研究者詳細 - 橋詰　淳

論文 - 橋詰　淳

分割表示 172 件中 141 - 160 件目／全件表示 >>

リュープロレリン酢酸塩の球脊髄性筋萎縮症患者に対する第3相長期継続投与試験(JASMITT-07OP試験) 査読有り

鈴木啓介, 勝野雅央, 坂野晴彦, 須賀徳明, 橋詰淳, 矢部一郎, 青木正志, 中野今治, 金井数明, 水澤英洋, 山本知孝, 長谷川一子, 西澤正豊, 宮嶋裕明, 苅田典生, 中島健二, 辻野彰, 内野誠, 田中章景, 祖父江元

臨床神経学 52 巻 ( 12 ) 頁： 1484 - 1484 2012年12月

嚥下造影3回法の信頼性・妥当性の検討 SBMAを例に査読有り

坂野晴彦, 勝野雅央, 鈴木啓介, 須賀徳明, 橋詰淳, 眞野智生, 田中誠也, 山本正彦, 祖父江元

臨床神経学 52 巻 ( 12 ) 頁： 1516 - 1516 2012年12月

球脊髄性筋萎縮症患者におけるリュープロレリン酢酸塩長期使用成績定量的筋力解析を用いた検討査読有り

須賀徳明, 勝野雅久, 坂野晴彦, 鈴木啓介, 橋詰淳, 眞野智生, 祖父江元

臨床神経学 52 巻 ( 12 ) 頁： 1604 - 1604 2012年12月

球脊髄性筋萎縮症の自然歴の検討(3年間の前向き研究) 査読有り

橋詰淳, 勝野雅央, 坂野晴彦, 鈴木啓介, 須賀徳明, 眞野智生, 田中章景, 祖父江元

臨床神経学 52 巻 ( 12 ) 頁： 1514 - 1514 2012年12月

球脊髄性筋萎縮症に対する疾患特異的機能評価スケール(SBMAFRS)の開発と信頼性・妥当性評価査読有り

真野智生, 勝野雅央, 坂野晴彦, 鈴木啓介, 須賀徳明, 橋詰淳, 田中章景, 祖父江元

臨床神経学 52 巻 ( 12 ) 頁： 1604 - 1604 2012年12月

球脊髄性筋萎縮症における発声障害の定量的検討査読有り

田中誠也, 坂野晴彦, 勝野雅央, 鈴木啓介, 眞野智生, 須賀徳明, 橋詰淳, 山本正彦, 祖父江元

臨床神経学 52 巻 ( 12 ) 頁： 1514 - 1514 2012年12月

Cross-sectional and longitudinal analysis of an oxidative stress biomarker for spinal and bulbar muscular atrophy 査読有り国際誌

Mano, T; Katsuno, M; Banno, H; Suzuki, K; Suga, N; Hashizume, A; Tanaka, F; Sobue, G

MUSCLE & NERVE 46 巻 ( 5 ) 頁： 692 - 7 2012年11月

記述言語：英語掲載種別：研究論文（学術雑誌）出版者・発行元：Muscle and Nerve

Introduction:: Spinal and bulbar muscular atrophy (SBMA) is an adult-onset motor neuron disease caused by a CAG repeat expansion in the androgen receptor gene. The aim of this study was to verify whether urinary 8-hydroxydeoxyguanosine (8-OHdG), an oxidative stress marker, is a biomarker for SBMA. Methods:: We measured the levels of urinary 8-OHdG in 33 genetically confirmed SBMA patients and 32 age-matched controls over a 24-month period at 6-month intervals. Results:: Urinary 8-OHdG levels in SBMA patients were significantly elevated compared with those of controls and correlated well with motor function scores. During the follow-up period, urinary 8-OHdG levels increased and correlated with motor function at each time-point. In addition, urinary 8-OHdG levels at baseline were correlated with changes in the 6-minute walk test during 24 months. Conclusions:: Urinary 8-OHdG is a biomarker for SBMA, reflecting the severity and possibly predicting the deterioration of motor function. © 2012 Wiley Periodicals, Inc.

DOI： 10.1002/mus.23413

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PubMed

1）神経変性疾患のトランスレーショナルリサーチ査読有り

勝野雅央, 坂野晴彦, 鈴木啓介, 橋詰淳, 足立弘明, 田中章景, 祖父江元

日本内科学会雑誌 101 巻 ( 9 ) 頁： 2533 - 8 2012年9月

Longitudinal changes of outcome measures in spinal and bulbar muscular atrophy 査読有り国際誌 Open Access

Hashizume, A; Katsuno, M; Banno, H; Suzuki, K; Suga, N; Mano, T; Atsuta, N; Oe, H; Watanabe, H; Tanaka, F; Sobue, G

BRAIN 135 巻 ( Pt 9 ) 頁： 2838 - 48 2012年9月

記述言語：英語掲載種別：研究論文（学術雑誌）出版者・発行元：Brain

Spinal and bulbar muscular atrophy is an adult-onset, hereditary motor neuron disease caused by the expansion of a trinucleotide CAG repeat within the gene encoding the androgen receptor. To date, several agents have been shown to prevent or slow disease progression in animal models of this disease. For the translational research of these agents, it is necessary to perform the detailed analysis of natural history with quantitative outcome measures and to establish sensitive and validated disease-specific endpoints in the clinical trials. To this end, we performed a prospective observation of disease progression over 3 years in 34 genetically confirmed Japanese patients with spinal and bulbar muscular atrophy by using quantitative outcome measures, including functional and blood parameters. The baseline evaluation revealed that CAG repeat length in the androgen receptor gene correlated not only with the age of onset but also with the timing of substantial changes in activity of daily living. Multiple regression analyses indicated that the serum level of creatinine is the most useful blood parameter that reflects the severity of motor dysfunction in spinal and bulbar muscular atrophy. In 3-year prospective analyses, a slow but steady progression was affirmed in most of the outcome measures we examined. In the analyses using random coefficient models that summarize the individual data into a representative line, disease progression was not affected by CAG repeat length or onset age. These models showed large interindividual variation, which was also independent of the differences of CAG repeat size. Analyses using these models also demonstrated that the subtle neurological deficits at an early or preclinical stage were more likely to be detected by objective motor functional tests such as the 6-min walk test and grip power or serum creatinine levels than by functional rating scales, such as the revised amyotrophic lateral sclerosis functional rating scale or modified Norris scale. Categorization of the clinical phenotypes using factor analysis showed that upper limb function is closely related to bulbar function, but not to lower limb function at baseline, whereas the site of onset had no substantial effects on disease progression. These results suggest that patients with spinal and bulbar muscular atrophy show a slow but steady progression of motor dysfunction over time that is independent of CAG repeat length or clinical phenotype, and that objective outcome measures may be used to evaluate disease severity at an early stage of this disease. © 2012 The Author. Published by Oxford University Press on behalf of the Guarantors of Brain. All rights reserved.

DOI： 10.1093/brain/aws170

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知的障害・小脳失調を認め、アキレス腱肥厚から診断しえた脳腱黄色腫症の治療経験査読有り

桝田道人, 橋詰淳, 伊藤瑞規, 熱田直樹, 渡辺宏久, 祖父江元

神経治療学 29 巻 ( 5 ) 頁： 659 - 659 2012年9月

Difference in chronological changes of outcome measures between untreated and placebo-treated patients of spinal and bulbar muscular atrophy 査読有り国際誌

Hashizume, A; Katsuno, M; Banno, H; Suzuki, K; Suga, N; Tanaka, F; Sobue, G

JOURNAL OF NEUROLOGY 259 巻 ( 4 ) 頁： 712 - 9 2012年4月

記述言語：英語掲載種別：研究論文（学術雑誌）出版者・発行元：Journal of Neurology

Spinal and bulbar muscular atrophy (SBMA) is an adult-onset, X-linked motor neuron disease characterized by muscle atrophy, weakness, and bulbar involvement. The aim of this study was to analyze the differential change of various outcome measures by comparing the progression of motor impairment in the two independent groups: placebotreated group (PTG) and natural history group (NHG). For the PTG, we analyzed 99 patients who participated in a previous double-blind phase III clinical trial and received placebo. For the NHG, a total of 34 patients were followed with no specific treatment. The characteristics of both groups did not differ at baseline except for disease duration. Although the 6 min walk distance (6MWD) showed almost the same progression in both groups (-14.7 ± 7.3 m in NHG, -14.0 ± 4.7 m in PTG; NS), there was a significant difference of progression in the ALSFRS-R between the NHG and PTG (-1.18 ± 0.38, -0.14 ± 0.24; p = 0.03). A similar tendency was also seen in the subgroup analysis of the patients whose disease durations were less than 10 years. Although the relationship between the ALSFRSR and 6MWD at week 48 was similar to that at baseline in the NHG, the slope of the regression at week 48 was significantly milder than at baseline in the PTG (p = 0.04). In conclusion, these two groups demonstrated a large difference in the chronological analysis of a motor function score, but showed similar changes in objective measures of walking capacity. These findings should be thoroughly considered when designing clinical trials for slowly progressive neurodegenerative diseases such as SBMA. © Springer-Verlag 2011.

DOI： 10.1007/s00415-011-6251-2

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水頭症術後に眼球運動障害とパーキンソニズムを呈し、中脳水道開窓術が有効であった中脳水道狭窄症の2例査読有り

桝田道人, 橋詰淳, 伊藤瑞規, 熱田直樹, 渡辺宏久, 祖父江元, 種村衣里子, 永谷哲也

臨床神経学 52 巻 ( 4 ) 頁： 273 - 273 2012年4月

球脊髄性筋萎縮症（SBMA）に対する抗アンドロゲン療法査読有り Open Access

勝野雅央, 坂野晴彦, 鈴木啓介, 橋詰淳, 足立弘明, 田中章景, 祖父江元

臨床神経学 52 巻 ( 11 ) 頁： 1207 - 9 2012年

記述言語：日本語掲載種別：研究論文（学術雑誌）出版者・発行元：日本神経学会

球脊髄性筋萎縮症（SBMA）はアンドロゲン受容体（AR）遺伝子におけるCAGくりかえし塩基配列の異常延長を原因とする運動ニューロン疾患であり，伸長ポリグルタミン鎖を有する異常AR蛋白質がテストステロンと結合することによってニューロンの核内に蓄積することが病態の本質と考えられている．テストステロンの分泌を抑制するリュープロレリン酢酸塩のSBMAに対する第III相臨床試験では，主要評価項目である咽頭部バリウム残留率について，被験者全体の解析では統計学的有意差はみとめられなかったが，罹病期間が10年以内の被験者のみを対象としたサブ解析では残留率が酢酸リュープロレリン群で有意に低下したことから，発症からの期間が薬効に影響をおよぼすことが示唆された．早期の治療介入や鋭敏なエンドポイントの開発が今後のトランスレーショナルリサーチに重要であると考えられる．<br>

DOI： 10.5692/clinicalneurol.52.1207

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CiNii Research

1）神経変性疾患のトランスレーショナルリサーチ

勝野雅央, 坂野晴彦, 鈴木啓介, 橋詰淳, 足立弘明, 田中章景, 祖父江元

日本内科学会雑誌 101 巻 ( Suppl ) 頁： 113a - 113a 2012年