| 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
Sato, Takahiko
Kurahashi, Shingo
Kawaguchi, Yuka
Kagaya, Yusuke
Ozawa, Yukiyasu
Hirano, Satoko
Goto, Yoji
Mano, Kazuo
Yokoi, Satoshi
Nakamura, Tomohiko
Murakami, Ayuka
Noda, Seiya
Kimura, Seigo
Sone, Jun
Kuru, Satoshi
Sobue, Gen
Katsuno, Masahisa
|
| 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 |
| 権利 |
|
|
言語 |
en |
|
権利情報Resource |
http://creativecommons.org/licenses/by-nc-nd/4.0/ |
|
権利情報 |
Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International |
| 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 |
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|
内容記述タイプ |
Abstract |
|
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 |
| 出版者 |
|
|
出版者 |
Nagoya University Graduate School of Medicine, School of Medicine |
|
言語 |
en |
| 言語 |
|
|
言語 |
eng |
| 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 |
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
|
19_Ando.pdf (6.2 MB)
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