研究者詳細 - 小野寺　理

論文 - 小野寺　理

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本邦における抗NMDA受容体脳炎の特徴自己免疫介在性脳炎・脳症の全国疫学調査

佐治越爾, 佐藤泰憲, 飯塚高浩, 木村暁夫, 佐久間啓, 中嶋秀人, 大石真莉子, 中島章博, 神田隆, 高橋幸利, 渡邊修, 鵜沢顕之, 桑原聡, 小野寺理, 河内泉

臨床神経学 64 ( Suppl. ) S252 - S252 2024年10月

Qki5 safeguards spinal motor neuron function by defining the motor neuron-specific transcriptome via pre-mRNA processing. 国際誌

Yoshika Hayakawa-Yano, Takako Furukawa, Tsuyoshi Matsuo, Takahisa Ogasawara, Masahiro Nogami, Kazumasa Yokoyama, Masato Yugami, Munehisa Shinozaki, Chihiro Nakamoto, Kenji Sakimura, Akihide Koyama, Kazuhiro Ogi, Osamu Onodera, Hirohide Takebayashi, Hideyuki Okano, Masato Yano

Proceedings of the National Academy of Sciences of the United States of America 121 ( 37 ) e2401531121 2024年9月

記述言語：英語掲載種別：研究論文（学術雑誌）

Many RNA-binding proteins (RBPs) are linked to the dysregulation of RNA metabolism in motor neuron diseases (MNDs). However, the molecular mechanisms underlying MN vulnerability have yet to be elucidated. Here, we found that such an RBP, Quaking5 (Qki5), contributes to formation of the MN-specific transcriptome profile, termed "MN-ness," through the posttranscriptional network and maintenance of the mature MNs. Immunohistochemical analysis and single-cell RNA sequencing (scRNA-seq) revealed that Qki5 is predominantly expressed in MNs, but not in other neuronal populations of the spinal cord. Furthermore, comprehensive RNA sequencing (RNA-seq) analyses revealed that Qki5-dependent RNA regulation plays a pivotal role in generating the MN-specific transcriptome through pre-messenger ribonucleic acid (mRNA) splicing for the synapse-related molecules and c-Jun N-terminal kinase/stress-activated protein kinase (JNK/SAPK) signaling pathways. Indeed, MN-specific ablation of the Qki5 caused neurodegeneration in postnatal mice and loss of Qki5 function resulted in the aberrant activation of stress-responsive JNK/SAPK pathway both in vitro and in vivo. These data suggested that Qki5 plays a crucial biological role in RNA regulation and safeguarding of MNs and might be associated with pathogenesis of MNDs.

DOI： 10.1073/pnas.2401531121

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Lessons from the ATTeST trial in ataxia telangiectasia. 国際誌

Yuka Koike, Osamu Onodera

The Lancet. Neurology 23 ( 9 ) 846 - 847 2024年9月

Indications for a brain biopsy in neurological diseases of unknown etiology: The role of magnetic resonance imaging findings and liquid biopsy in yielding definitive pathological diagnoses. 国際誌

Toshiya Kizaki, Masato Kanazawa, Takanobu Ishiguro, Manabu Natsumeda, Mari Tada, Hiroshi Shimizu, Kouichirou Okamoto, Makoto Oishi, Akiyoshi Kakita, Yukihiko Fujii, Osamu Onodera

Journal of the neurological sciences 463 123150 - 123150 2024年8月

記述言語：英語掲載種別：研究論文（学術雑誌）

Brain biopsies are often considered for patients who cannot be diagnosed with various laboratory test results. However, physicians tend to be hesitant regarding their application in possibly non-neoplastic brain diseases, due to the invasiveness and risks. The aim was to determine the indications for brain biopsies in cases of neurological diseases of unknown etiology. We retrospectively evaluated diagnostic accuracy, laboratory findings (including a liquid biopsy for malignant lymphoma), magnetic resonance imaging (MRI) characteristics and the post-treatment outcomes of patients undergoing brain biopsies for neurological diseases of unknown etiology. The data of patients who had undergone a brain biopsy during their admission to Niigata University Hospital, between 2011 and 2024, were reviewed. Moreover, the laboratory data and MRI findings between patients with definitive and nonspecific biopsy diagnoses were compared. Twenty-six patients underwent a brain biopsy, and a definitive diagnosis was obtained in 14 patients (53.8%). Even in cases where a nonspecific diagnosis was made, biopsy findings helped rule out malignancy and guide clinical diagnosis and treatment decisions. The liquid biopsy for malignant lymphoma was performed in eight patients, with one yielding a positive result, consistent with primary central nervous system lymphoma. The sensitivity and specificity of liquid biopsy were 0.5 and 1, respectively. Diffusely contrasted cortical lesions and the presence of mass effects on MRI, were significantly associated with a definitive diagnosis, compared to a nonspecific diagnosis. In conclusion, brain MRI and liquid biopsies can assist in determining the appropriate indications for brain biopsies in neurological diseases of unknown etiology.

DOI： 10.1016/j.jns.2024.123150

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A patient with neuronal intranuclear inclusion disease developed encephalitis‐like symptoms after cerebral angiography

Shin Koide, Shintaro Tsuboguchi, Shingo Koide, Itaru Ninomiya, Taiki Saito, Takanobu Ishiguro, Etsuji Saji, Yo Higuchi, Takeshi Ikeuchi, Makoto Oishi, Masato Kanazawa, Osamu Onodera

Neurology and Clinical Neuroscience 2024年6月

掲載種別：研究論文（学術雑誌）出版者・発行元：Wiley

Abstract

Patients with neuronal intranuclear inclusion disease (NIID) can present with encephalitis‐like symptoms such as recurrent paroxysmal fever and unconsciousness. To date, no specific triggers for these symptoms have been reported. In our case, an 78‐year‐old woman became unconscious and developed fever after cerebral angiography. The patient had experienced four episodes of unconsciousness and fever in the past 7 years. Postangiography, she immediately became unconscious and developed fever. No vascular abnormalities were found and magnetic resonance imaging of the brain revealed expanding white matter lesions and hyperintense lesions along the corticomedullary junction. Genetic analysis revealed an abnormal GGC repeat expansion in NOTCH2NLC. Thus, we diagnosed the patient with NIID. We suggest that cerebral angiography is a possible trigger for encephalitis‐like symptoms in NIID.

DOI： 10.1111/ncn3.12839

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Cerebellar compensation: a case of aphasia due to cerebellar hemorrhage

Yukiko Kinoshita, Masahiro Hatakeyama, Mika Otsuki, Takanobu Ishiguro, Etsuji Saji, Masato Kanazawa, Osamu Onodera

Journal of Neurology 2024年6月

UCP2 polymorphisms, daily step count, and number of teeth associated with all-cause mortality risk in Sado City: A hospital-based cohort study

Han Lyu, Noriko Sugita, Shigeki Komatsu, Minako Wakasugi, Akio Yokoseki, Akihiro Yoshihara, Tetsuo Kobayashi, Kenji Sato, Hiroyuki Kawashima, Osamu Onodera, Ichiei Narita, Koichi Tabeta

Heliyon 10 ( 12 ) e32512 - e32512 2024年6月

造影効果を伴う多発脳病変を呈し,悪性腫瘍との鑑別を要したウイルス性髄膜脳炎の73歳女性の一例

柏木健太, 大津裕, 岸諒太, 福本淳貴, 石黒敬信, 佐治越爾, 金澤雅人, 小野寺理

臨床神経学 64 ( 6 ) 438 - 438 2024年6月

From bench to bedside: US-Japan Collaborative Workshop on the NVU

Cesario Borlongan, Elga Esposito, Laarni Grace Corales, Yorito Hattori, Yu Hayashi, Masafumi Ihara, Jeffrey J. Iliff, Kassandra Kisler, Shinobu Kitazume, Schuichi Koizumi, Jialing Liu, Takakuni Maki, Osamu Onodera, Satoshi Saito, Kazunobu Sawamoto, Kazuhiro Sohya, Akihiko Taguchi, Shinichi Takahashi, Kenji Tanaka, Toshiaki Taoka, Hiroaki Wake, Michisuke Yuzaki

The Journal of Physiological Sciences 74 ( S1 ) 2024年5月

掲載種別：研究論文（学術雑誌）出版者・発行元：Springer Science and Business Media LLC

Abstract

The joint workshop between U.S. and Japanese researchers, supported by The U.S.–Japan Brain Research Cooperative Program, convened in January 2023 at Keio University Mita campus in Tokyo, Japan. The workshop had a threefold objective. Firstly, it aimed to facilitate robust exchanges between U.S. and Japanese researchers engaged in Neurovascular Unit (NVU) research, enhancing the global network of scholars in the field. Secondly, it aimed to encourage the initiation of collaborative research projects, fostering interdisciplinary efforts and synergistic advancements in understanding the brain vascular physiology and central nervous system. Lastly, the workshop emphasized the nurturing of young researchers, recognizing their pivotal role in shaping the future of NVU research. Throughout the workshop, participants discussed fundamental aspects of the NVU, exploring its complex connections and vital functions. By sharing their expertise and insights, the workshop attendees sought to uncover novel approaches to mitigate the burden of neurological diseases for individuals worldwide. This report provides a summary of the presentations and discussions held during the workshop, showcasing the collective efforts and progress made by the participants.

DOI： 10.1186/s12576-024-00917-4

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その他リンク： https://link.springer.com/article/10.1186/s12576-024-00917-4/fulltext.html

Distal CIDPと鑑別を要したが、M蛋白やVEGFの再検が診断に有用であったPOEMS症候群の一例

鈴木大介, 鈴木佑弥, 菊池謙次, 鈴木義広, 小出伸, 安藤昭一朗, 石黒敬信, 金澤雅人, 小野寺理, 諏訪部達也, 瀧澤淳

臨床神経学 64 ( 5 ) 365 - 365 2024年5月

Stage-dependent immunity orchestrates AQP4 antibody-guided NMOSD pathology: a role for netting neutrophils with resident memory T cells in situ

Akihiro Nakajima, Fumihiro Yanagimura, Etsuji Saji, Hiroshi Shimizu, Yasuko Toyoshima, Kaori Yanagawa, Musashi Arakawa, Mariko Hokari, Akiko Yokoseki, Takahiro Wakasugi, Kouichirou Okamoto, Hirohide Takebayashi, Chihiro Fujii, Kyoko Itoh, Yo-ichi Takei, Shinji Ohara, Mitsunori Yamada, Hitoshi Takahashi, Masatoyo Nishizawa, Hironaka Igarashi, Akiyoshi Kakita, Osamu Onodera, Izumi Kawachi

Acta Neuropathologica 147 ( 1 ) 2024年4月

Acute respiratory failure caused by brainstem demyelinating lesions in an older patient with an atypical relapsing autoimmune disorder

Shoko Hongo, Hiroshi Shimizu, Etsuji Saji, Akihiro Nakajima, Kouichirou Okamoto, Izumi Kawachi, Osamu Onodera, Akiyoshi Kakita

Neuropathology 2024年4月

掲載種別：研究論文（学術雑誌）出版者・発行元：Wiley

An 84‐year‐old man presented with somnolence, dysphagia, and right hemiplegia, all occurring within a month, approximately one year after initial admission due to subacute, transient cognitive decline suggestive of acute disseminated encephalomyelitis involving the cerebral white matter. Serial magnetic resonance imaging (MRI) studies over that period revealed three high‐intensity signal lesions on fluid‐attenuated inversion recovery images, appearing in chronological order in the left upper and left lower medulla oblongata and left pontine base. Despite some clinical improvement following methylprednisolone pulse therapy, the patient died of respiratory failure. Autopsy revealed four fresh, well‐defined lesions in the brainstem, three of which corresponded to the lesions detected radiologically. The remaining lesion was located in the dorsal medulla oblongata and involved the right solitary nucleus. This might have appeared at a later disease stage, eventually causing respiratory failure. Histologically, all four lesions showed loss of myelin, preservation of axons, and infiltration of lymphocytes, predominantly CD8‐positive T cells, consistent with the histological features of autoimmune demyelinating diseases, particularly the confluent demyelination observed in the early and acute phases of multiple sclerosis (MS). In the cerebral white matter, autoimmune demyelination appeared superimposed on ischemic changes, consistent with the cerebrospinal fluid (CSF) and MRI findings on initial admission. No anti‐AQP4 or MOG antibodies or those potentially causing autoimmune encephalitis/demyelination were detected in either the serum or CSF. Despite several similarities to MS, such as the relapsing–remitting disease course and lesion histology, the entire clinicopathological picture in the present patient, especially the advanced age at onset and development of brainstem lesions in close proximity within a short time frame, did not fit those of MS or other autoimmune diseases that are currently established. The present results suggest that exceptionally older individuals can be affected by an as yet unknown inflammatory demyelinating disease of the CNS.

DOI： 10.1111/neup.12976

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Dysregulation of stress granule dynamics by DCTN1 deficiency exacerbates TDP-43 pathology in Drosophila models of ALS/FTD

Tetsuhiro Ueda, Toshihide Takeuchi, Nobuhiro Fujikake, Mari Suzuki, Eiko N. Minakawa, Morio Ueyama, Yuzo Fujino, Nobuyuki Kimura, Seiichi Nagano, Akio Yokoseki, Osamu Onodera, Hideki Mochizuki, Toshiki Mizuno, Keiji Wada, Yoshitaka Nagai

Acta Neuropathologica Communications 12 ( 1 ) 2024年2月

掲載種別：研究論文（学術雑誌）出版者・発行元：Springer Science and Business Media LLC

Abstract

The abnormal aggregation of TDP-43 into cytoplasmic inclusions in affected neurons is a major pathological hallmark of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Although TDP-43 is aberrantly accumulated in the neurons of most patients with sporadic ALS/FTD and other TDP-43 proteinopathies, how TDP-43 forms cytoplasmic aggregates remains unknown. In this study, we show that a deficiency in DCTN1, a subunit of the microtubule-associated motor protein complex dynactin, perturbs the dynamics of stress granules and drives the formation of TDP-43 cytoplasmic aggregation in cultured cells, leading to the exacerbation of TDP-43 pathology and neurodegeneration in vivo. We demonstrated using a Drosophila model of ALS/FTD that genetic knockdown of DCTN1 accelerates the formation of ubiquitin-positive cytoplasmic inclusions of TDP-43. Knockdown of components of other microtubule-associated motor protein complexes, including dynein and kinesin, also increased the formation of TDP-43 inclusions, indicating that intracellular transport along microtubules plays a key role in TDP-43 pathology. Notably, DCTN1 knockdown delayed the disassembly of stress granules in stressed cells, leading to an increase in the formation of pathological cytoplasmic inclusions of TDP-43. Our results indicate that a deficiency in DCTN1, as well as disruption of intracellular transport along microtubules, is a modifier that drives the formation of TDP-43 pathology through the dysregulation of stress granule dynamics.

DOI： 10.1186/s40478-024-01729-8

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その他リンク： https://link.springer.com/article/10.1186/s40478-024-01729-8/fulltext.html

Validation study of the Japanese version of the King's Parkinson's Disease Pain Scale and the King's Parkinson's Disease Pain Questionnaire. 国際誌

Kanako Kurihara, Shinsuke Fujioka, Yasuaki Mizutani, Hirohisa Watanabe, Kazuhiro Iwaoka, Tetsuya Maeda, Morinobu Seki, Toshiki Tezuka, Jin Nakahara, Takuya Konno, Takanobu Ishiguro, Osamu Onodera, Yuri Asano, Kazushi Takahashi, Alexandra Rizos, K Ray Chaudhuri, Yoshio Tsuboi

Parkinsonism & related disorders 120 106012 - 106012 2024年1月

記述言語：英語掲載種別：研究論文（学術雑誌）

INTRODUCTION: The King's Parkinson's Disease Pain Scale (KPPS)/King's Parkinson's Disease Pain Questionnaire (KPPQ) was developed as a tool to quantitatively assess pain in patients with Parkinson's disease (PwPD). Here, we conducted a Japanese multicenter validation study to verify the reliability of KPPS/KPPQ in Japanese PwPD. METHODS: PwPD, ≥20 years, with unexplained pain were included; those with a definitive primary cause of pain other than PD were excluded. A total of 151 patients who fulfilled the criteria were analyzed, and test-retest reliability was investigated in 25 individuals. RESULTS: The 151 patients included 101 women (66.9 %); mean age 68.3 ± 9.9 years, mean disease duration 9.2 ± 5.2 years. The most frequent pain type in the KPPS classification was musculoskeletal pain (82.8 %). There was a positive correlation between KPPS total score and the Non-Motor Symptoms Scale (NMSS) total score, NMSS item 27, the Parkinson's disease sleep scale-version 2 (PDSS-2) total score, PDSS-2 item 10, the Parkinson's Disease Questionnaire-8 (PDQ-8) summary index and PDQ-8 item 7. Cronbach's alpha of KPPS was 0.626 (0.562-0.658) and the intraclass correlation coefficient of test-retest reliability was 0.740. Cronbach's alpha of KPPQ was 0.660 (0.617-0.705) and a test-retest reliability of kappa coefficient was 0.593 (0.0-1.0). CONCLUSIONS: KPPS correlated well with other scales for assessing pain. KPPS correlated well with patients' quality of life, non-motor symptoms, and sleep disturbances. The reproducibility of KPPS/KPPQ makes it suitable for continuous evaluation of the same patient. On the other hand, the internal consistency of KPPS/KPPQ is rather low.

DOI： 10.1016/j.parkreldis.2024.106012

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Corrigendum: Regeneration of the cerebral cortex by direct chemical reprogramming of macrophages into neuronal cells in acute ischemic stroke. 国際誌

Itaru Ninomiya, Akihide Koyama, Yutaka Otsu, Osamu Onodera, Masato Kanazawa

Frontiers in cellular neuroscience 18 1372045 - 1372045 2024年

The Japan MSA registry: A multicenter cohort study of multiple system atrophy

Ayaka Chikada, Kenta Orimo, Jun Mitsui, Takashi Matsukawa, Hiroyuki Ishiura, Tatsushi Toda, Hidehiro Mizusawa, Yuji Takahashi, Masahisa Katsuno, Kazuhiro Hara, Osamu Onodera, Tomohiko Ishihara, Masayoshi Tada, Satoshi Kuwabara, Atsuhiko Sugiyama, Yoshitaka Yamanaka, Ryosuke Takahashi, Nobukatsu Sawamoto, Yusuke Sakato, Tomoyuki Ishimoto, Ritsuko Hanajima, Yasuhiro Watanabe, Hiroshi Takigawa, Tadashi Adachi, Koji Abe, Toru Yamashita, Hiroshi Takashima, Keiko Higashi, Junichi Kira, Ichiro Yabe, Masaaki Matsushima, Katsuhisa Ogata, Kinya Ishikawa, Yoichiro Nishida, Taro Ishiguro, Kokoro Ozaki, Tetsuya Nagata, Shoji Tsuji

Neurology and Clinical Neuroscience 2024年

Reliability study for the Japanese version of the Columbia Muscle Cramp Scale in amyotrophic lateral sclerosis

Masahiro Sawada, Takehisa Hirayama, Masaru Yanagihashi, Koji Fukushima, Yuishin Izumi, Tameto Naoi, Mitsuya Morita, Hitoshi Warita, Masashi Aoki, Yohei Iguchi, Masahisa Katsuno, Nobuhiro Ogawa, Makoto Urusitani, Tomohiko Ishihara, Osamu Onodera, Yoshitaka Murakami, Hiroshi Mitsumoto, Osamu Kano

Neurology and Clinical Neuroscience 2024年

Case report: Progressive multifocal leukoencephalopathy co-occurring with neurosarcoidosis: early brain biopsy and appropriate therapy for PML resulted in a favorable prognosis. 国際誌

Qiannan Wang, Shintaro Tsuboguchi, Kouichirou Okamoto, Mari Tada, Akiyoshi Kakita, Kazuo Nakamichi, Makoto Oishi, Masato Kanazawa, Osamu Onodera

Frontiers in immunology 15 1447992 - 1447992 2024年

記述言語：英語掲載種別：研究論文（学術雑誌）

Progressive multifocal leukoencephalopathy (PML) is a rare central nervous system disease caused by JC virus (JCV) infection. Human immunodeficiency virus (HIV) infection is the greatest risk factor for PML. Other immunological diseases, including systemic sarcoidosis, have also been reported as risk factors for PML. Herein, we report a case of PML co-occurring with neurosarcoidosis. Early diagnosis using brain biopsy and appropriate therapeutic interventions achieved favorable outcomes. PML in patients with active intracranial neurosarcoidosis is extremely rare. We believe that it is important to perform brain biopsy at an early stage to allow diagnosis, even for central nervous system involvement with a progressive parenchymal lesion in patients with sarcoidosis, if PML is possible.

DOI： 10.3389/fimmu.2024.1447992

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Two cases of cerebral hemorrhage with ipsilateral hemichorea-hemiballism

Shinya Oginezawa, Shoichiro Ando, Yukiko Kinoshita, Shoko Aburatani, Ryota Kishi, Yutaka Otsu, Takanobu Ishiguro, Etsuji Saji, Masato Kanazawa, Osamu Onodera

Japanese Journal of Stroke 2024年

Japanese longitudinal biomarker study in progressive supranuclear palsy and corticobasal degeneration: Clinical features of the first registered patients and short-term follow-up analysis

Hiroshi Takigawa, Ritsuko Hanajima, Ikuko Aiba, Takayoshi Shimohata, Takahiko Tokuda, Mitsuya Morita, Osamu Onodera, Shigeo Murayama, Kazuko Hasegawa, Aya M. Tokumaru, Hisanori Kowa, Masato Kanazawa, Tameto Naoi, Kenji Nakashima, Takeshi Ikeuchi

Clinical Parkinsonism & Related Disorders 2024年

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