| Item type |
文献 / Documents(1) |
| 公開日 |
2024-07-05 |
| アクセス権 |
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アクセス権 |
open access |
| 資源タイプ |
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資源タイプ識別子 |
http://purl.org/coar/resource_type/c_6501 |
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資源タイプ |
journal article |
| 出版社版DOI |
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|
識別子タイプ |
DOI |
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|
関連識別子 |
https://doi.org/10.1212/NXI.0000000000200165 |
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言語 |
ja |
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|
関連名称 |
10.1212/NXI.0000000000200165 |
| 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 |
Prevalence, Clinical Profiles, and Prognosis of Stiff-Person Syndrome in a Japanese Nationwide Survey |
|
言語 |
en |
| 著者 |
松井, 尚子
タナカ, ケイコ
イシダ, ミツヨ
ヤマモト, ヨウヘイ
マツバラ, ユリ
サイカ, レイコ
イイヅカ, タカヒロ
ナカムラ, コウシ
クリヤマ, ナガト
マツイ, マコト
有澤, 孝吉
ナカムラ, ヨシカズ
梶, 龍兒
クワバラ, サトシ
和泉, 唯信
|
| 抄録 |
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|
内容記述タイプ |
Abstract |
|
内容記述 |
Background and Objectives
To elucidate current epidemiologic, clinical, and immunologic profiles and treatments of stiff-person syndrome (SPS) in Japan.
Methods
A nationwide mail survey was conducted using an established method. Data processing sheets were sent to randomly selected departments of internal medicine, neurology, pediatrics, psychiatry, and neurosurgery in hospitals and clinics throughout Japan to identify patients with SPS who were seen between January 2015 and December 2017.
Results
Thirty cases were identified as glutamic acid decarboxylase 65 (GAD65)–positive SPS cases on the basis of detailed clinical data of 55 cases. Four patients had α1 subunit of glycine receptor (GlyR) antibodies, and 1 patient had both GAD65 and GlyR antibodies. The total estimated number of patients with GAD65-positive SPS was 140, and the estimated prevalence was 0.11 per 100,000 population. The median age at onset was 51 years (range, 26–83 years), and 23 (76%) were female. Of these, 70% had classic SPS, and 30% had stiff-limb syndrome. The median time from symptom onset to diagnosis was significantly longer in the high-titer GAD65 antibody group than in the low-titer group (13 months vs 2.5 months, p = 0.01). The median modified Rankin Scale (mRS) at baseline was 4, and the median mRS at the last follow-up was 2. Among the 29 GAD65-positive patients with ≥1 year follow-up, 7 received only symptomatic treatment, 9 underwent immunotherapy without long-term immunotherapy, and 13 received long-term immunotherapy such as oral prednisolone. The coexistence of type 1 diabetes mellitus and the lack of long-term immunotherapy were independent risk factors for poor outcome (mRS ≥3) in the GAD65-positive patients (odds ratio, 15.0; 95% CI 2.6–131.6; p = 0.001; odds ratio, 19.8; 95% CI 3.2–191.5; p = 0.001, respectively).
Discussion
This study provides the current epidemiologic and clinical status of SPS in Japan. The symptom onset to the diagnosis of SPS was longer in patients with high-titer GAD65 antibodies than in those with low-titer GAD65 antibodies. The outcome of patients with SPS was generally favorable, but more aggressive immunotherapies are necessary for GAD65-positive patients with SPS. |
|
言語 |
en |
| 書誌情報 |
en : Neurology : Neuroimmunology & Neuroinflammation
巻 10,
号 6,
p. e200165,
発行日 2023-09-22
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| 収録物ID |
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収録物識別子タイプ |
ISSN |
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収録物識別子 |
23327812 |
| 収録物ID |
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収録物識別子タイプ |
NCID |
|
収録物識別子 |
AA12724386 |
| 出版者 |
|
|
出版者 |
Wolters Kluwer |
|
言語 |
en |
| 出版者 |
|
|
出版者 |
American Academy of Neurology |
|
言語 |
en |
| 権利情報 |
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|
言語 |
en |
|
権利情報 |
This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives License 4.0 (CC BY-NC-ND), which permits downloading and sharing the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal. |
| EID |
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|
識別子 |
403698 |
|
識別子タイプ |
URI |
| 言語 |
|
|
言語 |
eng |
nnninflam_10_6_e200165.pdf (363 KB)
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