研究者詳細 - 小野寺　理

論文 - 小野寺　理

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脳神経疾患克服に向けた研究推進の提言2020，各論II（疾患群別）

望月秀樹, 青木正志, 池中建介, 井上治久, 岩坪威, 宇川義一, 岡澤均, 小野賢二郎, 小野寺理, 北川一夫, 齊藤祐子, 下畑享良, 髙橋良輔, 戸田達史, 中原仁, 松本理器, 水澤英洋, 三井純, 村山繁雄, 勝野雅央, 日本神経学会将来構想委員会, 青木吉嗣, 石浦浩之, 和泉唯信, 小池春樹, 島田斉, 髙橋祐二, 徳田隆彦, 中嶋秀人, 波田野琢, 三澤園子, 渡辺宏久

臨床神経学 advpub ( 6 ) 443 - 457 2022年5月

脳神経疾患克服に向けた研究推進の提言2020，各論I（方法論別）

臨床神経学 advpub ( 6 ) 429 - 442 2022年5月

[ATTRv amyloidosis with early improvement demonstrated by the 6-minute walk test following Patisiran therapy: a case report].

Shinya Oginezawa, Tomohiko Ishihara, Yohei Iwafuchi, Yuya Hatano, Ken Kashimura, Osamu Onodera

Rinsho shinkeigaku = Clinical neurology 62 ( 5 ) 375 - 379 2022年4月

記述言語：日本語掲載種別：研究論文（学術雑誌）

We report the case of a 65-year-old man who gradually developed numbness in both hands, lower limb muscle weakness and atrophy, and orthostatic hypotension over two and a half years. These symptoms indicated hereditary ATTR amyloidosis (ATTRv amyloidosis), and the final diagnosis was established through proof of TTR gene mutation (V30M). We initiated patisiran therapy, and a continuous 6-minute walking test performed 3 weeks from the start of therapy demonstrated improvement in the walking distance. This is a single case report showing the improvement in the motor and sensory function on administration of patisiran monotherapy from an early stage.

DOI： 10.5692/clinicalneurol.cn-001693

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禿頭と腰椎症を呈し、ヘテロ接合性HTRA1変異を伴った脳小血管病の31歳女性例

安田真人, 杉山淳比古, 森雅裕, 水地智基, 福武敏夫, 上村昌寛, 小野寺理, 桑原聡

臨床神経学 62 ( 4 ) 314 - 314 2022年4月

Brain TDP-43 pathology in corticobasal degeneration: Topographical correlation with neuronal loss. 国際誌

Makoto Sainouchi, Mari Tada, Yusran Ady Fitrah, Norikazu Hara, Kou Tanaka, Jiro Idezuka, Izumi Aida, Takashi Nakajima, Akinori Miyashita, Kohei Akazawa, Takeshi Ikeuchi, Osamu Onodera, Akiyoshi Kakita

Neuropathology and applied neurobiology 48 ( 3 ) e12786 2022年4月

記述言語：英語掲載種別：研究論文（学術雑誌）

AIMS: Neuronal and glial inclusions comprising transactive response DNA-binding protein of 43 kDa (TDP-43) have been identified in the brains of patients with corticobasal degeneration (CBD), and a possible correlation between the presence of these inclusions and clinical phenotypes has been speculated. However, the significance of TDP-43 pathology in the pathomechanism of CBD has remained unclear. Here, we investigated the topographical relationship between TDP-43 inclusions and neuronal loss in CBD. METHODS: We estimated semi-quantitatively neuronal loss and TDP-43 pathology in the form of neuronal cytoplasmic inclusions (NCIs), astrocytic inclusions (AIs), oligodendroglial cytoplasmic inclusions (GCIs), and dystrophic neurites in 22 CNS regions in 10 patients with CBD. Then, the degree of correlation between the severity of neuronal loss and the quantity of each type of TDP-43 inclusion was assessed. We also investigated tau pathology in a similar manner. RESULTS: TDP-43 pathology was evident in nine patients. The putamen and globus pallidus were the regions most frequently affected (80%). NCIs were the most prominent form, and their quantity was significantly correlated with the severity of neuronal loss in more than half of the regions examined. The quantities of TDP-43 NCIs and tau NCIs were correlated in only a few regions. The number of regions where the quantities of TDP-43 AIs and GCIs were correlated with the severity of neuronal loss was apparently small in comparison with that of NCIs. CONCLUSIONS: TDP-43 alterations in neurons, not closely associated with tau pathology, may be involved in the pathomechanism underlying neuronal loss in CBD. There was a significant topographical correlation between neuronal cytoplasmic aggregation of TDP-43 and neuronal loss in CBD, suggesting that TDP-43 protein aberration might be associated with neuronal degeneration in CBD. There was no close correlation between the burden of TDP-43 and that of tau in neurons.

DOI： 10.1111/nan.12786

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Authors' reply.

Minako Wakasugi, Akio Yokoseki, Masakazu Wada, Takeshi Momotsu, Kenji Sato, Hiroyuki Kawashima, Kazutoshi Nakamura, Osamu Onodera, Ichiei Narita

Journal of bone and mineral metabolism 40 ( 3 ) 537 - 538 2022年2月

Endogenous human retrovirus-K is not increased in the affected tissues of Japanese ALS patients. 国際誌

Tomohiko Ishihara, Akihide Koyama, Yuya Hatano, Ryoko Takeuchi, Yuka Koike, Taisuke Kato, Mari Tada, Akiyoshi Kakita, Osamu Onodera

Neuroscience research 178 78 - 82 2022年2月

記述言語：英語掲載種別：研究論文（学術雑誌）

Activation of human endogenous retrovirus-K (HERV-K) is one of the proposed risk factors for amyotrophic lateral sclerosis (ALS). The HERV-K envelope protein has been reported to show neurotoxicity, and development of therapy with reverse transcriptase inhibitors is being investigated. On the other hand, some reports have failed to show HERV-K activation in ALS. In this study, we analyzed the expression of HERV-K mRNA in the motor cortex and spinal cord of 15 Japanese patients with sporadic ALS and 19 controls using reverse transcriptase droplet digital PCR. This revealed no significant increase of HERV-K expression in ALS-affected tissues, suggesting that the association between ALS and HERV-K remains questionable.

DOI： 10.1016/j.neures.2022.01.009

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Stem cell transplantation for pediatric patients with adrenoleukodystrophy: A nationwide retrospective analysis in Japan. 国際誌

Koji Kato, Hiromasa Yabe, Nobuyuki Shimozawa, Souichi Adachi, Mineo Kurokawa, Yoshiko Hashii, Atsushi Sato, Nao Yoshida, Makiko Kaga, Osamu Onodera, Shunichi Kato, Yoshiko Atsuta, Tomohiro Morio

Pediatric transplantation 26 ( 1 ) e14125 2022年2月

記述言語：英語掲載種別：研究論文（学術雑誌）

BACKGROUND: Adrenoleukodystrophy (ALD) is an X-linked recessive disorder and 30-40% of patients develop progressive cerebral neurodegeneration. For symptomatic ALD patients, allogeneic stem cell transplantation (SCT) is considered the standard treatment modality to stabilize or prevent the progression of neurological symptoms. METHODS: We retrospectively analyzed the transplant outcomes of 99 pediatric patients with cerebral ALD in Japan. The conditioning regimens included Regimen A: fludarabine/melphalan/low-dose total body irradiation (TBI) with brain sparing (n = 39), Regimen B; busulfan/cyclophosphamide ± others (n = 23), Regimen C: melphalan/total lymphoid irradiation/thoracoabdominal irradiation ± anti-T lymphocyte globulin ± fludarabine (n = 27), and Regimen D: others (n = 10). RESULTS: The 5-year overall survival (OS) and event-free survival (EFS) of all patients were 90.0% and 72.9%, respectively. The 5-year OS was 100.0% for Regimen A, 91.1% for Regimen B, 84.4% for Regimen C, and 67.5% for Regimen D (p = 0.028). The 5-year EFS was 78.3% for Regimen A, 78.0% for Regimen B, 70.4% for Regimen C, and 48.0% for Regimen D (p = 0.304). The OS marginally improved after 2007 compared with before 2006 (95.3% vs. 85.2%, p = 0.066), due to the improvement of cord blood transplantation (CBT) outcomes after 2007 compared with before 2006 (96.6% vs. 68.4%, p = 0.005). On magnetic resonance imaging of the brain, a reduced Loes score after SCT was only observed in one of the 15 bone marrow transplantation (BMT) patients, but in 5 of the 15 CBT patients (p = 0.173). CONCLUSIONS: Our study revealed that a reduced conditioning regimen with fludarabine/melphalan/low-dose TBI provides better outcomes, and the results of CBT significantly improved after 2007.

DOI： 10.1111/petr.14125

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USP10 inhibits aberrant cytoplasmic aggregation of TDP-43 by promoting stress granule clearance. 国際誌

Masahiko Takahashi, Hiroki Kitaura, Akiyoshi Kakita, Taichi Kakihana, Yoshinori Katsuragi, Osamu Onodera, Yuriko Iwakura, Hiroyuki Nawa, Masaaki Komatsu, Masahiro Fujii

Molecular and cellular biology 42 ( 3 ) MCB0039321 2022年1月

記述言語：英語掲載種別：研究論文（学術雑誌）

TDP-43 is a causative factor of amyotrophic lateral sclerosis (ALS). Cytoplasmic TDP-43 aggregates in neurons are a hallmark pathology of ALS. Under various stress conditions, TDP-43 localizes sequentially to two cytoplasmic protein aggregates: stress granules (SGs) first, and then aggresomes. Accumulating evidence suggests that delayed clearance of TDP-43-positive SGs is associated with pathological TDP-43 aggregates in ALS. We found that USP10 promotes the clearance of TDP-43-positive SGs in cells treated with proteasome inhibitor, thereby promoting the formation of TDP-43-positive aggresomes, and the depletion of USP10 increases the amount of insoluble TDP-35, a cleaved product of TDP-43, in the cytoplasm. TDP-35 interacted with USP10 in an RNA-binding dependent manner; however, impaired RNA-binding of TDP-35 reduced the localization in SGs and aggresomes and induced USP10-negative TDP-35 aggregates. Immunohistochemistry showed that most of the cytoplasmic TDP-43/TDP-35-aggregates in the neurons of ALS patients were USP10-negative. Our findings suggest that USP10 inhibits aberrant aggregation of TDP-43/TDP-35 in the cytoplasm of neuronal cells by promoting the clearance of TDP-43/TDP-35-positive SGs and facilitating the formation of TDP-43/TDP-35-positive aggresomes.

DOI： 10.1128/MCB.00393-21

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Polypharmacy, chronic kidney disease, and incident fragility fracture: a prospective cohort study.

Minako Wakasugi, Akio Yokoseki, Masakazu Wada, Takeshi Momotsu, Kenji Sato, Hiroyuki Kawashima, Kazutoshi Nakamura, Osamu Onodera, Ichiei Narita

Journal of bone and mineral metabolism 40 ( 1 ) 157 - 166 2022年1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：SPRINGER JAPAN KK

INTRODUCTION: Polypharmacy is associated with an increased risk of fracture in aging populations, but no study has accounted for the impact of kidney function on this association. This study aimed to examine the association between polypharmacy and incident fragility fracture based on chronic kidney disease (CKD) status. MATERIALS AND METHODS: Participants were 2023 patients (55% men; mean age, 69 years) of Sado General Hospital enrolled in the Project in Sado for Total Health (PROST) between June 2008 and December 2016. Among these, 65%, 28%, and 7% had non-CKD, non-dialysis-dependent CKD, and dialysis-dependent CKD, respectively. Multivariable Cox proportional hazards analysis was conducted with adjustments for potential confounders. RESULTS: Prevalences of polypharmacy (≥ 5 medications) and hyperpolypharmacy (≥ 10 medications) among participants were 43% and 9% for non-CKD, 62% and 23% for non-dialysis-dependent CKD, and 85% and 34% for dialysis-dependent CKD, respectively. During a median follow-up of 5.6 years, 256 fractures occurred. More medications were associated with a higher risk of fractures. Specifically, compared to participants without polypharmacy, adjusted hazard ratios were 1.32 (95% CI 0.96-1.79) and 1.99 (1.35-2.92) for those with polypharmacy and hyperpolypharmacy, respectively, after adjusting for osteoporosis risk factors, CKD status, and comorbidities. No effect modification by CKD status was observed (interaction P = 0.51). Population-attributable fractions of hyperpolypharmacy for fracture were 9.9% in the total cohort and 42.1% in dialysis-dependent CKD patients. CONCLUSION: Hyperpolypharmacy is associated with an increased risk of fragility fracture regardless of CKD status, and has a strong impact on incident fragility fractures in dialysis-dependent CKD patients.

DOI： 10.1007/s00774-021-01272-9

Web of Science

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Imaging Characteristics for Predicting Cognitive Impairment in Patients With Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy. 国際誌

Akira Taniguchi, Akihiro Shindo, Ken-Ichi Tabei, Osamu Onodera, Yukio Ando, Takao Urabe, Kazumi Kimura, Kazuo Kitagawa, Yoshihiro Miyamoto, Misa Takegami, Masafumi Ihara, Ikuko Mizuta, Toshiki Mizuno, Hidekazu Tomimoto

Frontiers in aging neuroscience 14 876437 - 876437 2022年

記述言語：英語掲載種別：研究論文（学術雑誌）

Objectives: Patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) show various clinical symptoms, including migraine, recurrent stroke, and cognitive impairment. We investigated the associations between magnetic resonance imaging (MRI) markers of small vessel disease and neuropsychological tests and identified the MRI characteristics for predicting cognitive impairment in patients with CADASIL. Methods: Subjects included 60 CADASIL patients diagnosed with genetic tests and registered in the Japanese CADASIL REDCap database between June 2016 and December 2020. Patient information including clinical data, modified Rankin Scale (mRS); MRI findings of small vessel disease including periventricular and deep white matter lesions (WML), lacunar infarcts, and cerebral microbleeds (CMBs); and neuropsychological tests, including the Japanese version of the Mini-Mental State Examination (MMSE), the Japanese version of the Montreal Cognitive Assessment (MoCA-J), and the Frontal Assessment Battery (FAB), were evaluated. Results: Data from 44 CADASIL patients were eligible for this study, compared between patients with and without dementia. Regarding the neuroimaging findings, the Fazekas score of periventricular and deep WML was higher in patients with dementia (periventricular, p = 0.003; deep, p = 0.009). The number of lacunar infarcts was higher in patients with dementia (p = 0.001). The standardized partial regression coefficient (SPRC) in MoCA-J was 0.826 (95% CI, 0.723-0.942; p = 0.005) for the number of CMBs. The SPRC in MMSE was 0.826 (95% CI, 0.719-0.949; p = 0.007) for the number of CMBs. The SPRC for FAB decreased significantly to 0.728 (95% CI, 0.551-0.960; p = 0.024) for the number of lacunar infarcts. Receiver operating characteristic (ROC) curves for dementia showed that in the number of lacunar infarcts, a cut-off score of 5.5 showed 90.9% sensitivity and 61.1% specificity. For the number of CMBs, a cut-off score of 18.5 showed 45.5% sensitivity and 100% specificity. Conclusion: The characteristic MRI findings were that CADASIL patients with dementia had severe WML, both periventricular and deep, and a larger number of lacunar infarcts than those without dementia. The risk of dementia may be associated with ≥ 6 lacunar infarcts, ≥19 CMBs, or a Fazekas scale score of 3 in periventricular and deep WML.

DOI： 10.3389/fnagi.2022.876437

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Neuronal Intranuclear Inclusion Disease Presenting with Voice Tremor 国際誌

Tomone Taneda, Masato Kanazawa, Yo Higuchi, Hironori Baba, Aiko Isami, Masahiro Uemura, Takuya Konno, Arata Horii, Takeshi Ikeuchi, Osamu Onodera

Movement Disorders Clinical Practice 9 ( 3 ) 404 - 406 2021年12月

Parkinson's disease and parkinsonism: Clinicopathological discrepancies on diagnosis in three patients. 国際誌

Yasuko Toyoshima, Hitoshi Takahashi, Shinnichi Katada, Naoyuki Kojima, Mari Tada, Takashi Tani, Ryoko Koike, Takanori Nozawa, Izumi Aida, Takashi Nakajima, Osamu Onodera, Akiyoshi Kakita

Neuropathology : official journal of the Japanese Society of Neuropathology 41 ( 6 ) 450 - 456 2021年12月

記述言語：英語掲載種別：研究論文（学術雑誌）

Parkinson's disease (PD) is one of the most common neurodegenerative disorders. The cardinal neuropathological features of PD include selective and progressive loss of pigmented neurons in the substantia nigra, deficiencies in dopaminergic signaling in the striatum, and occurrence of phosphorylated α-synuclein-identified Lewy bodies in the nervous system. Parkinsonism, the clinical presentation of movement disorders seen in PD, is a feature shared commonly by other pathologically distinct neurodegenerative diseases, such as progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), and multiple system atrophy (MSA). Consequently, it is sometimes difficult to distinguish PD from such parkinsonism-related neurological disorders. In addition, parkinsonism is not always a feature of certain neurodegenerative diseases, and it can sometimes develop as a result of various forms of drug intoxication or cerebrovascular disease. Here, we describe the clinicopathological features of three patients (cases 1, 2, and 3) diagnosed as having PSP, MSA, and PD, respectively, in each of whom the postmortem histopathological diagnosis differed from the final clinical diagnosis. Neuropathologically, they had suffered from coexistent disorders: PD, MSA, and argyrophilic grain disease (case 1); PD (case 2); and vascular parkinsonism (case 3). The variety of patients showing features of parkinsonism underlines the importance of careful long-term follow up followed by postmortem neuropathological evaluation.

DOI： 10.1111/neup.12777

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[Recommendations (Proposal) for promoting research for overcoming neurological diseases 2020].

Hideki Mochizuki, Masashi Aoki, Kensuke Ikenaka, Haruhisa Inoue, Takeshi Iwatsubo, Yoshikazu Ugawa, Hitoshi Okazawa, Kenjiro Ono, Osamu Onodera, Kazuo Kitagawa, Yuko Saito, Takayoshi Shimohata, Ryosuke Takahashi, Tatsushi Toda, Jin Nakahara, Riki Matsumoto, Hidehiro Mizusawa, Jun Mitsui, Shigeo Murayama, Masahisa Katsuno, Yoshitsugu Aoki, Hiroyuki Ishiura, Yuishin Izumi, Haruki Koike, Hitoshi Shimada, Yuji Takahashi, Takahiko Tokuda, Hideto Nakajima, Taku Hatano, Sonoko Misawa, Hirohisa Watanabe

Rinsho shinkeigaku = Clinical neurology 61 ( 11 ) 709 - 721 2021年11月

記述言語：日本語掲載種別：研究論文（学術雑誌）

The Japanese Society of Neurology discusses research, education, and medical care in the field of neurology and makes recommendations to the national government. Dr. Mizusawa, the former representative director of the Japanese Society of Neurology, selected committee members and made "Recommendations for Promotion of Research for Overcoming Neurological Diseases" in 2013. After that, the Future Vision Committee was established in 2014, and these recommendations have been revised once every few years by the committee. This time, the Future Vision Committee made the latest recommendations from 2020 to 2021. In this document, the general part is 1) What is neurological disease? 2) Current status of neurological disease overcoming research, 3) Significance and necessity of neurological disease overcoming research, 4) Research promotion system for overcoming neurological disease, 5) the roadmap for overcoming neuromuscular diseases, 6) a summary version of these recommendations are explained using figures that are easy for the general public to understand.

DOI： 10.5692/clinicalneurol.cn-001639

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ラット脳虚血後のリン酸化タウ発現の経時変化

大津裕, 金山武史, 二宮格, 畠山公大, 小野寺理, 金澤雅人

脳循環代謝 33 ( 1 ) 96 - 96 2021年11月

加齢と関連したDNA脱メチル化がTDP-43量の自己調節機構を障害する

小池佑佳, 須貝章弘, 原範和, 伊藤絢子, 横関明男, 石原智彦, 山岸拓磨, 坪口晋太朗, 他田真理, 池内健, 柿田明美, 小野寺理

Dementia Japan 35 ( 4 ) 610 - 610 2021年10月

パチシラン単独投与で早期より運動機能が改善したATTRアミロイドーシスの1例

荻根沢真也, 石原智彦, 岩淵洋平, 畠野雄也, 柏村健, 小野寺理

神経治療学 38 ( 6 ) S293 - S293 2021年10月

神経核内封入体病(NIID)におけるNOTCH2NLCリピート伸長と臨床的特徴の検討

樋口陽, 原範和, Yusran Ady Fitrah, 種田朝音, 徳武孝允, 三浦健, 岩淵洋平, 五十嵐修一, 林秀樹, 石黒敬信, 三瓶一弘, 武田勇人, 高橋俊昭, 金澤雅人, 宮下哲典, 小野寺理, 池内健

Dementia Japan 35 ( 4 ) 612 - 612 2021年10月

Do patients with multiple system atrophy have decreased nocturnal urinary concentration? 国際誌

Yusuke Sakata, Masato Kanazawa, Masahiro Hatakeyama, Takuya Konno, Tetsutaro Ozawa, Osamu Onodera

Clinical autonomic research : official journal of the Clinical Autonomic Research Society 2021年9月

小字症を呈した自己免疫性脳幹脳炎の臨床的特徴

助川真響, 畠山公大, 羽入龍太郎, 滑川将気, 大津裕, 橋田裕美, 金澤雅人, 小野寺理

臨床神経学 61 ( Suppl. ) S431 - S431 2021年9月

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