研究者詳細 - 橋詰　淳

論文 - 橋詰　淳

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Swallowing markers in spinal and bulbar muscular atrophy 査読有り国際誌 Open Access

Banno, H; Katsuno, M; Suzuki, K; Tanaka, S; Suga, N; Hashizume, A; Mano, T; Araki, A; Watanabe, H; Fujimoto, Y; Yamamoto, M; Sobue, G

ANNALS OF CLINICAL AND TRANSLATIONAL NEUROLOGY 4 巻 ( 8 ) 頁： 534 - 543 2017年8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：Annals of Clinical and Translational Neurology

Objective: We examined the characteristics of dysphagia in spinal and bulbar muscular atrophy, a hereditary neuromuscular disease causing weakness of limb, facial, and oropharyngeal muscles via a videofluoroscopic swallowing study, and investigated the plausibility of using these outcome measures for quantitative analysis. Methods: A videofluoroscopic swallowing study was performed on 111 consecutive patients with genetically confirmed spinal and bulbar muscular atrophy and 53 age- and sex-matched healthy controls. Swallowing of 3-mL liquid barium was analyzed by the Logemann's Videofluorographic Examination of Swallowing worksheet. Results: Of more than 40 radiographic findings, the most pertinent abnormal findings in patients with spinal and bulbar muscular atrophy, included vallecular residue after swallow (residue just behind the tongue base), nasal penetration, and insufficient tongue movement (P < 0.001 for each) compared with healthy controls. Quantitative analyses showed that pharyngeal residue after initial swallowing, oral residue after initial swallowing, multiple swallowing sessions, and the penetration–aspiration scale were significantly worse in these patients (P ≤ 0.005 for each) than in controls. In patients with spinal and bulbar muscular atrophy, laryngeal penetration was observed more frequently in those without subjective dysphagia. Interpretation: Dysphagia of spinal and bulbar muscular atrophy was characterized by impaired tongue movement in the oral phase and nasal penetration followed by pharyngeal residues, which resulted in multiple swallowing sessions and laryngeal penetration. Although major limitations of reproducibility and radiation exposure still exist with videofluoroscopy, pharyngeal residue after initial swallowing and the penetration–aspiration scale might serve as potential outcome measures in clinical studies.

DOI： 10.1002/acn3.425

Correlation of insulin resistance and motor function in spinal and bulbar muscular atrophy 査読有り国際誌 Open Access

Nakatsuji, H; Araki, A; Hashizume, A; Hijikata, Y; Yamada, S; Inagaki, T; Suzuki, K; Banno, H; Suga, N; Okada, Y; Ohyama, M; Nakagawa, T; Kishida, K; Funahashi, T; Shimomura, I; Okano, H; Katsuno, M; Sobue, G

JOURNAL OF NEUROLOGY 264 巻 ( 5 ) 頁： 839 - 847 2017年5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

This study aimed to evaluate various metabolic parameters in patients with spinal and bulbar muscular atrophy (SBMA), to investigate the association between those indices and disease severity, and to explore the underlying molecular pathogenesis. We compared the degree of obesity, metabolic parameters, and blood pressure in 55 genetically confirmed SBMA patients against those in 483 age- and sex-matched healthy control. In SBMA patients, we investigated the correlation between these factors and motor functional indices. SBMA patients had lower body mass index, blood glucose, and Hemoglobin A1c, but higher blood pressure, homeostasis model assessment of insulin resistance (HOMA-IR, a marker of insulin resistance), total cholesterol, and adiponectin levels than the control subjects. There were no differences in visceral fat areas, high-density lipoprotein-cholesterol (HDL-C), or triglyceride levels in two groups. Revised amyotrophic lateral sclerosis functional rating scale (ALSFRS-R) correlated positively with HDL-C, but negatively with HOMA-IR. Through stepwise multiple regression analysis, we identified HOMA-IR as a significant metabolic determinant of ALSFRS-R. In biochemical analysis, we found that decreased expressions of insulin receptors, insulin receptor substrate-1 and insulin receptor-β, in autopsied muscles and fibroblasts of SBMA patients. This study demonstrates that SBMA patients have insulin resistance, which is associated with the disease severity. The expressions of insulin receptors are attenuated in the skeletal muscle of SBMA, providing a possible pathomechanism of metabolic alterations. These findings suggested that insulin resistance is a metabolic index reflecting disease severity and pathogenesis as well as a potential therapeutic target for SBMA.

DOI： 10.1007/s00415-017-8405-3

Progress toward the development of treatment of spinal and bulbar muscular atrophy

Sahashi Kentaro, Hashizume Atsushi, Sobue Gen, Katsuno Masahisa

EXPERT OPINION ON ORPHAN DRUGS 5 巻 ( 6 ) 頁： 503-514 2017年

<b>神経変性疾患に対するリバーストランスレーショナルリサーチの</b><b>基盤としての患者レジストリおよびバイオバンク </b>

勝野雅央, 橋詰淳, 中村治雅, 小野賢二郎, 湯地晃一郎, 山田雅信, 渡邉琢也, 一丸勝彦, 加藤直人, 井上隆弘, 草間真紀子, 水島洋, 藤本陽子, 秋元周, 小林潔, 大脇健二, 上杉幸嗣

臨床薬理 48 巻 ( 4 ) 頁： 141 - 147 2017年

SBMA患者に対する医師主導治験・臨床研究におけるCRCの取り組みについて査読有り

中野春美, 中島智美, 永野優子, 山口真佐美, 鈴木啓介, 橋詰淳, 土方靖浩, 山田晋一郎, 稲垣智則, 勝野雅央, 祖父江元

臨床神経学 56 巻 ( Suppl. ) 頁： S258 - S258 2016年12月

SMBAとALSにおける速/遅筋線維の選択的関与(Selective involvement of fast-/slow-twitch muscle fibers in SBMA and ALS) 査読有り

Yamada Shinichiro, Hashizume Atsushi, Hijikata Yasuhiro, Inagaki Tomonori, Kondo Naohide, Kawai Kaori, Noda Seiya, Nakanishi Hirotaka, Katsuno Masahisa, Sobue Gen

臨床神経学 56 巻 ( Suppl. ) 頁： S330 - S330 2016年12月

球脊髄性筋萎縮症における新規の定量的複合機能評価基準の開発(Development of a novel quantitative composite functional measure in spinalbulbar muscular atrophy) 査読有り

Inagaki Tomonori, Hashizume Atsushi, Hijikata Yasuhiro, Yamada Shinichiro, Katsuno Masahisa

臨床神経学 56 巻 ( Suppl. ) 頁： S330 - S330 2016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)の開発査読有り

橋詰淳, 土方靖浩, 山田晋一郎, 稲垣智則, 鈴木啓介, Grunseich Christopher, Kokkinis Angela, Fischbeck Kenneth, 祖父江元

臨床神経学 56 巻 ( Suppl. ) 頁： S489 - S489 2016年12月

球脊髄性筋萎縮症における破壊されたクレアチンの筋肉取り込み(Disrupted muscle uptake of creatine in spinal and bulbar muscular atrophy) 査読有り

Hijikata Yasuhiro, Katsuno Masahisa, Hashizume Atsushi, Yamada Shinichiro, Inagaki Tomonori, Suzuki Keisuke, Banno Haruhiko, Sobue Gen

臨床神経学 56 巻 ( Suppl. ) 頁： S271 - S271 2016年12月

Impaired muscle uptake of creatine in spinal and bulbar muscular atrophy 査読有り国際誌 Open Access

Hijikata, Y; Katsuno, M; Suzuki, K; Hashizume, A; Araki, A; Yamada, S; Inagaki, T; Iida, M; Noda, S; Nakanishi, H; Banno, H; Mano, T; Hirakawa, A; Adachi, H; Watanabe, H; Yamamoto, M; Sobue, G

ANNALS OF CLINICAL AND TRANSLATIONAL NEUROLOGY 3 巻 ( 7 ) 頁： 537 - 46 2016年7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：Annals of Clinical and Translational Neurology

Objective: The aim of this study was to explore the pathomechanism underlying the reduction of serum creatinine (Cr) concentrations in spinal and bulbar muscular atrophy (SBMA). Methods: We evaluated blood chemistries, motor function, and muscle mass measured by dual-energy X-ray absorptiometry in male subjects with SBMA (n = 65), amyotrophic lateral sclerosis (ALS; n = 27), and healthy controls (n = 25). We also examined the intramuscular concentrations of creatine, a precursor of Cr, as well as the protein and mRNA expression levels of the creatine transporter (SLC6A8) in autopsy specimens derived from subjects who had SBMA and ALS and disease controls. Furthermore, we measured the mRNA expression levels of SLC6A8 in cultured muscle cells (C2C12) transfected with the polyglutamine-expanded androgen receptor (AR-97Q). Results: Serum Cr concentrations were significantly lower in subjects with SBMA than in those with ALS (P < 0.001), despite similar muscle mass values. Intramuscular creatine concentrations were also lower in with the autopsied specimen of SBMA subjects than in those with ALS subjects (P = 0.018). Moreover, the protein and mRNA expression levels of muscle SLC6A8 were suppressed in subjects with SBMA. The mRNA levels of SLC6A8 were also suppressed in C2C12 cells bearing AR-97Q. Interpretation: These results suggest that low serum Cr concentration in subjects with SBMA is caused by impaired muscle uptake of creatine in addition to being caused by neurogenic atrophy. Given that creatine serves as an energy source in skeletal muscle, increasing muscle creatine uptake is a possible therapeutic approach for treating SBMA.

DOI： 10.1002/acn3.324

Decreased Peak Expiratory Flow Associated with Muscle Fiber-Type Switching in Spinal and Bulbar Muscular Atrophy 査読有り国際誌 Open Access

Yamada, S; Hashizume, A; Hijikata, Y; Inagaki, T; Suzuki, K; Kondo, N; Kawai, K; Noda, S; Nakanishi, H; Banno, H; Hirakawa, A; Koike, H; Halievski, K; Jordan, CL; Katsuno, M; Sobue, G

PLoS One 11 巻 ( 12 ) 頁： e0168846 2016年

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

The aim of this study was to characterize the respiratory function profile of subjects with spinal and bulbar muscular atrophy (SBMA), and to explore the underlying pathological mechanism by comparing the clinical and biochemical indices of this disease with those of amyotrophic lateral sclerosis (ALS). We enrolled male subjects with SBMA (n = 40) and ALS (n = 25) along with 15 healthy control subjects, and assessed their respiratory function, motor function, and muscle strength. Predicted values of peak expiratory flow (%PEF) and forced vital capacity were decreased in subjects with SBMA compared with controls. In SBMA, both values were strongly correlated with the trunk subscores of the motor function tests and showed deterioration relative to disease duration. Compared with activities of daily living (ADL)-matched ALS subjects, %PEF, tongue pressure, and grip power were substantially decreased in subjects with SBMA. Both immunofluorescence and RT-PCR demonstrated a selective decrease in the expression levels of the genes encoding the myosin heavy chains specific to fast-twitch fibers in SBMA subjects. The mRNA levels of peroxisome proliferator-activated receptor gamma coactivator 1-alpha and peroxisome proliferator-activated receptor delta were up-regulated in SBMA compared with ALS and controls. In conclusion, %PEF is a disease-specific respiratory marker for the severity and progression of SBMA. Explosive muscle strength, including %PEF, was selectively affected in subjects with SBMA and was associated with activation of the mitochondrial biogenesis-related molecular pathway in skeletal muscles.

DOI： 10.1371/journal.pone.0168846

SBMAにおける構音障害の臨床的特徴(Clinical characteristics of dysarthria in SBMA) 査読有り

田中誠也, 坂野晴彦, 勝野雅央, 鈴木啓介, 橋詰淳, 土方靖浩, 山田晋一郎, 山本正彦, 祖父江元

臨床神経学 55 巻 ( Suppl. ) 頁： S377 - S377 2015年12月

球脊髄性筋萎縮症における筋クレアチン代謝障害(Impaired muscular creatine metabolism in spinal and bulbar muscular atrophy) 査読有り

Hijikata Yasuhiro, Katsuno Masahisa, Suzuki Keisuke, Banno Haruhiko, Hashizume Atsushi, Yamada Shinichiro, Sobue Gen

臨床神経学 55 巻 ( Suppl. ) 頁： S346 - S346 2015年12月

球脊髄性筋萎縮症患者に対するリュープロレリン酢酸塩長期使用の効果査読有り

橋詰淳, 勝野雅央, 鈴木啓介, 坂野晴彦, 須賀徳明, 矢部一郎, 青木正志, 森田光哉, 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 55 巻 ( Suppl. ) 頁： S200 - S200 2015年12月

Myotonia-like symptoms in a patient with spinal and bulbar muscular atrophy 査読有り国際誌

Araki, K; Nakanishi, H; Nakamura, T; Atsuta, N; Yamada, S; Hijikata, Y; Hashizume, A; Suzuki, K; Katsuno, M; Sobue, G

NEUROMUSCULAR DISORDERS 25 巻 ( 11 ) 頁： 913 - 5 2015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：Neuromuscular Disorders

We describe the case of a 33-year-old man with a 4-year history of worsening muscle stiffness and weakness in his right hand. He showed elevated serum creatine kinase levels at the onset of muscle stiffness that was characterized by delayed muscle relaxation after voluntary contraction. This symptom often occurred during cold exposure, and was partially attenuated by sodium channel blockade. Electrodiagnostic findings in repetitive nerve stimulation, short-exercise, and cooling tests were normal. Electromyography showed chronic denervation potentials in his cranial, cervical, thoracic, and lumbosacral myotomes without myotonic discharge. He exhibited facial and tongue fasciculations, hypernasality, gynecomastia, neurogenic changes in muscle biopsy, and increased serum testosterone levels. Spinal and bulbar muscular atrophy (SBMA) was diagnosed on the basis of the CAG trinucleotide expansion in the gene coding androgen receptor. A myotonia-like symptom without myotonic discharge may present as an early neurological sign of SBMA, which possibly reflects a sodium channel dysfunction in skeletal muscles.

DOI： 10.1016/j.nmd.2015.08.006

Web of Science

Scopus

PubMed

球脊髄性筋萎縮症におけるインスリン抵抗性と運動機能の関連査読有り

中辻秀朗, 荒木周, 勝野雅央, 鈴木啓介, 坂野晴彦, 須賀徳明, 橋詰淳, 土方靖浩, 祖父江元

神経治療学 32 巻 ( 5 ) 頁： 817 - 817 2015年9月

筋萎縮性側索硬化症における耐糖能異常査読有り

荒木周, 勝野雅央, 鈴木啓介, 坂野晴彦, 橋詰淳, 土方靖浩, 山田晋一郎, 中辻秀朗, 寺尾心一, 祖父江元

神経治療学 32 巻 ( 5 ) 頁： 818 - 818 2015年9月

球脊髄性筋萎縮症における骨格筋クレアチン取り込み異常査読有り

土方靖浩, 勝野雅央, 橋詰淳, 山田晋一郎, 稲垣智則, 鈴木啓介, 坂野晴彦, 祖父江元

神経治療学 32 巻 ( 5 ) 頁： 755 - 755 2015年9月

A functional scale for spinal and bulbar muscular atrophy: Cross-sectional and longitudinal study 査読有り国際誌 Open Access

Hashizume, A; Katsuno, M; Suzuki, K; Banno, H; Suga, N; Mano, T; Araki, A; Hijikata, Y; Grunseich, C; Kokkinis, A; Hirakawa, A; Watanabe, H; Yamamoto, M; Fischbeck, KH; Sobue, G

NEUROMUSCULAR DISORDERS 25 巻 ( 7 ) 頁： 554 - 62 2015年7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：Neuromuscular Disorders

We aimed to develop, validate, and evaluate a disease-specific outcome measure for SBMA: the Spinal and Bulbar Muscular Atrophy Functional Rating Scale (SBMAFRS). We examined the Japanese version (SBMAFRS-J) in 80 Japanese SBMA subjects to evaluate its validity and reliability. We then assessed this scale longitudinally in 41 additional SBMA subjects. The English version (SBMAFRS-E) was also tested in 15 US subjects. The total score of the SBMAFRS-J was distributed normally without an extreme ceiling or floor effect. For SBMAFRS-J, the high intra- and inter-rater agreement was confirmed (intra-class correlation coefficients [ICCs] 0.910 and 0.797, respectively), and internal consistency was satisfactory (Cronbach's alpha 0.700-0.822). In addition, SBMAFRS-J demonstrated concurrent, convergent, and discriminant validity, except for the respiratory subscale. The inter-rater reliability and internal consistency of SBMAFRS-E were also satisfactory. Longitudinally, SBMAFRS-J showed a higher sensitivity to disease progression than the existing clinical measures. In conclusion, we developed and validated a disease-specific functional rating scale for SBMA in both Japanese and English versions, although it needs to be re-assessed in interventional studies with a larger sample size including English speaking subjects.

DOI： 10.1016/j.nmd.2015.03.008

Web of Science

Scopus

PubMed

Head Lift Exercise Improves Swallowing Dysfunction in Spinal and Bulbar Muscular Atrophy 査読有り国際誌

Mano, T; Katsuno, M; Banno, H; Suzuki, K; Suga, N; Hashizume, A; Araki, A; Hijikata, Y; Tanaka, S; Takatsu, J; Watanabe, H; Yamamoto, M; Sobue, G

EUROPEAN NEUROLOGY 74 巻 ( 5-6 ) 頁： 251 - 8 2015年

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

Background: Dysphagia due to bulbar involvement is a major symptom of patients with spinal and bulbar muscular atrophy (SBMA). The aim of this pilot study was to test the efficacy and safety of the head lift exercise for swallowing dysfunction in SBMA. Methods: We enrolled 6 subjects with genetically confirmed SBMA and instructed them to perform the head lift exercise for 6 weeks. The efficacy outcome measures were the changes from baseline in tongue pressure, the scores of swallowing functional questionnaires, and the motor functional scales and parameters of videofluorography (VF). Results: All subjects completed the study and no major adverse effects were recorded. Tongue pressure significantly increased by 19.2 ± 0.15% (p < 0.05) after the 6-week head lift exercise. The scores for oral dysphagia also improved, although there was no significant change in VF parameters or other variables examined pre- and post-exercise. Conclusion: Our findings suggested that the head lift exercise may improve swallowing dysfunction, particularly tongue pressure, in SBMA.

DOI： 10.1159/000431088

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論文 - 橋詰 淳

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論文 - 橋詰　淳