A case of sporadic late-onset nemaline myopathy with monoclonal gammopathy of undetermined significance : long-term observation of neurological symptoms after autologous stem-cell transplantation
言語 en
著者 Ando, Takashi

× Ando, Takashi

en Ando, Takashi

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Sato, Takahiko

× Sato, Takahiko

en Sato, Takahiko

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Kurahashi, Shingo

× Kurahashi, Shingo

en Kurahashi, Shingo

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Kawaguchi, Yuka

× Kawaguchi, Yuka

en Kawaguchi, Yuka

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Kagaya, Yusuke

× Kagaya, Yusuke

en Kagaya, Yusuke

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Ozawa, Yukiyasu

× Ozawa, Yukiyasu

en Ozawa, Yukiyasu

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Hirano, Satoko

× Hirano, Satoko

en Hirano, Satoko

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Goto, Yoji

× Goto, Yoji

en Goto, Yoji

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Mano, Kazuo

× Mano, Kazuo

en Mano, Kazuo

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Yokoi, Satoshi

× Yokoi, Satoshi

en Yokoi, Satoshi

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Nakamura, Tomohiko

× Nakamura, Tomohiko

en Nakamura, Tomohiko

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Murakami, Ayuka

× Murakami, Ayuka

en Murakami, Ayuka

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Noda, Seiya

× Noda, Seiya

en Noda, Seiya

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Kimura, Seigo

× Kimura, Seigo

en Kimura, Seigo

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Sone, Jun

× Sone, Jun

en Sone, Jun

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Kuru, Satoshi

× Kuru, Satoshi

en Kuru, Satoshi

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Sobue, Gen

× Sobue, Gen

en Sobue, Gen

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Katsuno, Masahisa

× Katsuno, Masahisa

en Katsuno, Masahisa

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アクセス権
アクセス権 open access
アクセス権URI http://purl.org/coar/access_right/c_abf2
キーワード
主題Scheme Other
主題 sporadic late-onset nemaline myopathy
キーワード
主題Scheme Other
主題 monoclonal gammopathy
キーワード
主題Scheme Other
主題 intravenous immunoglobulin
キーワード
主題Scheme Other
主題 autologous stem-cell transplantation
内容記述
内容記述 A 47-year-old woman presented with progressive limb weakness. A neurological examination revealed proximal dominant symmetrical muscle weakness in her limbs, and electromyography revealed complex repetitive discharges and short motor unit potentials with positive sharp waves in the biceps. We observed early recruitment in the quadriceps, and laboratory tests revealed normal creatine kinase. Serum protein electrophoresis showed monoclonal IgG-lambda, but the bone marrow aspiration specimen was normal. A muscle biopsy revealed nemaline rod accumulations in the muscle fibers; based on the results, we diagnosed the patient with sporadic late-onset nemaline myopathy with monoclonal gammopathy of undetermined significance (SLONM-MGUS). We administered repeated intravenous immunoglobulin, but her limb weakness continued, and she developed a restrictive ventilatory defect. The patient received melphalan, followed by autologous stem-cell transplantation (ASCT). Her upper extremity strength and respiratory capability improved within one year after ASCT; however, it was not until six years after ASCT that her atrophied lower extremities strengthened. A discrepancy in the timeline of treatment response between the upper or respiratory muscles and the atrophied lower limb was characteristic in the patient, suggesting that the efficacy of ASCT on SLONM-MGUS should be evaluated in the long term, especially in severely atrophied muscles. In addition, this case showed that ASCT for SLOMN-MGUS is an effective treatment option in Asian populations.
言語 en
内容記述タイプ Abstract
内容記述
内容記述 This is an Open Access article distributed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License. To view the details of this license, please visit (http://creativecommons.org/licenses/by-nc-nd/4.0/).
言語 en
内容記述タイプ Other
出版者
言語 en
出版者 Nagoya University Graduate School of Medicine, School of Medicine
言語
言語 eng
資源タイプ
資源タイプresource http://purl.org/coar/resource_type/c_6501
タイプ departmental bulletin paper
出版タイプ
出版タイプ VoR
出版タイプResource http://purl.org/coar/version/c_970fb48d4fbd8a85
ID登録
ID登録 10.18999/nagjms.83.3.641
ID登録タイプ JaLC
関連情報
関連タイプ isVersionOf
識別子タイプ URI
関連識別子 https://www.med.nagoya-u.ac.jp/medlib/nagoya_j_med_sci/833.html
収録物識別子
収録物識別子タイプ PISSN
収録物識別子 0027-7622
収録物識別子
収録物識別子タイプ EISSN
収録物識別子 2186-3326
書誌情報 en : Nagoya Journal of Medical Science

巻 83, 号 3, p. 641-647, 発行日 2021-08
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