{"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"https://www.ncbi.nlm.nih.gov/pubmed/37739810","label":"url"},{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=403698","label":"url"}],"paper_title":{"en":"Prevalence, Clinical Profiles, and Prognosis of Stiff-Person Syndrome in a Japanese Nationwide Survey.","ja":"Prevalence, Clinical Profiles, and Prognosis of Stiff-Person Syndrome in a Japanese Nationwide Survey."},"authors":{"en":[{"name":"Matsui Naoko"},{"name":"Tanaka Keiko"},{"name":"Ishida Mitsuyo"},{"name":"Yamamoto Yohei"},{"name":"Matsubara Yuri"},{"name":"Saika Reiko"},{"name":"Iizuka Takahiro"},{"name":"Nakamura Koshi"},{"name":"Kuriyama Nagato"},{"name":"Matsui Makoto"},{"name":"Arisawa Kokichi"},{"name":"Nakamura Yosikazu"},{"name":"Kaji Ryuji"},{"name":"Kuwabara Satoshi"},{"name":"Izumi Yuishin"}],"ja":[{"name":"松井 尚子"},{"name":"Tanaka Keiko"},{"name":"Ishida Mitsuyo"},{"name":"Yamamoto Yohei"},{"name":"Matsubara Yuri"},{"name":"Saika Reiko"},{"name":"Iizuka Takahiro"},{"name":"Nakamura Koshi"},{"name":"Kuriyama Nagato"},{"name":"Matsui Makoto"},{"name":"有澤 孝吉"},{"name":"Nakamura Yosikazu"},{"name":"梶 龍兒"},{"name":"Kuwabara Satoshi"},{"name":"和泉 唯信"}]},"description":{"en":"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.","ja":"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."},"publication_date":"2023-09-22","publication_name":{"en":"Neurology® Neuroimmunology & Neuroinflammation","ja":"Neurology® Neuroimmunology & Neuroinflammation"},"volume":"Vol.10","number":"No.6","starting_page":"e200165","ending_page":"e200165","languages":["eng"],"referee":true,"identifiers":{"doi":["10.1212/NXI.0000000000200165"],"issn":["2332-7812"]},"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"https://www.ncbi.nlm.nih.gov/pubmed/36402755","label":"url"},{"@id":"https://www.scopus.com/record/display.url?eid=2-s2.0-85142267950&origin=inward","label":"url"},{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=401619","label":"url"}],"paper_title":{"en":"Pulmonary cancers across different histotypes share hybrid tuft cell/ionocyte-like molecular features and potentially druggable vulnerabilities","ja":"Pulmonary cancers across different histotypes share hybrid tuft cell/ionocyte-like molecular features and potentially druggable vulnerabilities"},"authors":{"en":[{"name":"Yamada Yosuke"},{"name":"Belharazem-Vitacolonnna Djeda"},{"name":"Bohnenberger Hanibal"},{"name":"Weiß Christel"},{"name":"Matsui Naoko"},{"name":"Kriegsmann Mark"},{"name":"Kriegsmann Katharina"},{"name":"Sinn Peter"},{"name":"Simon-Keller Katja"},{"name":"Hamilton Gerhard"},{"name":"Graeter Thomas"},{"name":"Preissler Gerhard"},{"name":"Ott German"},{"name":"Schölch Sebastian"},{"name":"Nakajima Naoki"},{"name":"Yoshizawa Akihiko"},{"name":"Haga Hironori"},{"name":"Date Hiroshi"},{"name":"Thomas K Roman"},{"name":"Petrini Iacopo"},{"name":"Giaccone Giuseppe"},{"name":"Ströbel Philipp"},{"name":"Marx Alexander"}],"ja":[{"name":"Yamada Yosuke"},{"name":"Belharazem-Vitacolonnna Djeda"},{"name":"Bohnenberger Hanibal"},{"name":"Weiß Christel"},{"name":"松井 尚子"},{"name":"Kriegsmann Mark"},{"name":"Kriegsmann Katharina"},{"name":"Sinn Peter"},{"name":"Simon-Keller Katja"},{"name":"Hamilton Gerhard"},{"name":"Graeter Thomas"},{"name":"Preissler Gerhard"},{"name":"Ott German"},{"name":"Schölch Sebastian"},{"name":"Nakajima Naoki"},{"name":"Yoshizawa Akihiko"},{"name":"Haga Hironori"},{"name":"Date Hiroshi"},{"name":"Thomas K Roman"},{"name":"Petrini Iacopo"},{"name":"Giaccone Giuseppe"},{"name":"Ströbel Philipp"},{"name":"Marx Alexander"}]},"publication_date":"2022-11-19","publication_name":{"en":"Cell Death & Disease","ja":"Cell Death & Disease"},"volume":"Vol.13","number":"No.11","starting_page":"979","ending_page":"979","languages":["eng"],"referee":true,"identifiers":{"doi":["10.1038/s41419-022-05428-x"],"issn":["2041-4889"]},"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=401627","label":"url"}],"paper_title":{"en":"A case of anti-NT5c1A antibody-seropositive inclusion body myositis associated with severe dysphagia and prominent forearm weakness","ja":"A case of anti-NT5c1A antibody-seropositive inclusion body myositis associated with severe dysphagia and prominent forearm weakness"},"authors":{"en":[{"name":"Matsui Naoko"},{"name":"Takahara Mika"},{"name":"Yamazaki Hiroki"},{"name":"Takamatsu Naoko"},{"name":"Osaki Yusuke"},{"name":"Kaji Ryuji"},{"name":"Nishino Ichizo"},{"name":"Yamashita Satoshi"},{"name":"Izumi Yuishin"}],"ja":[{"name":"松井 尚子"},{"name":"Takahara Mika"},{"name":"山﨑 博輝"},{"name":"Takamatsu Naoko"},{"name":"大崎 裕亮"},{"name":"梶 龍兒"},{"name":"Nishino Ichizo"},{"name":"Yamashita Satoshi"},{"name":"和泉 唯信"}]},"publication_date":"2022-10-24","publication_name":{"en":"Neurology and Clinical Neuroscience","ja":"Neurology and Clinical Neuroscience"},"volume":"Vol.11","number":"No.1","starting_page":"46","ending_page":"48","languages":["eng"],"referee":true,"identifiers":{"doi":["10.1111/ncn3.12678"],"issn":["2049-4173"]},"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"https://www.ncbi.nlm.nih.gov/pubmed/35235001","label":"url"},{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=401607","label":"url"}],"paper_title":{"en":"Complex hereditary peripheral neuropathies caused by novel variants in mitochondrial-related nuclear genes","ja":"Complex hereditary peripheral neuropathies caused by novel variants in mitochondrial-related nuclear genes"},"authors":{"en":[{"name":"Hiramatsu Yu"},{"name":"Okamoto Yuji"},{"name":"Yoshimura Akiko"},{"name":"Yuan JunHui"},{"name":"Ando Masahiro"},{"name":"Higuchi Yujiro"},{"name":"Hashiguchi Akihiro"},{"name":"Matsuura Eiji"},{"name":"Nozaki Fumihito"},{"name":"Kumada Tomohiro"},{"name":"Murayama Kei"},{"name":"Suzuki Mikiya"},{"name":"Yamamoto Yuki"},{"name":"Matsui Naoko"},{"name":"Miyazaki Yoshimichi"},{"name":"Yamaguchi Masamitsu"},{"name":"Suzuki Youji"},{"name":"Mitsui Jun"},{"name":"Ishiura Hiroyuki"},{"name":"Tanaka Masaki"},{"name":"Morishita Shinichi"},{"name":"Nishino Ichizo"},{"name":"Tsuji Shoji"},{"name":"Takashima Hiroshi"}],"ja":[{"name":"Hiramatsu Yu"},{"name":"Okamoto Yuji"},{"name":"Yoshimura Akiko"},{"name":"Yuan JunHui"},{"name":"Ando Masahiro"},{"name":"Higuchi Yujiro"},{"name":"Hashiguchi Akihiro"},{"name":"Matsuura Eiji"},{"name":"Nozaki Fumihito"},{"name":"Kumada Tomohiro"},{"name":"Murayama Kei"},{"name":"Suzuki Mikiya"},{"name":"山本 雄貴"},{"name":"松井 尚子"},{"name":"宮﨑 由道"},{"name":"Yamaguchi Masamitsu"},{"name":"Suzuki Youji"},{"name":"Mitsui Jun"},{"name":"Ishiura Hiroyuki"},{"name":"Tanaka Masaki"},{"name":"Morishita Shinichi"},{"name":"Nishino Ichizo"},{"name":"Tsuji Shoji"},{"name":"Takashima Hiroshi"}]},"description":{"en":"Mitochondrial disorders are a group of clinically and genetically heterogeneous multisystem disorders and peripheral neuropathy is frequently described in the context of mutations in mitochondrial-related nuclear genes. This study aimed to identify the causative mutations in mitochondrial-related nuclear genes in suspected hereditary peripheral neuropathy patients. We enrolled a large Japanese cohort of clinically suspected hereditary peripheral neuropathy patients who were mutation negative in the prescreening of the known Charcot-Marie-Tooth disease-causing genes. We performed whole-exome sequencing on 247 patients with autosomal recessive or sporadic inheritance for further analysis of 167 mitochondrial-related nuclear genes. We detected novel bi-allelic likely pathogenic/pathogenic variants in four patients, from four mitochondrial-related nuclear genes: pyruvate dehydrogenase beta-polypeptide (PDHB), mitochondrial poly(A) polymerase (MTPAP), hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase, beta subunit (HADHB), and succinate-CoA ligase ADP-forming beta subunit (SUCLA2). All these patients showed sensory and motor axonal polyneuropathy, combined with central nervous system or multisystem involvements. The pathological analysis of skeletal muscles revealed mild neurogenic changes without significant mitochondrial abnormalities. Targeted screening of mitochondria-related nuclear genes should be considered for patients with complex hereditary axonal polyneuropathy, accompanied by central nervous system dysfunctions, or with unexplainable multisystem disorders.","ja":"Mitochondrial disorders are a group of clinically and genetically heterogeneous multisystem disorders and peripheral neuropathy is frequently described in the context of mutations in mitochondrial-related nuclear genes. This study aimed to identify the causative mutations in mitochondrial-related nuclear genes in suspected hereditary peripheral neuropathy patients. We enrolled a large Japanese cohort of clinically suspected hereditary peripheral neuropathy patients who were mutation negative in the prescreening of the known Charcot-Marie-Tooth disease-causing genes. We performed whole-exome sequencing on 247 patients with autosomal recessive or sporadic inheritance for further analysis of 167 mitochondrial-related nuclear genes. We detected novel bi-allelic likely pathogenic/pathogenic variants in four patients, from four mitochondrial-related nuclear genes: pyruvate dehydrogenase beta-polypeptide (PDHB), mitochondrial poly(A) polymerase (MTPAP), hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase, beta subunit (HADHB), and succinate-CoA ligase ADP-forming beta subunit (SUCLA2). All these patients showed sensory and motor axonal polyneuropathy, combined with central nervous system or multisystem involvements. The pathological analysis of skeletal muscles revealed mild neurogenic changes without significant mitochondrial abnormalities. Targeted screening of mitochondria-related nuclear genes should be considered for patients with complex hereditary axonal polyneuropathy, accompanied by central nervous system dysfunctions, or with unexplainable multisystem disorders."},"publication_date":"2022-08","publication_name":{"en":"Journal of Neurology","ja":"Journal of Neurology"},"volume":"Vol.269","number":"No.8","starting_page":"4129","ending_page":"4140","languages":["eng"],"referee":true,"identifiers":{"doi":["10.1007/s00415-022-11026-w"],"issn":["1432-1459"]},"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"https://www.ncbi.nlm.nih.gov/pubmed/35672204","label":"url"},{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=396476","label":"url"}],"paper_title":{"en":"Application of ultrasound in a case of eosinophilic fasciitis mimicking stiff-person syndrome","ja":"Application of ultrasound in a case of eosinophilic fasciitis mimicking stiff-person syndrome"},"authors":{"en":[{"name":"Yamazaki Hiroki"},{"name":"Matsui Naoko"},{"name":"Takamatsu N"},{"name":"Yoshida Takeshi"},{"name":"Fukushima Koji"},{"name":"Takata T"},{"name":"Osaki Yusuke"},{"name":"Tanaka K"},{"name":"Kubo Y"},{"name":"Izumi Yuishin"}],"ja":[{"name":"山﨑 博輝"},{"name":"松井 尚子"},{"name":"Takamatsu N"},{"name":"吉田 剛"},{"name":"福島 功士"},{"name":"Takata T"},{"name":"大崎 裕亮"},{"name":"Tanaka K"},{"name":"Kubo Y"},{"name":"和泉 唯信"}]},"description":{"en":"Eosinophilic fasciitis (EF) is a rare disorder characterized by muscle stiffness mimicking other neuromuscular diseases. The diagnosis of EF is made on the basis of typical skin lesions. We report a case of a 36-year-old male patient with suspected stiff-person syndrome (SPS), who presented with progressive limb muscle stiffness and limited mobility of both wrists without obvious skin changes. Ultrasound revealed fascial thickening of bilateral upper and lower limb muscles and enlargement of hypoechoic tissues around the flexor digitorum tendons of the wrist. Skin and fascia biopsy confirmed the diagnosis of EF. Prednisolone therapy resulted in the improvement of muscle stiffness and tightness. Our findings suggest the need to consider connective tissue diseases such as EF in a patient with atypical features of SPS. Ultrasound is helpful for visualizing the causes of muscle stiffness and joint contractures in EF patients.","ja":"Eosinophilic fasciitis (EF) is a rare disorder characterized by muscle stiffness mimicking other neuromuscular diseases. The diagnosis of EF is made on the basis of typical skin lesions. We report a case of a 36-year-old male patient with suspected stiff-person syndrome (SPS), who presented with progressive limb muscle stiffness and limited mobility of both wrists without obvious skin changes. Ultrasound revealed fascial thickening of bilateral upper and lower limb muscles and enlargement of hypoechoic tissues around the flexor digitorum tendons of the wrist. Skin and fascia biopsy confirmed the diagnosis of EF. Prednisolone therapy resulted in the improvement of muscle stiffness and tightness. Our findings suggest the need to consider connective tissue diseases such as EF in a patient with atypical features of SPS. Ultrasound is helpful for visualizing the causes of muscle stiffness and joint contractures in EF patients."},"publication_date":"2022-07","publication_name":{"en":"Neuromuscular Disorders","ja":"Neuromuscular Disorders"},"volume":"Vol.32","number":"No.7","starting_page":"590","ending_page":"593","languages":["eng"],"referee":true,"identifiers":{"doi":["10.1016/j.nmd.2022.05.009"],"issn":["1873-2364"]},"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"https://repo.lib.tokushima-u.ac.jp/ja/116884","label":"url"},{"@id":"https://www.ncbi.nlm.nih.gov/pubmed/34561276","label":"url"},{"@id":"https://www.scopus.com/record/display.url?eid=2-s2.0-85117018419&origin=inward","label":"url"},{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=384892","label":"url"}],"paper_title":{"en":"Intrathymic Plasmablasts Are Affected in Patients With Myasthenia Gravis With Active Disease.","ja":"Intrathymic Plasmablasts Are Affected in Patients With Myasthenia Gravis With Active Disease."},"authors":{"en":[{"name":"Yamamoto Yohei"},{"name":"Matsui Naoko"},{"name":"Uzawa Akiyuki"},{"name":"Ozawa Yukiko"},{"name":"Kanai Tetsuya"},{"name":"Oda Fumiko"},{"name":"Kondo Hiroyuki"},{"name":"Ohigashi Izumi"},{"name":"Takizawa Hiromitsu"},{"name":"Kondo Kazuya"},{"name":"Sugano Mikio"},{"name":"Kitaichi Takashi"},{"name":"Hata Hiroki"},{"name":"Kaji Ryuji"},{"name":"Kuwabara Satoshi"},{"name":"Yamamura Takashi"},{"name":"Izumi Yuishin"}],"ja":[{"name":"Yamamoto Yohei"},{"name":"松井 尚子"},{"name":"Uzawa Akiyuki"},{"name":"Ozawa Yukiko"},{"name":"Kanai Tetsuya"},{"name":"Oda Fumiko"},{"name":"近藤 博之"},{"name":"大東 いずみ"},{"name":"滝沢 宏光"},{"name":"Kondo Kazuya"},{"name":"菅野 幹雄"},{"name":"北市 隆"},{"name":"Hata Hiroki"},{"name":"Kaji Ryuji"},{"name":"Kuwabara Satoshi"},{"name":"Yamamura Takashi"},{"name":"和泉 唯信"}]},"description":{"en":"Our findings confirmed a correlation between increased frequency of intrathymic plasmablasts and disease activity before thymectomy. We postulate that activated intrathymic plasmablasts endow pathogenic capacity in MG.","ja":"Our findings confirmed a correlation between increased frequency of intrathymic plasmablasts and disease activity before thymectomy. We postulate that activated intrathymic plasmablasts endow pathogenic capacity in MG."},"publication_date":"2021-09-24","publication_name":{"en":"Neurology® Neuroimmunology & Neuroinflammation","ja":"Neurology® Neuroimmunology & Neuroinflammation"},"volume":"Vol.8","number":"No.6","starting_page":"e1087","ending_page":"e1087","languages":["eng"],"referee":true,"identifiers":{"doi":["10.1212/NXI.0000000000001087"],"issn":["2332-7812"]},"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"http://id.ndl.go.jp/bib/031510247","label":"url"},{"@id":"https://cir.nii.ac.jp/crid/1523951030927469056/","label":"url"},{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=402624","label":"url"}],"paper_title":{"en":"Stiff-Person Syndrome","ja":"Stiff-Person Syndrome"},"authors":{"en":[{"name":"Matsui Naoko"},{"name":"田中 惠子"},{"name":"Izumi Yuishin"}],"ja":[{"name":"松井 尚子"},{"name":"田中 惠子"},{"name":"和泉 唯信"}]},"publication_date":"2021-05","publication_name":{"en":"Brain and Nerve = Shinkei kenkyū no shinpo","ja":"脳と神経 - 神経研究の進歩"},"volume":"Vol.73","number":"No.5","starting_page":"640","ending_page":"646","languages":["jpn"],"referee":true,"identifiers":{"issn":["1881-6096"]},"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"https://www.ncbi.nlm.nih.gov/pubmed/33450618","label":"url"},{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=377394","label":"url"}],"paper_title":{"en":"Reversible mixed perfusion on 123 I-IMP SPECT in anti-AMPA receptor encephalitis: A case report","ja":"Reversible mixed perfusion on 123 I-IMP SPECT in anti-AMPA receptor encephalitis: A case report"},"authors":{"en":[{"name":"Fukumoto T"},{"name":"Miyamoto Ryosuke"},{"name":"Fujita Koji"},{"name":"Murakami N"},{"name":"Matsui Naoko"},{"name":"Izumi Yuishin"}],"ja":[{"name":"福本 竜也"},{"name":"宮本 亮介"},{"name":"藤田 浩司"},{"name":"Murakami N"},{"name":"松井 尚子"},{"name":"和泉 唯信"}]},"publication_date":"2021-01-04","publication_name":{"en":"Journal of the Neurological Sciences","ja":"Journal of the Neurological Sciences"},"volume":"Vol.421","starting_page":"117306","ending_page":"117306","languages":["eng"],"referee":true,"identifiers":{"doi":["10.1016/j.jns.2020.117306"],"issn":["1878-5883"]},"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"https://repo.lib.tokushima-u.ac.jp/ja/116622","label":"url"},{"@id":"https://www.ncbi.nlm.nih.gov/pubmed/33017427","label":"url"},{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=377239","label":"url"}],"paper_title":{"en":"Prediction of improvement after extended thymectomy in non-thymomatous myasthenia gravis patients","ja":"Prediction of improvement after extended thymectomy in non-thymomatous myasthenia gravis patients"},"authors":{"en":[{"name":"Yoshida M"},{"name":"Kondo K"},{"name":"Matsui Naoko"},{"name":"Izumi Yuishin"},{"name":"Bando Y"},{"name":"Yokoishi M"},{"name":"Kajiura K"},{"name":"Tangoku Akira"}],"ja":[{"name":"Yoshida M"},{"name":"Kondo K"},{"name":"松井 尚子"},{"name":"和泉 唯信"},{"name":"Bando Y"},{"name":"Yokoishi M"},{"name":"Kajiura K"},{"name":"丹黒 章"}]},"description":{"en":"It is popularly believed that myasthenia gravis (MG) patients show acetylcholine receptor antibody (AChRAb) production associated with the thymus (germinal centers, approximately 80%). It has been suggested that thymectomy can remove the area of autoantibody production. This study aimed to determine whether the solid volume of the thymus calculated using three-dimensional (3D) imaging could be used to predict the efficacy of thymectomy. Additionally, the study assessed the relationships of the solid volume with germinal centers, change in the serum AChRAb level, postoperative MG improvement, and prednisolone (PSL) dose reduction extent. This retrospective study included 12 consecutive non-thymomatous MG patients (9 female and 3 male patients), who underwent extended thymectomy at our institution over the last 10 years. The mean patient age was 43.3 ± 14.2 years (range, 12-59 years). The study assessed the number of germinal centers per unit area, change in the serum AChRAb level, postoperative MG improvement, PSL dose reduction extent, and solid volume of the thymus. The number of germinal centers per unit area was significantly correlated with the solid volume of the thymus. The PSL dose reduction extent tended to be correlated with the solid volume. Our findings suggest that the solid volume of the thymus can possibly predict steroid dose reduction. Additionally, the solid volume of the thymus in 3D images is the most important indicator for predicting the efficacy of extended thymectomy.","ja":"It is popularly believed that myasthenia gravis (MG) patients show acetylcholine receptor antibody (AChRAb) production associated with the thymus (germinal centers, approximately 80%). It has been suggested that thymectomy can remove the area of autoantibody production. This study aimed to determine whether the solid volume of the thymus calculated using three-dimensional (3D) imaging could be used to predict the efficacy of thymectomy. Additionally, the study assessed the relationships of the solid volume with germinal centers, change in the serum AChRAb level, postoperative MG improvement, and prednisolone (PSL) dose reduction extent. This retrospective study included 12 consecutive non-thymomatous MG patients (9 female and 3 male patients), who underwent extended thymectomy at our institution over the last 10 years. The mean patient age was 43.3 ± 14.2 years (range, 12-59 years). The study assessed the number of germinal centers per unit area, change in the serum AChRAb level, postoperative MG improvement, PSL dose reduction extent, and solid volume of the thymus. The number of germinal centers per unit area was significantly correlated with the solid volume of the thymus. The PSL dose reduction extent tended to be correlated with the solid volume. Our findings suggest that the solid volume of the thymus can possibly predict steroid dose reduction. Additionally, the solid volume of the thymus in 3D images is the most important indicator for predicting the efficacy of extended thymectomy."},"publication_date":"2020-10-05","publication_name":{"en":"PLoS ONE","ja":"PLoS ONE"},"volume":"Vol.15","number":"No.10","starting_page":"e0239756","ending_page":"e0239756","languages":["eng"],"referee":true,"identifiers":{"doi":["10.1371/journal.pone.0239756"],"issn":["1932-6203"]},"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"https://www.ncbi.nlm.nih.gov/pubmed/29337417","label":"url"},{"@id":"https://www.scopus.com/record/display.url?eid=2-s2.0-85042582939&origin=inward","label":"url"},{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=339859","label":"url"}],"paper_title":{"en":"Guillan-Barre syndrome in local area in Japan, 2006-2015: an epidemiological and clinical study of 108 patients","ja":"Guillan-Barre syndrome in local area in Japan, 2006-2015: an epidemiological and clinical study of 108 patients"},"authors":{"en":[{"name":"Matsui Naoko"},{"name":"Nodera Hiroyuki"},{"name":"Kuzume D"},{"name":"Iwasa N"},{"name":"Unai Y"},{"name":"Sakai W"},{"name":"Miyazaki Y"},{"name":"Yamazaki H"},{"name":"Osaki Yusuke"},{"name":"Mori A"},{"name":"Furukawa T"},{"name":"Tsukamoto-Miyashiro A"},{"name":"Shimatani Y"},{"name":"Yamasaki M"},{"name":"Izumi Yuishin"},{"name":"Kusunoki S"},{"name":"Arisawa Kokichi"},{"name":"Kaji Ryuji"}],"ja":[{"name":"松井 尚子"},{"name":"野寺 裕之"},{"name":"Kuzume D"},{"name":"Iwasa N"},{"name":"Unai Y"},{"name":"Sakai W"},{"name":"Miyazaki Y"},{"name":"Yamazaki H"},{"name":"大崎 裕亮"},{"name":"Mori A"},{"name":"Furukawa T"},{"name":"Tsukamoto-Miyashiro A"},{"name":"Shimatani Y"},{"name":"Yamasaki M"},{"name":"和泉 唯信"},{"name":"Kusunoki S"},{"name":"有澤 孝吉"},{"name":"梶 龍兒"}]},"description":{"en":"Many epidemiological studies of Guillain-Barré syndrome (GBS) and Fisher syndrome (FS) have been conducted in Europe and America. In contrast, epidemiological studies are rare in Asia where the GBS subtypes differ from those in Western countries. This study was undertaken to clarify the incidence of GBS and FS in a local area in Japan as well as their seasonal trends. Seventy-one GBS and 37 FS patients were recorded from 2006 to 2015 in an area of approximately 1.5 million inhabitants in Japan. The incidence, seasonal trends and clinical features of GBS and FS were examined. The incidence rate of GBS was 0.42 cases per 100 000 person-years and that of FS was 0.22 cases per 100 000 person-years. The incidence of GBS increased with age and FS affected predominantly patients aged from 45 to 64 years old. There was some seasonal clustering of acute motor axonal neuropathy (AMAN) and FS in spring and summer, but it was not significant. AMAN and FS patients had a high frequency of preceding infection (AMAN, 68% gastrointestinal infection; FS, 65% upper respiratory infection). Antecedent respiratory infection was significantly associated with FS as an outcome. Serum antibodies to ganglioside GM1 were detected in 71% of AMAN patients and antibodies to GQ1b were detected in 81% of FS patients. Our study offers evidence of a lower incidence of GBS and a higher incidence of FS in a local area in Japan than in Western countries.","ja":"Many epidemiological studies of Guillain-Barré syndrome (GBS) and Fisher syndrome (FS) have been conducted in Europe and America. In contrast, epidemiological studies are rare in Asia where the GBS subtypes differ from those in Western countries. This study was undertaken to clarify the incidence of GBS and FS in a local area in Japan as well as their seasonal trends. Seventy-one GBS and 37 FS patients were recorded from 2006 to 2015 in an area of approximately 1.5 million inhabitants in Japan. The incidence, seasonal trends and clinical features of GBS and FS were examined. The incidence rate of GBS was 0.42 cases per 100 000 person-years and that of FS was 0.22 cases per 100 000 person-years. The incidence of GBS increased with age and FS affected predominantly patients aged from 45 to 64 years old. There was some seasonal clustering of acute motor axonal neuropathy (AMAN) and FS in spring and summer, but it was not significant. AMAN and FS patients had a high frequency of preceding infection (AMAN, 68% gastrointestinal infection; FS, 65% upper respiratory infection). Antecedent respiratory infection was significantly associated with FS as an outcome. Serum antibodies to ganglioside GM1 were detected in 71% of AMAN patients and antibodies to GQ1b were detected in 81% of FS patients. Our study offers evidence of a lower incidence of GBS and a higher incidence of FS in a local area in Japan than in Western countries."},"publication_date":"2018-05-25","publication_name":{"en":"European Journal of Neurology","ja":"European Journal of Neurology"},"volume":"Vol.25","number":"No.5","starting_page":"718","ending_page":"724","languages":["eng"],"referee":true,"identifiers":{"doi":["10.1111/ene.13569"],"issn":["1468-1331"]},"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=363872","label":"url"}],"paper_title":{"en":"未治療のサルコイドーシスに合併した進行性多巣性白質脳症","ja":"未治療のサルコイドーシスに合併した進行性多巣性白質脳症"},"authors":{"en":[{"name":"山上 圭"},{"name":"Furukawa Takahiro"},{"name":"Osaki Yusuke"},{"name":"村上 永尚"},{"name":"中道 一生"},{"name":"西條 政幸"},{"name":"Matsui Naoko"},{"name":"Izumi Yuishin"},{"name":"Kaji Ryuji"}],"ja":[{"name":"山上 圭"},{"name":"古川 貴大"},{"name":"大崎 裕亮"},{"name":"村上 永尚"},{"name":"中道 一生"},{"name":"西條 政幸"},{"name":"松井 尚子"},{"name":"和泉 唯信"},{"name":"梶 龍兒"}]},"publication_date":"2018-04-30","publication_name":{"en":"Neuroinfection","ja":"神経感染症"},"volume":"Vol.23","number":"No.1","starting_page":"151","ending_page":"156","languages":["jpn"],"referee":true,"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"https://www.ncbi.nlm.nih.gov/pubmed/29685815","label":"url"},{"@id":"https://www.scopus.com/record/display.url?eid=2-s2.0-85045902073&origin=inward","label":"url"},{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=345407","label":"url"}],"paper_title":{"en":"Safety and efficacy of eculizumab in Guillain-Barré syndrome: a multicentre, double-blind, randomised phase 2 trial.","ja":"Safety and efficacy of eculizumab in Guillain-Barré syndrome: a multicentre, double-blind, randomised phase 2 trial."},"authors":{"en":[{"name":"Sonoko Misawa"},{"name":"Satoshi Kuwabara"},{"name":"Yasunori Sato"},{"name":"Nobuko Yamaguchi"},{"name":"Kengo Nagashima"},{"name":"Kanako Katayama"},{"name":"Yukari Sekiguchi"},{"name":"Yuta Iwai"},{"name":"Hiroshi Amino"},{"name":"Tomoki Suichi"},{"name":"Takanori Yokota"},{"name":"Yoichiro Nishida"},{"name":"Tadashi Kanouchi"},{"name":"Nobuo Kohara"},{"name":"Michi Kawamoto"},{"name":"Junko Ishii"},{"name":"Motoi Kuwabara"},{"name":"Hidekazu Suzuki"},{"name":"Koichi Hirata"},{"name":"Norito Kokubun"},{"name":"Ray Masuda"},{"name":"Juntaro Kaneko"},{"name":"Ichiro Yabe"},{"name":"Hidenao Sasaki"},{"name":"Ken-ichi Kaida"},{"name":"Hiroshi Takazaki"},{"name":"Norihiro Suzuki"},{"name":"Shigeaki Suzuki"},{"name":"Nodera Hiroyuki"},{"name":"Matsui Naoko"},{"name":"Shoji Tsuji"},{"name":"Haruki Koike"},{"name":"Ryo Yamasaki"},{"name":"Susumu Kusunki"}],"ja":[{"name":"Sonoko Misawa"},{"name":"Satoshi Kuwabara"},{"name":"Yasunori Sato"},{"name":"Nobuko Yamaguchi"},{"name":"Kengo Nagashima"},{"name":"Kanako Katayama"},{"name":"Yukari Sekiguchi"},{"name":"Yuta Iwai"},{"name":"Hiroshi Amino"},{"name":"Tomoki Suichi"},{"name":"Takanori Yokota"},{"name":"Yoichiro Nishida"},{"name":"Tadashi Kanouchi"},{"name":"Nobuo Kohara"},{"name":"Michi Kawamoto"},{"name":"Junko Ishii"},{"name":"Motoi Kuwabara"},{"name":"Hidekazu Suzuki"},{"name":"Koichi Hirata"},{"name":"Norito Kokubun"},{"name":"Ray Masuda"},{"name":"Juntaro Kaneko"},{"name":"Ichiro Yabe"},{"name":"Hidenao Sasaki"},{"name":"Ken-ichi Kaida"},{"name":"Hiroshi Takazaki"},{"name":"Norihiro Suzuki"},{"name":"Shigeaki Suzuki"},{"name":"野寺 裕之"},{"name":"松井 尚子"},{"name":"Shoji Tsuji"},{"name":"Haruki Koike"},{"name":"Ryo Yamasaki"},{"name":"Susumu Kusunki"}]},"description":{"en":"Despite the introduction of plasmapheresis and immunoglobulin therapy, many patients with Guillain-Barré syndrome still have an incomplete recovery. Evidence from pathogenesis studies suggests the involvement of complement-mediated peripheral nerve damage. We aimed to investigate the safety and efficacy of eculizumab, a humanised monoclonal antibody against the complement protein C5, in patients with severe Guillain-Barré syndrome. This study was a 24 week, multicentre, double-blind, placebo-controlled, randomised phase 2 trial done at 13 hospitals in Japan. Eligible patients with Guillain-Barré syndrome were aged 18 years or older and could not walk independently (Guillain-Barré syndrome functional grade 3-5). Patients were randomly assigned (2:1) to receive 4 weeks of intravenous immunoglobulin plus either eculizumab (900 mg) or placebo; randomisation was done via a computer-generated process and web response system with minimisation for functional grade and age. The study had a parallel non-comparative single-arm outcome measure. The primary outcomes were efficacy (the proportion of patients with restored ability to walk independently [functional grade 2] at week 4) in the eculizumab group and safety in the full analysis set. For the efficacy endpoint, we predefined a response rate threshold of the lower 90% CI boundary exceeding 50%. This trial is registered with ClinicalTrials.gov, number, NCT02493725. Between Aug 10, 2015, and April 21, 2016, 34 patients were assigned to receive either eculizumab (n=23) or placebo (n=11). At week 4, the proportion of the patients able to walk independently (functional grade 2) was 61% (90% CI 42-78; n=14) in the eculizumab group, and 45% (20-73; n=5) in the placebo group. Adverse events occurred in all 34 patients. Three patients had serious adverse events: two in the eculizumab group (anaphylaxis in one patient and intracranial haemorrhage and abscess in another patient) and one in the placebo group (depression). The possibility that anaphylaxis and intracranial abscess were related to eculizumab could not be excluded. No deaths or meningococcal infections occurred. The primary outcome measure did not reach the predefined response rate. However, because this is a small study without statistical comparison with the placebo group, the efficacy and safety of eculizumab could be investigated in larger, randomised controlled trials. The Japan Agency for Medical Research and Development, Ministry of Health, Labor and Welfare, and Alexion Pharmaceuticals.","ja":"Despite the introduction of plasmapheresis and immunoglobulin therapy, many patients with Guillain-Barré syndrome still have an incomplete recovery. Evidence from pathogenesis studies suggests the involvement of complement-mediated peripheral nerve damage. We aimed to investigate the safety and efficacy of eculizumab, a humanised monoclonal antibody against the complement protein C5, in patients with severe Guillain-Barré syndrome. This study was a 24 week, multicentre, double-blind, placebo-controlled, randomised phase 2 trial done at 13 hospitals in Japan. Eligible patients with Guillain-Barré syndrome were aged 18 years or older and could not walk independently (Guillain-Barré syndrome functional grade 3-5). Patients were randomly assigned (2:1) to receive 4 weeks of intravenous immunoglobulin plus either eculizumab (900 mg) or placebo; randomisation was done via a computer-generated process and web response system with minimisation for functional grade and age. The study had a parallel non-comparative single-arm outcome measure. The primary outcomes were efficacy (the proportion of patients with restored ability to walk independently [functional grade 2] at week 4) in the eculizumab group and safety in the full analysis set. For the efficacy endpoint, we predefined a response rate threshold of the lower 90% CI boundary exceeding 50%. This trial is registered with ClinicalTrials.gov, number, NCT02493725. Between Aug 10, 2015, and April 21, 2016, 34 patients were assigned to receive either eculizumab (n=23) or placebo (n=11). At week 4, the proportion of the patients able to walk independently (functional grade 2) was 61% (90% CI 42-78; n=14) in the eculizumab group, and 45% (20-73; n=5) in the placebo group. Adverse events occurred in all 34 patients. Three patients had serious adverse events: two in the eculizumab group (anaphylaxis in one patient and intracranial haemorrhage and abscess in another patient) and one in the placebo group (depression). The possibility that anaphylaxis and intracranial abscess were related to eculizumab could not be excluded. No deaths or meningococcal infections occurred. The primary outcome measure did not reach the predefined response rate. However, because this is a small study without statistical comparison with the placebo group, the efficacy and safety of eculizumab could be investigated in larger, randomised controlled trials. The Japan Agency for Medical Research and Development, Ministry of Health, Labor and Welfare, and Alexion Pharmaceuticals."},"publication_date":"2018-04-21","publication_name":{"en":"The Lancet Neurology","ja":"The Lancet Neurology"},"volume":"Vol.17","number":"No.6","starting_page":"519","ending_page":"529","languages":["eng"],"referee":true,"identifiers":{"doi":["10.1016/S1474-4422(18)30114-5"],"issn":["1474-4465"]},"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"https://www.ncbi.nlm.nih.gov/pubmed/28694074","label":"url"},{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=328824","label":"url"}],"paper_title":{"en":"Memory B cell resurgence requires repeated rituximab in myasthenia gravis","ja":"Memory B cell resurgence requires repeated rituximab in myasthenia gravis"},"authors":{"en":[{"name":"Kohei Muto"},{"name":"Matsui Naoko"},{"name":"Yuki Unai"},{"name":"Waka Sakai"},{"name":"Shotaro Haji"},{"name":"Kengo Udaka"},{"name":"Miki Hirokazu"},{"name":"Furukawa Takahiro"},{"name":"Abe Masahiro"},{"name":"Kaji Ryuji"}],"ja":[{"name":"Kohei Muto"},{"name":"松井 尚子"},{"name":"Yuki Unai"},{"name":"酒井 和香"},{"name":"土師 正太郎"},{"name":"Kengo Udaka"},{"name":"三木 浩和"},{"name":"古川 貴大"},{"name":"安倍 正博"},{"name":"梶 龍兒"}]},"description":{"en":"The immunologic effects of rituximab (RTX) in myasthenia gravis (MG) remain to be explored. We aimed to clarify immunologic reactions and their association with response to RTX in MG. Regulatory T cell and B cell profiles of MG patients were monitored. Two patients presenting with generalized MG with anti-acetylcholine receptor antibodies were treated with RTX. The treatment led to sustained clinical improvement, discontinuation of intravenous immunoglobulin or plasma exchange, and reduction of prednisolone and other drugs. One patient was in remission for more than one year, whereas the other patient exhibited deterioration of symptoms within one year. Disease activity was associated with the repopulation of IgDCD27 and IgDCD27 memory B cells. Clinicians should be aware of the possibility that MG ranges in the duration of B cell depletion and additional RTX should be prescribed upon resurgence of memory B cells.","ja":"The immunologic effects of rituximab (RTX) in myasthenia gravis (MG) remain to be explored. We aimed to clarify immunologic reactions and their association with response to RTX in MG. Regulatory T cell and B cell profiles of MG patients were monitored. Two patients presenting with generalized MG with anti-acetylcholine receptor antibodies were treated with RTX. The treatment led to sustained clinical improvement, discontinuation of intravenous immunoglobulin or plasma exchange, and reduction of prednisolone and other drugs. One patient was in remission for more than one year, whereas the other patient exhibited deterioration of symptoms within one year. Disease activity was associated with the repopulation of IgDCD27 and IgDCD27 memory B cells. Clinicians should be aware of the possibility that MG ranges in the duration of B cell depletion and additional RTX should be prescribed upon resurgence of memory B cells."},"publication_date":"2017-06-21","publication_name":{"en":"Neuromuscular Disorders","ja":"Neuromuscular Disorders"},"volume":"Vol.27","number":"No.16","starting_page":"918","ending_page":"922","languages":["eng"],"referee":true,"identifiers":{"doi":["10.1016/j.nmd.2017.06.012"],"issn":["1873-2364"]},"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"https://www.ncbi.nlm.nih.gov/pubmed/28132977","label":"url"},{"@id":"https://www.scopus.com/record/display.url?eid=2-s2.0-85017414707&origin=inward","label":"url"},{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=328809","label":"url"}],"paper_title":{"en":"Mitochondrial trifunctional protein deficiency: an adult patient with similar progress to Charcot-Marie-Tooth disease.","ja":"シャルコー・マリー・トウース病に類似した三頭酵素欠損症の成人例"},"authors":{"en":[{"name":"Yamamoto Yuki"},{"name":"Matsui Naoko"},{"name":"平松 有"},{"name":"Miyazaki Yoshimichi"},{"name":"Nodera Hiroyuki"},{"name":"Izumi Yuishin"},{"name":"高嶋 博"},{"name":"Kaji Ryuji"}],"ja":[{"name":"山本 雄貴"},{"name":"松井 尚子"},{"name":"平松 有"},{"name":"宮﨑 由道"},{"name":"野寺 裕之"},{"name":"和泉 唯信"},{"name":"高嶋 博"},{"name":"梶 龍兒"}]},"description":{"en":"A 45-year-old man presented to us due to slowly progressive muscle weakness and sensory disturbances in his lower limbs since his 40's. He reported multiple episodes of exercise-induced severe muscle fatigue and brown urine in his childhood, which disappeared by age 20. A nerve conduction study showed peripheral axonal neuropathy and then Charcot-Marie-Tooth disease (CMT) was considered as the most likely diagnosis; however, exome sequencing failed to identify a mutation in the known genes of CMTs. Since age 55, he recurrently developed severe rhabdomyolysis that required hospitalization. On suspicion of lipid metabolism disorders, we performed serum acylcarnitine analysis, and which revealed mildly elevated long-chain fatty acids. We re-examined variants obtained via exome sequencing and found a mutation in HADHB. Mitochondrial trifunctional protein (MTP) deficiency is a rare autosomal recessive disorder of mitochondrial fatty acid beta-oxidation caused by HADHA or HADHB mutation. It can be a life-threatening multiorgan disorder with early infantile onset, but it can also present in childhood or adolescence with peripheral neuropathy and recurrent rhabdomyolysis. This case of adult-diagnosed MTP deficiency was characterized by slowly progressive peripheral neuropathy masquerading CMT in addition to muscular symptoms. MTP deficiency should be considered in patients with the combination of peripheral neuropathy and recurrent rhabdomyolysis.","ja":"A 45-year-old man presented to us due to slowly progressive muscle weakness and sensory disturbances in his lower limbs since his 40's. He reported multiple episodes of exercise-induced severe muscle fatigue and brown urine in his childhood, which disappeared by age 20. A nerve conduction study showed peripheral axonal neuropathy and then Charcot-Marie-Tooth disease (CMT) was considered as the most likely diagnosis; however, exome sequencing failed to identify a mutation in the known genes of CMTs. Since age 55, he recurrently developed severe rhabdomyolysis that required hospitalization. On suspicion of lipid metabolism disorders, we performed serum acylcarnitine analysis, and which revealed mildly elevated long-chain fatty acids. We re-examined variants obtained via exome sequencing and found a mutation in HADHB. Mitochondrial trifunctional protein (MTP) deficiency is a rare autosomal recessive disorder of mitochondrial fatty acid beta-oxidation caused by HADHA or HADHB mutation. It can be a life-threatening multiorgan disorder with early infantile onset, but it can also present in childhood or adolescence with peripheral neuropathy and recurrent rhabdomyolysis. This case of adult-diagnosed MTP deficiency was characterized by slowly progressive peripheral neuropathy masquerading CMT in addition to muscular symptoms. MTP deficiency should be considered in patients with the combination of peripheral neuropathy and recurrent rhabdomyolysis."},"publication_date":"2017-02","publication_name":{"en":"Clinical Neurology","ja":"臨床神経学"},"volume":"Vol.57","number":"No.2","starting_page":"82","ending_page":"87","languages":["jpn"],"referee":true,"identifiers":{"doi":["10.5692/clinicalneurol.cn-000976"],"issn":["1882-0654"]},"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"https://www.ncbi.nlm.nih.gov/pubmed/28132975","label":"url"},{"@id":"https://www.scopus.com/record/display.url?eid=2-s2.0-85017467963&origin=inward","label":"url"},{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=328807","label":"url"}],"paper_title":{"en":"A case of neuromyelitis optica spectrum disorder (NMOSD) with Sjögren's syndrome manifested only brain involvement by preceding parotitis.","ja":"耳下腺炎を契機に発症し,脳病変のみを反復したシェーグレン症候群合併視神経脊髄炎関連疾患の一例"},"authors":{"en":[{"name":"Furukawa Takahiro"},{"name":"Matsui Naoko"},{"name":"田中 惠子"},{"name":"Izumi Yuishin"},{"name":"Kaji Ryuji"}],"ja":[{"name":"古川 貴大"},{"name":"松井 尚子"},{"name":"田中 惠子"},{"name":"和泉 唯信"},{"name":"梶 龍兒"}]},"description":{"en":"A 33 year-old woman presented with intentional incontinence, motor aphasia, supranuclear gaze palsy, and spasticity after parotitis. Brain magnetic resonance images (MRI) showed abnormal signaling in long corticospinal tract involving internal capsules and cerebral peduncles, middle cerebellar peduncle, and frontal subcortical white matter lesions. She had a long history of dry eye and mouth. Immunoserological study showed that she was positive for anti-SS-A, aquaporin 4 (AQP4), and AQP5 antibodies. She clinically showed not only Sjögren's syndrome but also neuromyelitis optica spectrum disorder (NMOSD) without optic neuritis or myelitis. She responded to steroid followed by plasma exchange dramatically. Thereafter, the relapse of brain lesion was once detected while tapering of steroid, but her symptoms have been stable for several years after administration of immunosuppressant. This case suggested that salivary gland inflammation might be associated with the pathogenesis of NMOSD.","ja":"A 33 year-old woman presented with intentional incontinence, motor aphasia, supranuclear gaze palsy, and spasticity after parotitis. Brain magnetic resonance images (MRI) showed abnormal signaling in long corticospinal tract involving internal capsules and cerebral peduncles, middle cerebellar peduncle, and frontal subcortical white matter lesions. She had a long history of dry eye and mouth. Immunoserological study showed that she was positive for anti-SS-A, aquaporin 4 (AQP4), and AQP5 antibodies. She clinically showed not only Sjögren's syndrome but also neuromyelitis optica spectrum disorder (NMOSD) without optic neuritis or myelitis. She responded to steroid followed by plasma exchange dramatically. Thereafter, the relapse of brain lesion was once detected while tapering of steroid, but her symptoms have been stable for several years after administration of immunosuppressant. This case suggested that salivary gland inflammation might be associated with the pathogenesis of NMOSD."},"publication_date":"2017-02","publication_name":{"en":"Clinical Neurology","ja":"臨床神経学"},"volume":"Vol.57","number":"No.2","starting_page":"77","ending_page":"81","languages":["jpn"],"referee":true,"identifiers":{"doi":["10.5692/clinicalneurol.cn-000924"],"issn":["1882-0654"]},"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"https://repo.lib.tokushima-u.ac.jp/ja/111103","label":"url"},{"@id":"https://www.ncbi.nlm.nih.gov/pubmed/28373613","label":"url"},{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=329379","label":"url"}],"paper_title":{"en":"Influential factors on serum albumin concentration in hospitalized chronic kidney disease patients.","ja":"Influential factors on serum albumin concentration in hospitalized chronic kidney disease patients."},"authors":{"en":[{"name":"Yoshimoto Sakiya"},{"name":"Nagai Kojiro"},{"name":"Shibata Eriko"},{"name":"Ueda Sayo"},{"name":"Ono Hiroyuki"},{"name":"Tamaki Masanori"},{"name":"Nishimura Kenji"},{"name":"Obata Fumiaki"},{"name":"Inagaki Taizo"},{"name":"Minato Masanori"},{"name":"Kishi Fumi"},{"name":"Matsuura Motokazu"},{"name":"Matsui Naoko"},{"name":"Endo Itsuro"},{"name":"Hann Michael"},{"name":"Kishi Seiji"},{"name":"Murakami Taichi"},{"name":"Abe Hideharu"},{"name":"Doi Toshio"}],"ja":[{"name":"吉本 咲耶"},{"name":"長井 幸二郎"},{"name":"Shibata Eriko"},{"name":"Ueda Sayo"},{"name":"Ono Hiroyuki"},{"name":"Tamaki Masanori"},{"name":"Nishimura Kenji"},{"name":"Obata Fumiaki"},{"name":"Inagaki Taizo"},{"name":"Minato Masanori"},{"name":"Kishi Fumi"},{"name":"松浦 元一"},{"name":"松井 尚子"},{"name":"遠藤 逸朗"},{"name":"Hann Michael"},{"name":"岸 誠司"},{"name":"村上 太一"},{"name":"安部 秀斉"},{"name":"土井 俊夫"}]},"description":{"en":"SAC was affected by not only proteinuria, but also postural change, physical activity, and food in CKD patients. SAC should be analyzed by standardizing a patient's condition during phlebotomy. J. Med. Invest. 64: 146-152, February, 2017.","ja":"SAC was affected by not only proteinuria, but also postural change, physical activity, and food in CKD patients. SAC should be analyzed by standardizing a patient's condition during phlebotomy. J. Med. Invest. 64: 146-152, February, 2017."},"publication_date":"2017","publication_name":{"en":"The Journal of Medical Investigation : JMI","ja":"The Journal of Medical Investigation : JMI"},"volume":"Vol.64","number":"No.1.2","starting_page":"146","ending_page":"152","languages":["eng"],"referee":true,"identifiers":{"doi":["10.2152/jmi.64.146"],"issn":["1349-6867"]},"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"https://repo.lib.tokushima-u.ac.jp/ja/116229","label":"url"},{"@id":"https://www.ncbi.nlm.nih.gov/pubmed/28025469","label":"url"},{"@id":"https://www.scopus.com/record/display.url?eid=2-s2.0-85031294587&origin=inward","label":"url"},{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=328253","label":"url"}],"paper_title":{"en":"MR Spectroscopy in Patients with Hereditary Diffuse Leukoencephalopathy with Spheroids and Asymptomatic Carriers of Colony-stimulating Factor 1 Receptor Mutation.","ja":"MR Spectroscopy in Patients with Hereditary Diffuse Leukoencephalopathy with Spheroids and Asymptomatic Carriers of Colony-stimulating Factor 1 Receptor Mutation."},"authors":{"en":[{"name":"Abe Takashi"},{"name":"Kawarai Toshitaka"},{"name":"Fujita Koji"},{"name":"Sako Wataru"},{"name":"Terasawa Yuka"},{"name":"Matsuda Tsuyoshi"},{"name":"Sakai Waka"},{"name":"Tsukamoto-Miyashiro Ai"},{"name":"Matsui Naoko"},{"name":"Izumi Yuishin"},{"name":"Kaji Ryuji"},{"name":"Harada Masafumi"}],"ja":[{"name":"阿部 考志"},{"name":"瓦井 俊孝"},{"name":"藤田 浩司"},{"name":"佐光 亘"},{"name":"寺澤 由佳"},{"name":"Matsuda Tsuyoshi"},{"name":"Sakai Waka"},{"name":"Tsukamoto-Miyashiro Ai"},{"name":"松井 尚子"},{"name":"Izumi Yuishin"},{"name":"梶 龍兒"},{"name":"原田 雅史"}]},"description":{"en":"Hereditary diffuse leukoencephalopathy with spheroids (HDLS) is a rare neurodegenerative disorder with various clinical presentations. Mutation of the colony-stimulating factor 1 receptor (CSF1R) gene is considered to be a cause of this autosomal dominant disorder. The purpose of this study was to report magnetic resonance spectroscopy (MRS) findings in patients with HDLS and asymptomatic carriers and to clarify the use of MRS in this disease. In this retrospective, institutional review board-approved study, we included four consecutive patients, genetically diagnosed with HDLS, and two asymptomatic carriers after acquiring informed consent. We performed single-voxel MRS of the left centrum semiovale on a 3-T clinical scanner. We also included a sex-matched normal dataset. We quantified N-acetylaspartate (NAA), creatine, choline-containing compounds (Cho), glutamine, glutamate (Glu), myo-inositol (Ins), glutathione, lactate (Lac), and gamma-amino butyric acid using LCModel. We performed statistical analysis, and P value <0.05 was considered significant. In HDLS cases, MRS revealed decreased NAA and Glu concentrations, which probably reflected neuronal damage and/or loss, and a subsequent reduction of neurotransmitters. A patient with HDLS also had increased Cho and Ins concentrations, indicating gliosis, and increased Cho concentration was also observed in an asymptomatic carrier. This suggests that metabolic changes had already occurred in an asymptomatic state. We demonstrated changes in metabolite concentrations not only in patients with HDLS but also in asymptomatic CSF1R mutation carriers. Our study indicates that MRS is a potentially useful tool for the analysis of metabolic and pathophysiological findings of HDLS, even during the early stages of disease.","ja":"Hereditary diffuse leukoencephalopathy with spheroids (HDLS) is a rare neurodegenerative disorder with various clinical presentations. Mutation of the colony-stimulating factor 1 receptor (CSF1R) gene is considered to be a cause of this autosomal dominant disorder. The purpose of this study was to report magnetic resonance spectroscopy (MRS) findings in patients with HDLS and asymptomatic carriers and to clarify the use of MRS in this disease. In this retrospective, institutional review board-approved study, we included four consecutive patients, genetically diagnosed with HDLS, and two asymptomatic carriers after acquiring informed consent. We performed single-voxel MRS of the left centrum semiovale on a 3-T clinical scanner. We also included a sex-matched normal dataset. We quantified N-acetylaspartate (NAA), creatine, choline-containing compounds (Cho), glutamine, glutamate (Glu), myo-inositol (Ins), glutathione, lactate (Lac), and gamma-amino butyric acid using LCModel. We performed statistical analysis, and P value <0.05 was considered significant. In HDLS cases, MRS revealed decreased NAA and Glu concentrations, which probably reflected neuronal damage and/or loss, and a subsequent reduction of neurotransmitters. A patient with HDLS also had increased Cho and Ins concentrations, indicating gliosis, and increased Cho concentration was also observed in an asymptomatic carrier. This suggests that metabolic changes had already occurred in an asymptomatic state. We demonstrated changes in metabolite concentrations not only in patients with HDLS but also in asymptomatic CSF1R mutation carriers. Our study indicates that MRS is a potentially useful tool for the analysis of metabolic and pathophysiological findings of HDLS, even during the early stages of disease."},"publication_date":"2016-12-26","publication_name":{"en":"Magnetic Resonance in Medical Sciences","ja":"Magnetic Resonance in Medical Sciences"},"volume":"Vol.16","number":"No.4","starting_page":"297","ending_page":"303","languages":["eng"],"referee":true,"identifiers":{"doi":["10.2463/mrms.mp.2016-0016"],"issn":["1880-2206"]},"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"https://repo.lib.tokushima-u.ac.jp/ja/114517","label":"url"},{"@id":"https://www.ncbi.nlm.nih.gov/pubmed/27821140","label":"url"},{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=328820","label":"url"}],"paper_title":{"en":"Multicenter questionnaire survey for sporadic inclusion body myositis in Japan","ja":"Multicenter questionnaire survey for sporadic inclusion body myositis in Japan"},"authors":{"en":[{"name":"Suzuki Naoki"},{"name":"Mori-Yoshimura Madoka"},{"name":"Yamashita Satoshi"},{"name":"Nakano Satoshi"},{"name":"Murata Ken-ya"},{"name":"Inamori Yukie"},{"name":"Matsui Naoko"},{"name":"Kimura En"},{"name":"Kusaka Hirofumi"},{"name":"Kondo Tomoyoshi"},{"name":"Higuchi Itsuro"},{"name":"Kaji Ryuji"},{"name":"Tateyama Maki"},{"name":"Izumi Rumiko"},{"name":"Ono Hiroya"},{"name":"Kato Masaaki"},{"name":"Warita Hitoshi"},{"name":"Takahashi Toshiaki"},{"name":"Nishino Ichizo"},{"name":"Aoki Masashi"}],"ja":[{"name":"Suzuki Naoki"},{"name":"Mori-Yoshimura Madoka"},{"name":"Yamashita Satoshi"},{"name":"Nakano Satoshi"},{"name":"Murata Ken-ya"},{"name":"Inamori Yukie"},{"name":"松井 尚子"},{"name":"Kimura En"},{"name":"Kusaka Hirofumi"},{"name":"Kondo Tomoyoshi"},{"name":"Higuchi Itsuro"},{"name":"梶 龍兒"},{"name":"Tateyama Maki"},{"name":"Izumi Rumiko"},{"name":"Ono Hiroya"},{"name":"Kato Masaaki"},{"name":"Warita Hitoshi"},{"name":"Takahashi Toshiaki"},{"name":"Nishino Ichizo"},{"name":"Aoki Masashi"}]},"description":{"en":"Sporadic inclusion body myositis (sIBM) is the most prevalent acquired muscle disease in the elderly. sIBM is an intractable and progressive disease of unknown cause and without effective treatment. The etiology of sIBM is still unknown; however, genetic factors, aging, lifestyles, and environmental factors may be involved. The purpose of this study is to elucidate the cross-sectional profile of patients affected by sIBM in Japan. We surveyed patient data for 146 cases diagnosed at a number of centers across Japan. We also issued a questionnaire for 67 patients and direct caregivers to further elucidate the natural history of the disease. The mean age at the onset was 63.4 ± 9.2 years. The mean length of time from the onset to diagnosis was 55.52 ± 49.72 months, suggesting that there is a difficulty in diagnosing this disease with long-term consequences because of late treatment. 73 % described the psychological/mental aspect of the disease. The most popular primary caregiver was the patient's spouse and 57 % patients mentioned that they were having problems managing the finances. Through these surveys, we described the cross-sectional profiles of sIBM in Japan. Many patients described psychological/mental and financial anxiety because of the aged profile of sIBM patients. The profiles of sIBM patients are similar to those in Western countries.","ja":"Sporadic inclusion body myositis (sIBM) is the most prevalent acquired muscle disease in the elderly. sIBM is an intractable and progressive disease of unknown cause and without effective treatment. The etiology of sIBM is still unknown; however, genetic factors, aging, lifestyles, and environmental factors may be involved. The purpose of this study is to elucidate the cross-sectional profile of patients affected by sIBM in Japan. We surveyed patient data for 146 cases diagnosed at a number of centers across Japan. We also issued a questionnaire for 67 patients and direct caregivers to further elucidate the natural history of the disease. The mean age at the onset was 63.4 ± 9.2 years. The mean length of time from the onset to diagnosis was 55.52 ± 49.72 months, suggesting that there is a difficulty in diagnosing this disease with long-term consequences because of late treatment. 73 % described the psychological/mental aspect of the disease. The most popular primary caregiver was the patient's spouse and 57 % patients mentioned that they were having problems managing the finances. Through these surveys, we described the cross-sectional profiles of sIBM in Japan. Many patients described psychological/mental and financial anxiety because of the aged profile of sIBM patients. The profiles of sIBM patients are similar to those in Western countries."},"publication_date":"2016-11-08","publication_name":{"en":"Orphanet Journal of Rare Diseases","ja":"Orphanet Journal of Rare Diseases"},"volume":"Vol.11","number":"No.1","starting_page":"146","ending_page":"146","languages":["eng"],"referee":true,"identifiers":{"doi":["10.1186/s13023-016-0524-x"],"issn":["1750-1172"]},"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"https://repo.lib.tokushima-u.ac.jp/ja/112325","label":"url"},{"@id":"https://www.ncbi.nlm.nih.gov/pubmed/27659682","label":"url"},{"@id":"https://www.scopus.com/record/display.url?eid=2-s2.0-84988568998&origin=inward","label":"url"},{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=320770","label":"url"}],"paper_title":{"en":"Transmission of survival signals through Delta-like 1 on activated CD4(+) T cells.","ja":"Transmission of survival signals through Delta-like 1 on activated CD4(+) T cells."},"authors":{"en":[{"name":"Furukawa Takahiro"},{"name":"Ishifune Chieko"},{"name":"Tsukumo Shin-ichi"},{"name":"Hozumi Katsuto"},{"name":"Maekawa Yoichi"},{"name":"Matsui Naoko"},{"name":"Kaji Ryuji"},{"name":"Yasutomo Koji"}],"ja":[{"name":"Furukawa Takahiro"},{"name":"石舟 智恵子"},{"name":"九十九 伸一"},{"name":"Hozumi Katsuto"},{"name":"前川 洋一"},{"name":"松井 尚子"},{"name":"梶 龍兒"},{"name":"安友 康二"}]},"description":{"en":"Notch expressed on CD4(+) T cells transduces signals that mediate their effector functions and survival. Although Notch signaling is known to be cis-inhibited by Notch ligands expressed on the same cells, the role of Notch ligands on T cells remains unclear. In this report we demonstrate that the CD4(+) T cell Notch ligand Dll1 transduces signals required for their survival. Co-transfer of CD4(+) T cells from Dll1(-/-) and control mice into recipient mice followed by immunization revealed a rapid decline of CD4(+) T cells from Dll1(-/-) mice compared with control cells. Dll1(-/-) mice exhibited lower clinical scores of experimental autoimmune encephalitis than control mice. The expression of Notch target genes in CD4(+) T cells from Dll1(-/-) mice was not affected, suggesting that Dll1 deficiency in T cells does not affect cis Notch signaling. Overexpression of the intracellular domain of Dll1 in Dll1-deficient CD4(+) T cells partially rescued impaired survival. Our data demonstrate that Dll1 is an independent regulator of Notch-signaling important for the survival of activated CD4(+) T cells, and provide new insight into the physiological roles of Notch ligands as well as a regulatory mechanism important for maintaining adaptive immune responses.","ja":"Notch expressed on CD4(+) T cells transduces signals that mediate their effector functions and survival. Although Notch signaling is known to be cis-inhibited by Notch ligands expressed on the same cells, the role of Notch ligands on T cells remains unclear. In this report we demonstrate that the CD4(+) T cell Notch ligand Dll1 transduces signals required for their survival. Co-transfer of CD4(+) T cells from Dll1(-/-) and control mice into recipient mice followed by immunization revealed a rapid decline of CD4(+) T cells from Dll1(-/-) mice compared with control cells. Dll1(-/-) mice exhibited lower clinical scores of experimental autoimmune encephalitis than control mice. The expression of Notch target genes in CD4(+) T cells from Dll1(-/-) mice was not affected, suggesting that Dll1 deficiency in T cells does not affect cis Notch signaling. Overexpression of the intracellular domain of Dll1 in Dll1-deficient CD4(+) T cells partially rescued impaired survival. Our data demonstrate that Dll1 is an independent regulator of Notch-signaling important for the survival of activated CD4(+) T cells, and provide new insight into the physiological roles of Notch ligands as well as a regulatory mechanism important for maintaining adaptive immune responses."},"publication_date":"2016-09-23","publication_name":{"en":"Scientific Reports","ja":"Scientific Reports"},"volume":"Vol.6","starting_page":"33692","ending_page":"33692","languages":["eng"],"referee":true,"identifiers":{"doi":["10.1038/srep33692"],"issn":["2045-2322"]},"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"http://ci.nii.ac.jp/naid/130005950164/","label":"url"},{"@id":"https://cir.nii.ac.jp/crid/1390282681425704832/","label":"url"},{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=328806","label":"url"}],"paper_title":{"en":"A Case of Anti-SRP Positive Immune-mediated Necrotizing Myopathy Treated Effectively with Combined Immunotherapy","ja":"複合的免疫治療が奏功した抗SRP抗体陽性壊死性ミオパチーの1例"},"authors":{"en":[{"name":"山下 雄也"},{"name":"Yamazaki Hiroki"},{"name":"Matsui Naoko"},{"name":"Miyazaki Yoshimichi"},{"name":"塚本 愛"},{"name":"西野 一三"},{"name":"鈴木 重明"},{"name":"西田 善彦"},{"name":"Nodera Hiroyuki"},{"name":"Izumi Yuishin"},{"name":"Kaji Ryuji"}],"ja":[{"name":"山下 雄也"},{"name":"山崎 博輝"},{"name":"松井 尚子"},{"name":"宮﨑 由道"},{"name":"塚本 愛"},{"name":"西野 一三"},{"name":"鈴木 重明"},{"name":"西田 善彦"},{"name":"野寺 裕之"},{"name":"和泉 唯信"},{"name":"梶 龍兒"}]},"description":{"en":"
症例は60歳代,男性.全身倦怠感,四肢脱力を主訴に受診.筋炎としステロイド単独で治療を開始したが,反応性は乏しく,筋病理・抗体検査にて抗SRP抗体陽性壊死性ミオパチー(anti-signal recognition particle immune-mediated necrotizing myopathy:anti-SRP iNM)と診断し,免疫グロブリン大量静注療法,免疫抑制薬を併用したところ,症状の改善を得た.積極的に自己抗体の測定を行うことが壊死性ミオパチー(immune-mediated necrotizing myopathy:iNM)の早期診断・治療に重要である.
","ja":"症例は60歳代,男性.全身倦怠感,四肢脱力を主訴に受診.筋炎としステロイド単独で治療を開始したが,反応性は乏しく,筋病理・抗体検査にて抗SRP抗体陽性壊死性ミオパチー(anti-signal recognition particle immune-mediated necrotizing myopathy:anti-SRP iNM)と診断し,免疫グロブリン大量静注療法,免疫抑制薬を併用したところ,症状の改善を得た.積極的に自己抗体の測定を行うことが壊死性ミオパチー(immune-mediated necrotizing myopathy:iNM)の早期診断・治療に重要である.
"},"publication_date":"2016-08","publication_name":{"en":"The Journal of the Japanese Society of Internal Medicine","ja":"日本内科学会雑誌"},"volume":"Vol.105","number":"No.8","starting_page":"1443","ending_page":"1450","languages":["jpn"],"referee":true,"identifiers":{"doi":["10.2169/naika.105.1443"],"issn":["1883-2083"]},"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"https://repo.lib.tokushima-u.ac.jp/ja/110923","label":"url"},{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=310575","label":"url"}],"paper_title":{"en":"Late-onset myasthenia gravis is predisposed to become generalized in the elderly","ja":"Late-onset myasthenia gravis is predisposed to become generalized in the elderly"},"authors":{"en":[{"name":"Sakai Waka"},{"name":"Matsui Naoko"},{"name":"Ishida Mitsuyo"},{"name":"Furukawa Takahiro"},{"name":"Miyazaki Yoshimichi"},{"name":"Fujita Koji"},{"name":"Miyamoto Ryosuke"},{"name":"Yamamoto Nobuaki"},{"name":"Sako Wataru"},{"name":"Sato Kenta"},{"name":"Kondo Kazuya"},{"name":"Nishida Yoshihiko"},{"name":"Mitsui Takao"},{"name":"Izumi Yuishin"},{"name":"Kaji Ryuji"}],"ja":[{"name":"酒井 和香"},{"name":"松井 尚子"},{"name":"Ishida Mitsuyo"},{"name":"古川 貴大"},{"name":"宮﨑 由道"},{"name":"藤田 浩司"},{"name":"宮本 亮介"},{"name":"山本 伸昭"},{"name":"佐光 亘"},{"name":"佐藤 健太"},{"name":"近藤 和也"},{"name":"西田 善彦"},{"name":"三ツ井 貴夫"},{"name":"和泉 唯信"},{"name":"梶 龍兒"}]},"publication_date":"2016-02-11","publication_name":{"en":"eNeurologicalSci","ja":"eNeurologicalSci"},"volume":"Vol.2","starting_page":"17","ending_page":"20","languages":["eng"],"referee":true,"identifiers":{"doi":["10.1016/j.ensci.2016.02.004"],"issn":["2405-6502"]},"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=328816","label":"url"}],"paper_title":{"en":"Multimodal analysis based on high-field magnetic resonance and motor evoked potentials: A case report of multiple sclerosis","ja":"Multimodal analysis based on high-field magnetic resonance and motor evoked potentials: A case report of multiple sclerosis"},"authors":{"en":[{"name":"Nakane Shunya"},{"name":"Furutani Kaori"},{"name":"Harada Masafumi"},{"name":"Urushihara Ryo"},{"name":"Matsui Naoko"},{"name":"Izumi Yuishin"},{"name":"Kaji Ryuji"}],"ja":[{"name":"中根 俊成"},{"name":"古谷 かおり"},{"name":"原田 雅史"},{"name":"Urushihara Ryo"},{"name":"松井 尚子"},{"name":"和泉 唯信"},{"name":"梶 龍兒"}]},"publication_date":"2016-01","publication_name":{"en":"Clinical & Experimental Neuroimmunology","ja":"Clinical & Experimental Neuroimmunology"},"volume":"Vol.8","number":"No.1","starting_page":"43","ending_page":"46","languages":["eng"],"referee":true,"identifiers":{"doi":["10.1111/cen3.12352"],"issn":["1759-1961"]},"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"https://www.scopus.com/record/display.url?eid=2-s2.0-84961266632&origin=inward","label":"url"},{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=328815","label":"url"}],"paper_title":{"en":"Ocular myasthenia gravis with anti-muscle-specific tyrosine kinase antibodies: Two new cases and a systematic literature review","ja":"Ocular myasthenia gravis with anti-muscle-specific tyrosine kinase antibodies: Two new cases and a systematic literature review"},"authors":{"en":[{"name":"Kamata Masaki"},{"name":"Nakane Shunya"},{"name":"Matsui Naoko"},{"name":"Higuchi Osamu"},{"name":"Waka Sakai"},{"name":"Fujita Koji"},{"name":"Izumi Yuishin"},{"name":"Matsuo Hidenori"},{"name":"Kaji Ryuji"}],"ja":[{"name":"Kamata Masaki"},{"name":"中根 俊成"},{"name":"松井 尚子"},{"name":"Higuchi Osamu"},{"name":"Waka Sakai"},{"name":"藤田 浩司"},{"name":"和泉 唯信"},{"name":"Matsuo Hidenori"},{"name":"梶 龍兒"}]},"publication_date":"2016-01","publication_name":{"en":"Clinical & Experimental Neuroimmunology","ja":"Clinical & Experimental Neuroimmunology"},"volume":"Vol.7","number":"No.2","starting_page":"168","ending_page":"173","languages":["eng"],"referee":true,"identifiers":{"doi":["10.1111/cen3.12296"],"issn":["1759-1961"]},"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"https://www.ncbi.nlm.nih.gov/pubmed/26706399","label":"url"},{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=315938","label":"url"}],"paper_title":{"en":"Intramuscular dissociation of echogenicity in the triceps surae characterizes sporadic inclusion body myositis","ja":"Intramuscular dissociation of echogenicity in the triceps surae characterizes sporadic inclusion body myositis"},"authors":{"en":[{"name":"Nodera Hiroyuki"},{"name":"Takamatsu Naoko"},{"name":"Matsui Naoko"},{"name":"Mori Atsuko"},{"name":"Terasawa Yuka"},{"name":"Shimatani Yoshimitsu"},{"name":"Osaki Yusuke"},{"name":"Maruyama Keiko"},{"name":"Izumi Yuishin"},{"name":"Kaji Ryuji"}],"ja":[{"name":"野寺 裕之"},{"name":"Takamatsu Naoko"},{"name":"松井 尚子"},{"name":"Mori Atsuko"},{"name":"寺澤 由佳"},{"name":"島谷 佳光"},{"name":"Osaki Yusuke"},{"name":"Maruyama Keiko"},{"name":"和泉 唯信"},{"name":"梶 龍兒"}]},"description":{"en":"Differential diagnosis of sporadic inclusion body myositis (s-IBM) and polymyositis (PM)/dermatomyositis (DM) is difficult and can affect proper disease management. Detection of heterogeneous muscular involvement in s-IBM by muscle sonography could be a unique diagnostic feature. Sonography of the lower leg and forearm was performed in patients with s-IBM, PM/DM and control subjects (n = 11 each). Echo intensities (EIs) of the adjacent muscles [medial head of the gastrocnemius versus soleus and the flexor digitorum profundus (FDP) versus flexor carpi ulnaris (FCU)] were scored by three blinded raters. The mean EIs of these muscles were compared using computer-assisted histogram analysis. Both evaluation methods showed high echoic signals in the gastrocnemius of patients with s-IBM. EIs were significantly different between the gastrocnemius and soleus in patients with s-IBM, but not in those with DM/PM and the controls. In the forearm, although the EI of the FDP was higher in the s-IBM group than in the other groups, the EI differences between the FDP and FCU did not differ significantly between disease groups. The difference in area under the curves to differentiate between s-IBM and DM/PM was greatest between the gastrocnemius-soleus EIs (0.843; P = 0.006). High echoic signals in the medial gastrocnemius compared with those of the soleus are suggestive of s-IBM over PM/DM.","ja":"Differential diagnosis of sporadic inclusion body myositis (s-IBM) and polymyositis (PM)/dermatomyositis (DM) is difficult and can affect proper disease management. Detection of heterogeneous muscular involvement in s-IBM by muscle sonography could be a unique diagnostic feature. Sonography of the lower leg and forearm was performed in patients with s-IBM, PM/DM and control subjects (n = 11 each). Echo intensities (EIs) of the adjacent muscles [medial head of the gastrocnemius versus soleus and the flexor digitorum profundus (FDP) versus flexor carpi ulnaris (FCU)] were scored by three blinded raters. The mean EIs of these muscles were compared using computer-assisted histogram analysis. Both evaluation methods showed high echoic signals in the gastrocnemius of patients with s-IBM. EIs were significantly different between the gastrocnemius and soleus in patients with s-IBM, but not in those with DM/PM and the controls. In the forearm, although the EI of the FDP was higher in the s-IBM group than in the other groups, the EI differences between the FDP and FCU did not differ significantly between disease groups. The difference in area under the curves to differentiate between s-IBM and DM/PM was greatest between the gastrocnemius-soleus EIs (0.843; P = 0.006). High echoic signals in the medial gastrocnemius compared with those of the soleus are suggestive of s-IBM over PM/DM."},"publication_date":"2016","publication_name":{"en":"European Journal of Neurology","ja":"European Journal of Neurology"},"volume":"Vol.23","number":"No.3","starting_page":"588","ending_page":"596","languages":["eng"],"referee":true,"identifiers":{"doi":["10.1111/ene.12899"],"issn":["1468-1331"]},"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"https://www.ncbi.nlm.nih.gov/pubmed/26671123","label":"url"},{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=307136","label":"url"}],"paper_title":{"en":"Late-onset spastic paraplegia: Aberrant SPG11 transcripts generated by a novel splice site donor mutation.","ja":"Late-onset spastic paraplegia: Aberrant SPG11 transcripts generated by a novel splice site donor mutation."},"authors":{"en":[{"name":"Kawarai Toshitaka"},{"name":"Miyamoto Ryosuke"},{"name":"Mori Atsuko"},{"name":"Oki Ryosuke"},{"name":"Tsukamoto-Miyashiro Ai"},{"name":"Matsui Naoko"},{"name":"Miyazaki Yoshimichi"},{"name":"Orlacchio Antonio"},{"name":"Izumi Yuishin"},{"name":"Nishida Yoshihiko"},{"name":"Kaji Ryuji"}],"ja":[{"name":"瓦井 俊孝"},{"name":"Miyamoto Ryosuke"},{"name":"Mori Atsuko"},{"name":"Oki Ryosuke"},{"name":"Tsukamoto-Miyashiro Ai"},{"name":"松井 尚子"},{"name":"宮﨑 由道"},{"name":"Orlacchio Antonio"},{"name":"和泉 唯信"},{"name":"Nishida Yoshihiko"},{"name":"梶 龍兒"}]},"description":{"en":"We identified a novel homozygous mutation in the splice site donor (SSD) of intron 30 (c.5866+1G>A) in consanguineous Japanese SPG11 siblings showing late-onset spastic paraplegia using the whole-exome sequencing. Phenotypic variability was observed, including age-at-onset, dysarthria and pes cavus. Coding DNA sequencing revealed that the mutation affected the recognition of the constitutive SSD of intron 30, splicing upstream onto a nearby cryptic SSD in exon 30. The use of constitutive splice sites of intron 29 was confirmed by sequencing. The mutant transcripts are mostly subject to degradation by the nonsense-mediated mRNA decay system. SPG11 transcripts, escaping from the nonsense-mediated mRNA decay pathway, would generate a truncated protein (p.Tyr1900Phefs5X) containing the first 1899 amino acids and followed by 4 aberrant amino acids. This study showed a successful clinical application of whole-exome sequencing in spastic paraplegia and demonstrated a further evidence of allelic heterogeneity in SPG11. The confirmation of aberrant transcript by splice site mutation is a prerequisite for a more precise molecular diagnosis.","ja":"We identified a novel homozygous mutation in the splice site donor (SSD) of intron 30 (c.5866+1G>A) in consanguineous Japanese SPG11 siblings showing late-onset spastic paraplegia using the whole-exome sequencing. Phenotypic variability was observed, including age-at-onset, dysarthria and pes cavus. Coding DNA sequencing revealed that the mutation affected the recognition of the constitutive SSD of intron 30, splicing upstream onto a nearby cryptic SSD in exon 30. The use of constitutive splice sites of intron 29 was confirmed by sequencing. The mutant transcripts are mostly subject to degradation by the nonsense-mediated mRNA decay system. SPG11 transcripts, escaping from the nonsense-mediated mRNA decay pathway, would generate a truncated protein (p.Tyr1900Phefs5X) containing the first 1899 amino acids and followed by 4 aberrant amino acids. This study showed a successful clinical application of whole-exome sequencing in spastic paraplegia and demonstrated a further evidence of allelic heterogeneity in SPG11. The confirmation of aberrant transcript by splice site mutation is a prerequisite for a more precise molecular diagnosis."},"publication_date":"2015-10-30","publication_name":{"en":"Journal of the Neurological Sciences","ja":"Journal of the Neurological Sciences"},"volume":"Vol.359","number":"No.1-2","starting_page":"250","ending_page":"255","languages":["eng"],"referee":true,"identifiers":{"doi":["10.1016/j.jns.2015.10.045"],"issn":["1878-5883"]},"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"https://repo.lib.tokushima-u.ac.jp/ja/109981","label":"url"},{"@id":"https://www.ncbi.nlm.nih.gov/pubmed/26280014","label":"url"},{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=303186","label":"url"}],"paper_title":{"en":"CSF cytokine profile distinguishes multifocal motor neuropathy from progressive muscular atrophy.","ja":"CSF cytokine profile distinguishes multifocal motor neuropathy from progressive muscular atrophy."},"authors":{"en":[{"name":"Furukawa Takahiro"},{"name":"Matsui Naoko"},{"name":"Fujita Koji"},{"name":"Nodera Hiroyuki"},{"name":"Shimizu Fumitaka"},{"name":"Miyamoto Katsuichi"},{"name":"Takahashi Yukitoshi"},{"name":"Kanda Takashi"},{"name":"Kusunoki Susumu"},{"name":"Izumi Yuishin"},{"name":"Kaji Ryuji"}],"ja":[{"name":"Furukawa Takahiro"},{"name":"松井 尚子"},{"name":"藤田 浩司"},{"name":"野寺 裕之"},{"name":"Shimizu Fumitaka"},{"name":"Miyamoto Katsuichi"},{"name":"Takahashi Yukitoshi"},{"name":"Kanda Takashi"},{"name":"Kusunoki Susumu"},{"name":"和泉 唯信"},{"name":"梶 龍兒"}]},"description":{"en":"The CSF cytokine profile of patients with MMN is distinct from that of patients with PMA and ALS. The similarity of the cytokine profiles between patients with PMA and ALS suggests that PMA shares common immunologic features with ALS in the CNS, even without clinical evidence of upper motor neuron involvement.","ja":"The CSF cytokine profile of patients with MMN is distinct from that of patients with PMA and ALS. The similarity of the cytokine profiles between patients with PMA and ALS suggests that PMA shares common immunologic features with ALS in the CNS, even without clinical evidence of upper motor neuron involvement."},"publication_date":"2015-08-06","publication_name":{"en":"Neurology® Neuroimmunology & Neuroinflammation","ja":"Neurology® Neuroimmunology & Neuroinflammation"},"volume":"Vol.2","number":"No.5","starting_page":"e138","ending_page":"e138","languages":["eng"],"referee":true,"identifiers":{"doi":["10.1212/NXI.0000000000000138"],"issn":["2332-7812"]},"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"https://www.ncbi.nlm.nih.gov/pubmed/25130929","label":"url"},{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=284689","label":"url"}],"paper_title":{"en":"Increased proinflammatory cytokines in sera of patients with multifocal motor neuropathy.","ja":"Increased proinflammatory cytokines in sera of patients with multifocal motor neuropathy."},"authors":{"en":[{"name":"Furukawa Takahiro"},{"name":"Matsui Naoko"},{"name":"Fujita Koji"},{"name":"Miyashiro Ai"},{"name":"Nodera Hiroyuki"},{"name":"Izumi Yuishin"},{"name":"Shimizu Fumitaka"},{"name":"Miyamoto Katsuichi"},{"name":"Takahashi Yukitoshi"},{"name":"Kanda Takashi"},{"name":"Kusunoki Susumu"},{"name":"Kaji Ryuji"}],"ja":[{"name":"Furukawa Takahiro"},{"name":"松井 尚子"},{"name":"藤田 浩司"},{"name":"宮城 愛"},{"name":"野寺 裕之"},{"name":"和泉 唯信"},{"name":"Shimizu Fumitaka"},{"name":"Miyamoto Katsuichi"},{"name":"Takahashi Yukitoshi"},{"name":"Kanda Takashi"},{"name":"Kusunoki Susumu"},{"name":"梶 龍兒"}]},"description":{"en":"Proinflammatory cytokines may contribute to peripheral nerve demyelination in MMN.","ja":"Proinflammatory cytokines may contribute to peripheral nerve demyelination in MMN."},"publication_date":"2014-08-04","publication_name":{"en":"Journal of the Neurological Sciences","ja":"Journal of the Neurological Sciences"},"volume":"Vol.346","number":"No.1-2","starting_page":"75","ending_page":"79","languages":["eng"],"referee":true,"identifiers":{"doi":["10.1016/j.jns.2014.07.059"],"issn":["1878-5883"]},"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"https://www.ncbi.nlm.nih.gov/pubmed/25087556","label":"url"},{"@id":"https://www.scopus.com/record/display.url?eid=2-s2.0-84906089774&origin=inward","label":"url"},{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=303863","label":"url"}],"paper_title":{"en":"Case of neuromyelitis optica spectrum disorder associated with central pontine and extrapontine myelinolysis preceded by syndrome of inappropriate antidiuretic hormone secretion","ja":"抗利尿ホルモン分泌異常症候群で発症し,橋中心・橋外髄鞘崩壊症を合併した視神経脊髄炎関連疾患の1例"},"authors":{"en":[{"name":"酒井 和香"},{"name":"Matsui Naoko"},{"name":"Fujita Koji"},{"name":"Izumi Yuishin"},{"name":"西田 喜彦"},{"name":"高橋 利幸"},{"name":"神林 崇"},{"name":"Kaji Ryuji"}],"ja":[{"name":"酒井 和香"},{"name":"松井 尚子"},{"name":"藤田 浩司"},{"name":"和泉 唯信"},{"name":"西田 喜彦"},{"name":"高橋 利幸"},{"name":"神林 崇"},{"name":"梶 龍兒"}]},"description":{"en":"A 36-year-old woman complained of general malaise. She presented with hyponatremia and plasma osmotic pressure was lower than urinary osmotic pressure. In addition, serum antidiuretic hormone level was higher than the measurement sensitivity. She was diagnosed with the syndrome of inappropriate antidiuretic hormone secretion (SIADH). She fell into a coma despite correction of serum sodium level. Brain magnetic resonance imaging (MRI) revealed high signal intensities in the cerebral cortex, striatum, thalamus, hypothalamus, midbrain, and pons in fluid-attenuated inversion recovery images. Spinal MRI revealed a longitudinally extending lesion in the cervical cord. Serum sample was positive for anti-aquaporin-4 antibody, supporting the diagnosis of neuromyelitis optica spectrum disorder (NMOSD) combined with central pontine and extrapontine myelinolysis. In patients with NMOSD, the immune reaction can gradually cause destructive changes of the hypothalamus and lead to unstable ADH secretion in the absence of immunomodulatory treatment.","ja":"A 36-year-old woman complained of general malaise. She presented with hyponatremia and plasma osmotic pressure was lower than urinary osmotic pressure. In addition, serum antidiuretic hormone level was higher than the measurement sensitivity. She was diagnosed with the syndrome of inappropriate antidiuretic hormone secretion (SIADH). She fell into a coma despite correction of serum sodium level. Brain magnetic resonance imaging (MRI) revealed high signal intensities in the cerebral cortex, striatum, thalamus, hypothalamus, midbrain, and pons in fluid-attenuated inversion recovery images. Spinal MRI revealed a longitudinally extending lesion in the cervical cord. Serum sample was positive for anti-aquaporin-4 antibody, supporting the diagnosis of neuromyelitis optica spectrum disorder (NMOSD) combined with central pontine and extrapontine myelinolysis. In patients with NMOSD, the immune reaction can gradually cause destructive changes of the hypothalamus and lead to unstable ADH secretion in the absence of immunomodulatory treatment."},"publication_date":"2014-08-02","publication_name":{"en":"Clinical Neurology","ja":"臨床神経学"},"volume":"Vol.54","number":"No.7","starting_page":"556","ending_page":"560","languages":["jpn"],"referee":true,"identifiers":{"doi":["10.5692/clinicalneurol.54.556"],"issn":["1882-0654"]},"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"https://www.ncbi.nlm.nih.gov/pubmed/23926278","label":"url"},{"@id":"https://www.scopus.com/record/display.url?eid=2-s2.0-84898925618&origin=inward","label":"url"},{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=303979","label":"url"}],"paper_title":{"en":"Sera from patients with multifocal motor neuropathy disrupt the blood-nerve barrier","ja":"Sera from patients with multifocal motor neuropathy disrupt the blood-nerve barrier"},"authors":{"en":[{"name":"Shimizu Fumitaka"},{"name":"Omoto Masatoshi"},{"name":"Sano Yasuteru"},{"name":"Matsui Naoko"},{"name":"Miyashiro Ai"},{"name":"Tasaki Ayako"},{"name":"Maeda Toshihiko"},{"name":"Koga Michiaki"},{"name":"Kaji Ryuji"},{"name":"Kanda Takashi"}],"ja":[{"name":"Shimizu Fumitaka"},{"name":"Omoto Masatoshi"},{"name":"Sano Yasuteru"},{"name":"松井 尚子"},{"name":"宮城 愛"},{"name":"Tasaki Ayako"},{"name":"Maeda Toshihiko"},{"name":"Koga Michiaki"},{"name":"梶 龍兒"},{"name":"Kanda Takashi"}]},"description":{"en":"In multifocal motor neuropathy (MMN), the destruction of the blood-nerve barrier (BNB) has been considered to be the key step in the disease process. The purpose of the present study was to ascertain whether sera from patients with MMN can open the BNB, and which component of patient sera is the most important for this disruption. We evaluated the effects of sera from patients with MMN, patients with amyotrophic lateral sclerosis, and control subjects on the expression of tight junction proteins and vascular cell adhesion molecule-1 (VCAM-1), and on the transendothelial electrical resistance (TEER) in human peripheral nerve microvascular endothelial cells (PnMECs). The sera from patients with MMN decreased the claudin-5 protein expression and the TEER in PnMECs. However, this effect was reversed after application of an anti-vascular endothelial growth factor (anti-VEGF) neutralising antibody. The VEGF secreted by PnMECs was significantly increased after exposure to the sera from patients with MMN. The sera from patients with MMN also increased the VCAM-1 protein expression by upregulating the nuclear factor kappa-B (NF-κB) signalling. The immunoglobulin G purified from MMN sera decreased the expression of claudin-5 and increased the VCAM-1 expression in PnMECs. The sera from MMN patients may disrupt the BNB function via the autocrine secretion of VEGF in PnMECs, or the exposure to autoantibodies against PnMECs that are contained in the MMN sera. Autoantibodies against PnMECs in MMN sera may activate the BNB by upregulating the VCAM-1 expression, thereby allowing for the entry of a large number of circulating inflammatory cells into the peripheral nervous system.","ja":"In multifocal motor neuropathy (MMN), the destruction of the blood-nerve barrier (BNB) has been considered to be the key step in the disease process. The purpose of the present study was to ascertain whether sera from patients with MMN can open the BNB, and which component of patient sera is the most important for this disruption. We evaluated the effects of sera from patients with MMN, patients with amyotrophic lateral sclerosis, and control subjects on the expression of tight junction proteins and vascular cell adhesion molecule-1 (VCAM-1), and on the transendothelial electrical resistance (TEER) in human peripheral nerve microvascular endothelial cells (PnMECs). The sera from patients with MMN decreased the claudin-5 protein expression and the TEER in PnMECs. However, this effect was reversed after application of an anti-vascular endothelial growth factor (anti-VEGF) neutralising antibody. The VEGF secreted by PnMECs was significantly increased after exposure to the sera from patients with MMN. The sera from patients with MMN also increased the VCAM-1 protein expression by upregulating the nuclear factor kappa-B (NF-κB) signalling. The immunoglobulin G purified from MMN sera decreased the expression of claudin-5 and increased the VCAM-1 expression in PnMECs. The sera from MMN patients may disrupt the BNB function via the autocrine secretion of VEGF in PnMECs, or the exposure to autoantibodies against PnMECs that are contained in the MMN sera. Autoantibodies against PnMECs in MMN sera may activate the BNB by upregulating the VCAM-1 expression, thereby allowing for the entry of a large number of circulating inflammatory cells into the peripheral nervous system."},"publication_date":"2014-05","publication_name":{"en":"Journal of Neurology, Neurosurgery, and Psychiatry","ja":"Journal of Neurology, Neurosurgery, and Psychiatry"},"volume":"Vol.85","number":"No.5","starting_page":"526","ending_page":"537","languages":["eng"],"referee":true,"identifiers":{"doi":["10.1136/jnnp-2013-305405"],"issn":["1468-330X"]},"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"https://repo.lib.tokushima-u.ac.jp/ja/105906","label":"url"},{"@id":"https://www.ncbi.nlm.nih.gov/pubmed/24741683","label":"url"},{"@id":"https://www.scopus.com/record/display.url?eid=2-s2.0-84893935930&origin=inward","label":"url"},{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=288179","label":"url"}],"paper_title":{"en":"Are multifocal motor neuropathy patients underdiagnosed? An epidemiological survey in Japan","ja":"Are multifocal motor neuropathy patients underdiagnosed? An epidemiological survey in Japan"},"authors":{"en":[{"name":"Miyashiro Ai"},{"name":"Matsui Naoko"},{"name":"Shimatani Yoshimitsu"},{"name":"Nodera Hiroyuki"},{"name":"Izumi Yuishin"},{"name":"Kawabata Satoshi"},{"name":"Imai Tomihiro"},{"name":"Baba Masayuki"},{"name":"Komori Tetsuo"},{"name":"Sonoo Masahiro"},{"name":"Maezaki Takahiro"},{"name":"Kawamata Jun"},{"name":"Hitomi Takefumi"},{"name":"Kohara Nobuo"},{"name":"Arimura Kimiyoshi"},{"name":"Hashimoto Shuji"},{"name":"Arisawa Kokichi"},{"name":"Kusunoki Susumu"},{"name":"Kaji Ryuji"}],"ja":[{"name":"宮城 愛"},{"name":"松井 尚子"},{"name":"島谷 佳光"},{"name":"野寺 裕之"},{"name":"和泉 唯信"},{"name":"Kawabata Satoshi"},{"name":"Imai Tomihiro"},{"name":"Baba Masayuki"},{"name":"Komori Tetsuo"},{"name":"Sonoo Masahiro"},{"name":"Maezaki Takahiro"},{"name":"Kawamata Jun"},{"name":"Hitomi Takefumi"},{"name":"Kohara Nobuo"},{"name":"Arimura Kimiyoshi"},{"name":"Hashimoto Shuji"},{"name":"有澤 孝吉"},{"name":"Kusunoki Susumu"},{"name":"梶 龍兒"}]},"description":{"en":"Our objective was to do an epidemiologic survey of patients with multifocal motor neuropathy (MMN) in comparison with those with amyotrophic lateral sclerosis (ALS) in Japan. In this retrospective study, we examined 46 patients with MMN and 1,051 patients with ALS from major neuromuscular centers in Japan from 2005 to 2009. Diagnosis was based on the European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) and the revised El Escorial criteria. The efficacy of intravenous immunoglobulin (IVIg) was also taken into consideration in the diagnosis of MMN. The ratio of MMN to ALS patients (0-0.10) varied among the centers, but mostly converged to 0.05. The prevalence was estimated to be 0.29 MMN patients and 6.63 ALS patients per 100,000 population. The frequency of MMN patients was around 1 out of 20 ALS patients, and MMN was possibly underdiagnosed in some centers.","ja":"Our objective was to do an epidemiologic survey of patients with multifocal motor neuropathy (MMN) in comparison with those with amyotrophic lateral sclerosis (ALS) in Japan. In this retrospective study, we examined 46 patients with MMN and 1,051 patients with ALS from major neuromuscular centers in Japan from 2005 to 2009. Diagnosis was based on the European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) and the revised El Escorial criteria. The efficacy of intravenous immunoglobulin (IVIg) was also taken into consideration in the diagnosis of MMN. The ratio of MMN to ALS patients (0-0.10) varied among the centers, but mostly converged to 0.05. The prevalence was estimated to be 0.29 MMN patients and 6.63 ALS patients per 100,000 population. The frequency of MMN patients was around 1 out of 20 ALS patients, and MMN was possibly underdiagnosed in some centers."},"publication_date":"2014-03","publication_name":{"en":"Muscle & Nerve","ja":"Muscle & Nerve"},"volume":"Vol.49","number":"No.3","starting_page":"357","ending_page":"361","languages":["eng"],"referee":true,"identifiers":{"doi":["10.1002/mus.23930"],"issn":["0148-639X"]},"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"https://www.ncbi.nlm.nih.gov/pubmed/24492738","label":"url"},{"@id":"https://www.scopus.com/record/display.url?eid=2-s2.0-84897898031&origin=inward","label":"url"},{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=303998","label":"url"}],"paper_title":{"en":"Longitudinal Monitoring with Multiple MR Techniques in a Case of Progressive Multifocal Leukoencephalopathy Associated with Multiple Myeloma","ja":"Longitudinal Monitoring with Multiple MR Techniques in a Case of Progressive Multifocal Leukoencephalopathy Associated with Multiple Myeloma"},"authors":{"en":[{"name":"Majigusuren Mungunkhuyag"},{"name":"Harada Masafumi"},{"name":"Abe Takashi"},{"name":"Fujita Koji"},{"name":"Matsui Naoko"},{"name":"Kaji Ryuji"}],"ja":[{"name":"Majigusuren Mungunkhuyag"},{"name":"原田 雅史"},{"name":"阿部 考志"},{"name":"藤田 浩司"},{"name":"松井 尚子"},{"name":"梶 龍兒"}]},"description":{"en":"Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease of the brain caused by the JC virus in immunocompromised patients. We report characteristic features of proton MR spectroscopy, 3-dimensional pseudo-continuous arterial spin labeling imaging, and diffusion tensor imaging in a 53-year-old patient with PML. The utility of multi-modal magnetic resonance techniques for longitudinal monitoring was indicated by their reevaluation over time and consideration of their relation to prognosis.","ja":"Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease of the brain caused by the JC virus in immunocompromised patients. We report characteristic features of proton MR spectroscopy, 3-dimensional pseudo-continuous arterial spin labeling imaging, and diffusion tensor imaging in a 53-year-old patient with PML. The utility of multi-modal magnetic resonance techniques for longitudinal monitoring was indicated by their reevaluation over time and consideration of their relation to prognosis."},"publication_date":"2014-02-05","publication_name":{"en":"Magnetic Resonance in Medical Sciences","ja":"Magnetic Resonance in Medical Sciences"},"volume":"Vol.13","number":"No.1","starting_page":"55","ending_page":"59","languages":["eng"],"referee":true,"identifiers":{"doi":["10.2463/mrms.2013-0037"],"issn":["1880-2206"]},"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"https://www.ncbi.nlm.nih.gov/pubmed/24556356","label":"url"},{"@id":"https://www.scopus.com/record/display.url?eid=2-s2.0-84896393117&origin=inward","label":"url"},{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=285940","label":"url"}],"paper_title":{"en":"Increased number of Hassall's corpuscles in myasthenia gravis patients with thymic hyperplasia.","ja":"Increased number of Hassall's corpuscles in myasthenia gravis patients with thymic hyperplasia."},"authors":{"en":[{"name":"Matsui Naoko"},{"name":"Ohigashi Izumi"},{"name":"Tanaka Keijirou"},{"name":"Sakata Mie"},{"name":"Furukawa Takahiro"},{"name":"Nakagawa Yasushi"},{"name":"Kondo Kazuya"},{"name":"Kitagawa Tetsuya"},{"name":"Yamashita Sumimasa"},{"name":"Nomura Yoshiko"},{"name":"Takahama Yousuke"},{"name":"Kaji Ryuji"}],"ja":[{"name":"松井 尚子"},{"name":"大東 いずみ"},{"name":"Tanaka Keijirou"},{"name":"Sakata Mie"},{"name":"Furukawa Takahiro"},{"name":"中川 靖士"},{"name":"近藤 和也"},{"name":"北川 哲也"},{"name":"Yamashita Sumimasa"},{"name":"Nomura Yoshiko"},{"name":"高浜 洋介"},{"name":"梶 龍兒"}]},"description":{"en":"The thymus is implicated as an organ that contributes to autoimmunity in myasthenia gravis (MG) patients. Hassall's corpuscles (HCs) are assumed to represent the terminally differentiated stage of medullary thymic epithelial cells (mTECs). By using multicolor immunohistofluorescence analysis, we examined HCs in thymuses that were therapeutically excised from MG (+) and MG (-) patients. We found that the number of HCs per unit area of the thymic medulla was significantly elevated in the thymuses of MG (+) patients with thymic hyperplasia. CCL21 expression increased in the hyperplastic MG thymuses. We speculate that the altered differentiation of mTECs is associated with the thymic hyperplasia and the onset of MG.","ja":"The thymus is implicated as an organ that contributes to autoimmunity in myasthenia gravis (MG) patients. Hassall's corpuscles (HCs) are assumed to represent the terminally differentiated stage of medullary thymic epithelial cells (mTECs). By using multicolor immunohistofluorescence analysis, we examined HCs in thymuses that were therapeutically excised from MG (+) and MG (-) patients. We found that the number of HCs per unit area of the thymic medulla was significantly elevated in the thymuses of MG (+) patients with thymic hyperplasia. CCL21 expression increased in the hyperplastic MG thymuses. We speculate that the altered differentiation of mTECs is associated with the thymic hyperplasia and the onset of MG."},"publication_date":"2014-01-28","publication_name":{"en":"Journal of Neuroimmunology","ja":"Journal of Neuroimmunology"},"volume":"Vol.269","number":"No.1-2","starting_page":"56","ending_page":"61","languages":["eng"],"referee":true,"identifiers":{"doi":["10.1016/j.jneuroim.2014.01.011"],"issn":["1872-8421"]},"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"https://www.ncbi.nlm.nih.gov/pubmed/24482655","label":"url"},{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=304028","label":"url"}],"paper_title":{"en":"FK506 attenuates thymic output in patients with myasthenia gravis","ja":"FK506 attenuates thymic output in patients with myasthenia gravis"},"authors":{"en":[{"name":"Mitsui Takao"},{"name":"Kuroda Yukiko"},{"name":"Ueono Shu-ichi"},{"name":"Matsui Naoko"},{"name":"Kaji Ryuji"}],"ja":[{"name":"三ツ井 貴夫"},{"name":"Kuroda Yukiko"},{"name":"Ueono Shu-ichi"},{"name":"松井 尚子"},{"name":"梶 龍兒"}]},"description":{"en":"Myasthenia gravis (MG) is an antibody-mediated, T-cell-dependent autoimmune disease. The symptoms are caused by high-affinity IgG against the muscle acetylcholine receptor (AChR) at the neuromuscular junction. The production of these antibodies in B-cells depends on AChR-specific CD4(+) T-cells and the thymus gland seems to play a significant role in the pathogenesis of MG. Altered thymic T-cell export seems to be associated with a pathological mechanism in myasthenia gravis. Tacrolimus (FK506) has recently been used to treat MG. We examined the effects of tacrolimus on thymic T-cell export in patients with MG. Sixteen patients with nonthymomatous and/or thymectomized MG were treated with oral administrations of tacrolimus. To assess the effect of tacrolimus on the thymic output, we assayed the levels of T-cell receptor excision circle (TREC), a molecular marker of thymus emigrants. T-cell receptor excision circle was not significantly different from those in age-matched controls before tacrolimus therapy, but they were partially decreased 4 months after tacrolimus therapy. T-cell receptor excision circle levels were significantly decreased in the thymomatous group (p < 0.05), but not in the nonthymomatous group. Tacrolimus treatment significantly attenuated TREC levels in cultured CD4(-)CD8(+) cells (p < 0.05), but total cell counts were not significantly changed. These results indicate that TREC levels may become a marker of the curative effect of tacrolimus therapy for thymomatous MG, and that tacrolimus suppresses not only activating T-lymphocytes, but also naïve T-cells.","ja":"Myasthenia gravis (MG) is an antibody-mediated, T-cell-dependent autoimmune disease. The symptoms are caused by high-affinity IgG against the muscle acetylcholine receptor (AChR) at the neuromuscular junction. The production of these antibodies in B-cells depends on AChR-specific CD4(+) T-cells and the thymus gland seems to play a significant role in the pathogenesis of MG. Altered thymic T-cell export seems to be associated with a pathological mechanism in myasthenia gravis. Tacrolimus (FK506) has recently been used to treat MG. We examined the effects of tacrolimus on thymic T-cell export in patients with MG. Sixteen patients with nonthymomatous and/or thymectomized MG were treated with oral administrations of tacrolimus. To assess the effect of tacrolimus on the thymic output, we assayed the levels of T-cell receptor excision circle (TREC), a molecular marker of thymus emigrants. T-cell receptor excision circle was not significantly different from those in age-matched controls before tacrolimus therapy, but they were partially decreased 4 months after tacrolimus therapy. T-cell receptor excision circle levels were significantly decreased in the thymomatous group (p < 0.05), but not in the nonthymomatous group. Tacrolimus treatment significantly attenuated TREC levels in cultured CD4(-)CD8(+) cells (p < 0.05), but total cell counts were not significantly changed. These results indicate that TREC levels may become a marker of the curative effect of tacrolimus therapy for thymomatous MG, and that tacrolimus suppresses not only activating T-lymphocytes, but also naïve T-cells."},"publication_date":"2013-12-26","publication_name":{"en":"Archives of Medical Science : AMS","ja":"Archives of Medical Science : AMS"},"volume":"Vol.9","number":"No.6","starting_page":"1090","ending_page":"1096","languages":["eng"],"referee":true,"identifiers":{"doi":["10.5114/aoms.2013.39797"],"issn":["1734-1922"]},"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"https://www.ncbi.nlm.nih.gov/pubmed/24219883","label":"url"},{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=303978","label":"url"}],"paper_title":{"en":"Increased interleukin-17 in the cerebrospinal fluid in sporadic Creutzfeldt-Jakob disease: a case-control study of rapidly progressive dementia","ja":"Increased interleukin-17 in the cerebrospinal fluid in sporadic Creutzfeldt-Jakob disease: a case-control study of rapidly progressive dementia"},"authors":{"en":[{"name":"Fujita Koji"},{"name":"Matsui Naoko"},{"name":"Takahashi Yukitoshi"},{"name":"Iwasaki Yasushi"},{"name":"Yoshida Mari"},{"name":"Yuasa Tatsuhiko"},{"name":"Izumi Yuishin"},{"name":"Kaji Ryuji"}],"ja":[{"name":"藤田 浩司"},{"name":"松井 尚子"},{"name":"Takahashi Yukitoshi"},{"name":"Iwasaki Yasushi"},{"name":"Yoshida Mari"},{"name":"Yuasa Tatsuhiko"},{"name":"和泉 唯信"},{"name":"梶 龍兒"}]},"description":{"en":"Inflammatory responses in the cerebrospinal fluid (CSF) of patients with sporadic Creutzfeldt-Jakob disease (sCJD) remain elusive. We conducted a case-control study, in which 14 patients with sCJD, 14 with noninflammatory neurological disorders, and 14 with autoimmune encephalitis were enrolled. We used the suspension array system to measure the concentrations of 27 cytokines in CSF. The cytokine titers of the three groups were compared, and the correlation between the relevant cytokine titers and clinical parameters was investigated in the patients with sCJD. Levels of the two cytokines interleukin (IL)-1 receptor antagonist and IL-17 were significantly elevated in the patients with sCJD compared with those in the patients with noninflammatory neurological disorders: IL-17 levels in sCJD were approximately ten times higher than in the noninflammatory neurological disorders (mean, 35.46 vs. 3.45 pg/ml; P < 0.001) but comparable to that in encephalitis (mean, 32.16 pg/ml). In contrast, levels of classical proinflammatory cytokines such as IL-12(p70) and tumor necrosis factor-α were increased only in encephalitis. Although not significant, IL-17 titers tended to be higher in the patients with shorter disease duration before CSF sampling (r = -0.452; P = 0.104) and in those with lower CSF total protein concentrations (r = -0.473; P = 0.086). IL-17 is significantly increased in CSF in sCJD, which can be an early event in the pathogenesis of sCJD.","ja":"Inflammatory responses in the cerebrospinal fluid (CSF) of patients with sporadic Creutzfeldt-Jakob disease (sCJD) remain elusive. We conducted a case-control study, in which 14 patients with sCJD, 14 with noninflammatory neurological disorders, and 14 with autoimmune encephalitis were enrolled. We used the suspension array system to measure the concentrations of 27 cytokines in CSF. The cytokine titers of the three groups were compared, and the correlation between the relevant cytokine titers and clinical parameters was investigated in the patients with sCJD. Levels of the two cytokines interleukin (IL)-1 receptor antagonist and IL-17 were significantly elevated in the patients with sCJD compared with those in the patients with noninflammatory neurological disorders: IL-17 levels in sCJD were approximately ten times higher than in the noninflammatory neurological disorders (mean, 35.46 vs. 3.45 pg/ml; P < 0.001) but comparable to that in encephalitis (mean, 32.16 pg/ml). In contrast, levels of classical proinflammatory cytokines such as IL-12(p70) and tumor necrosis factor-α were increased only in encephalitis. Although not significant, IL-17 titers tended to be higher in the patients with shorter disease duration before CSF sampling (r = -0.452; P = 0.104) and in those with lower CSF total protein concentrations (r = -0.473; P = 0.086). IL-17 is significantly increased in CSF in sCJD, which can be an early event in the pathogenesis of sCJD."},"publication_date":"2013-11-13","publication_name":{"en":"Journal of Neuroinflammation","ja":"Journal of Neuroinflammation"},"volume":"Vol.10","starting_page":"135","ending_page":"135","languages":["eng"],"referee":true,"identifiers":{"doi":["10.1186/1742-2094-10-135"],"issn":["1742-2094"]},"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"https://www.ncbi.nlm.nih.gov/pubmed/23715915","label":"url"},{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=303977","label":"url"}],"paper_title":{"en":"Successful treatment of stiff person syndrome with sequential use of tacrolimus","ja":"Successful treatment of stiff person syndrome with sequential use of tacrolimus"},"authors":{"en":[{"name":"Nakane Syunya"},{"name":"Fujita Koji"},{"name":"Shibuta Yoshiko"},{"name":"Matsui Naoko"},{"name":"Harada Masafumi"},{"name":"Urushihara Ryo"},{"name":"Nishida Yoshihiko"},{"name":"Izumi Yuishin"},{"name":"Kaji Ryuji"}],"ja":[{"name":"Nakane Syunya"},{"name":"藤田 浩司"},{"name":"Shibuta Yoshiko"},{"name":"松井 尚子"},{"name":"原田 雅史"},{"name":"Urushihara Ryo"},{"name":"Nishida Yoshihiko"},{"name":"和泉 唯信"},{"name":"梶 龍兒"}]},"publication_date":"2013-05-28","publication_name":{"en":"Journal of Neurology, Neurosurgery, and Psychiatry","ja":"Journal of Neurology, Neurosurgery, and Psychiatry"},"volume":"Vol.84","number":"No.10","starting_page":"1177","ending_page":"1180","languages":["eng"],"referee":true,"identifiers":{"doi":["10.1136/jnnp-2013-305425"],"issn":["1468-330X"]},"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=304027","label":"url"}],"paper_title":{"en":"Diagnostic spectrum of multifocal motor neuropathy","ja":"Diagnostic spectrum of multifocal motor neuropathy"},"authors":{"en":[{"name":"Matsui Naoko"},{"name":"Miyashiro Ai"},{"name":"Shimatani Yoshimitsu"},{"name":"Nodera Hiroyuki"},{"name":"Izumi Yuishin"},{"name":"Kuwabara Satoshi"},{"name":"Baba Masayuki"},{"name":"Komori Tetsuo"},{"name":"Sonoo Masahiro"},{"name":"Mezaki Takahiro"},{"name":"Kawamata Jun"},{"name":"Hitomi Takefumi"},{"name":"Imai Tomihiro"},{"name":"Kohara Nobuo"},{"name":"Arimura Kimiyoshi"},{"name":"Arisawa Kokichi"},{"name":"Kusunoki Susumu"},{"name":"Kaji Ryuji"}],"ja":[{"name":"松井 尚子"},{"name":"宮城 愛"},{"name":"島谷 佳光"},{"name":"野寺 裕之"},{"name":"和泉 唯信"},{"name":"Kuwabara Satoshi"},{"name":"Baba Masayuki"},{"name":"Komori Tetsuo"},{"name":"Sonoo Masahiro"},{"name":"Mezaki Takahiro"},{"name":"Kawamata Jun"},{"name":"Hitomi Takefumi"},{"name":"Imai Tomihiro"},{"name":"Kohara Nobuo"},{"name":"Arimura Kimiyoshi"},{"name":"有澤 孝吉"},{"name":"Kusunoki Susumu"},{"name":"梶 龍兒"}]},"publication_date":"2013","publication_name":{"en":"Clinical & Experimental Neuroimmunology","ja":"Clinical & Experimental Neuroimmunology"},"volume":"Vol.4","number":"No.2","starting_page":"210","ending_page":"215","languages":["eng"],"referee":true,"identifiers":{"doi":["10.1111/cen3.12025"],"issn":["1759-1961"]},"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"http://search.jamas.or.jp/link/ui/2012112946","label":"url"},{"@id":"https://cir.nii.ac.jp/crid/1390282679397005184/","label":"url"},{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=307155","label":"url"}],"paper_title":{"en":"A case of enlargement of the right atrium associated with myositis detected by muscle ultrasonography","ja":"筋エコーにより筋炎の関与が判明した右房拡張症の一例"},"authors":{"en":[{"name":"Miyazaki Yoshimichi"},{"name":"高松 直子"},{"name":"Miyashiro Ai"},{"name":"Terasawa Yuka"},{"name":"Matsui Naoko"},{"name":"Asanuma Koutaro"},{"name":"Izumi Yuishin"},{"name":"Kaji Ryuji"}],"ja":[{"name":"宮﨑 由道"},{"name":"高松 直子"},{"name":"宮城 愛"},{"name":"寺澤 由佳"},{"name":"松井 尚子"},{"name":"浅沼 光太郎"},{"name":"和泉 唯信"},{"name":"梶 龍兒"}]},"description":{"en":"A 45-year-old woman was admitted to the hospital because of general malaise since 2005. She have not remarkable disease in the past history. In the family history, one of her older sister died of ulcerative colitis, another one suffers from systemic lupus erythematosis, and her older brother affected of Behçet's disease. On electrocardiogram examination, atrial fibrillation was revealed associated with remarkable dilation of the right atrium in echocardiogram, but any abnormal finding is not seen both ventricles. The ultrasonographic findings of the biceps brachi showed remarkable myopathic pattern. A biopsy specimen obtained from the biceps brachi, these muscles revealed degeneration and necrosis of muscle fibers, interstitial infiltration with monocytes, and perivascular infiltrates of inflammatory cells. In the magnetic resonance imaging of the heart showed, not only remarkable dilatation of right atria, but also diffuse high intensity areas in left ventricle under T2-intensified conditions. We diagnosed generalized myositis involved with myocarditis, and treated with steroid. One year later, she still takes steroid, and her symptoms improvement continuously.If one of cardiac atrium or ventricle enlarged, there is possibility of the cardiac myositis or systemic myositis. In these cases, skeletal muscle ultrasonography may be non-invasive and useful method of differential diagnosis.","ja":"症例は45歳女性で,全身倦怠感を主訴にした.42歳時に動悸症状を自覚し,44歳時に全身倦怠感を強く自覚し,近医の心電図で心房細動を指摘された.聴診では心雑音や過剰心音はなく,肺音は清,頸動脈雑音を認めなかった.頸部,四肢近位部にごく軽度の筋力低下を認め,Gowers徴候は明らかでなかった.24時間心電図検査で頻発する発作性心房細動を認め,モニター心電図で最大約7秒の洞停止を確認した.心エコーでは右房の著明な拡張,左房の軽度拡張を認めた.三尖弁に軽度逆流を認めたが,逆流の程度からは右房拡張の直接原因とは考え難かった.僧帽弁に異常は認めず,左室収縮能は保たれていた.膠原病関連疾患としての筋炎,サルコイドーシスなどの鑑別目的に筋エコー検査を施行した.特発性右房拡張症中に,全身性筋炎を伴う心筋炎を原因とした一群の存在の可能性があり,スクリーニング検査として,筋エコー検査は侵襲性が少なく,簡便で,またペースメーカー導入後も使用でき有用であった."},"publication_date":"2011-04","publication_name":{"en":"Neurosonology","ja":"神経超音波医学"},"volume":"Vol.24","number":"No.1","starting_page":"12","ending_page":"14","languages":["jpn"],"referee":true,"identifiers":{"doi":["10.2301/neurosonology.24.12"],"issn":["0917-074X"]},"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"http://ci.nii.ac.jp/naid/40018806453/","label":"url"},{"@id":"https://cir.nii.ac.jp/crid/1521417755902852864/","label":"url"},{"@id":"https://www.scopus.com/record/display.url?eid=2-s2.0-79955804438&origin=inward","label":"url"},{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=250568","label":"url"}],"paper_title":{"en":"Clinical features of optic neuritis positive for anti-aquaporin 4 antibody","ja":"抗アクアポリン4抗体陽性視神経炎の臨床像 (特集 第64回日本臨床眼科学会講演集(2))"},"authors":{"en":[{"name":"Chihiro Yamanaka"},{"name":"Egawa Mariko"},{"name":"Mitamura Yoshinori"},{"name":"Matsui Naoko"},{"name":"Kaji Ryuji"}],"ja":[{"name":"山中 千尋"},{"name":"江川 麻理子"},{"name":"三田村 佳典"},{"name":"松井 尚子"},{"name":"梶 龍兒"}]},"publication_date":"2011-04","publication_name":{"en":"Japanese Journal of Clinical Ophthalmology","ja":"臨床眼科"},"volume":"Vol.65","number":"No.4","starting_page":"521","ending_page":"526","languages":["jpn"],"referee":true,"identifiers":{"issn":["0370-5579"]},"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"https://www.ncbi.nlm.nih.gov/pubmed/20211905","label":"url"},{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=257217","label":"url"}],"paper_title":{"en":"Undiminished regulatory T cells in the thymus of myathenia gravis patients","ja":"Undiminished regulatory T cells in the thymus of myathenia gravis patients"},"authors":{"en":[{"name":"Matsui Naoko"},{"name":"Nakane Shunya"},{"name":"Saitou Fumi"},{"name":"Ohigashi Izumi"},{"name":"Nakagawa Yasushi"},{"name":"Kurobe Hirotsugu"},{"name":"Takizawa Hiromitsu"},{"name":"Mitsui Takao"},{"name":"Kondo Kazuya"},{"name":"Kitagawa Tetsuya"},{"name":"Takahama Yousuke"},{"name":"Kaji Ryuji"}],"ja":[{"name":"松井 尚子"},{"name":"中根 俊成"},{"name":"Saitou Fumi"},{"name":"大東 いずみ"},{"name":"中川 靖士"},{"name":"黒部 裕嗣"},{"name":"滝沢 宏光"},{"name":"三ツ井 貴夫"},{"name":"近藤 和也"},{"name":"北川 哲也"},{"name":"高浜 洋介"},{"name":"梶 龍兒"}]},"description":{"en":"The thymus has been implicated as a possible site of origin that triggers autoimmunity in myasthenia gravis (MG). Although several groups have suggested that the decrease in the number of regulatory T (Treg) cells contributes to the onset of MG, the exact role of Treg cells in MG remains unclear. To address this point, we examined the number and distribution of Treg cells in a large number of patients with MG. Immunohistofluorescence analysis of Foxp3 along with CD4 and CD8 was performed in thymic sections of MG (+) (n = 24) and MG (-) patients (n = 27). Circulating CD4(+)CD25(+) cells in the peripheral blood of patients with MG (n = 15) and age-matched healthy subjects (n = 15) were also analyzed. Foxp3(+)CD4(+)CD8(-) cells were predominantly found in the thymic medulla and their number declined with age. There was no significant difference in the number or the distribution of Foxp3(+)CD4(+)CD8(-) cells in the thymus between MG (+) and MG (-) patients. The number of circulating CD4(+)CD25(+) cells in the peripheral blood of patients with MG was not significantly altered compared to that in healthy subjects. The cellularity of Treg cells in the thymus and circulation is not diminished in patients with myasthenia gravis.","ja":"The thymus has been implicated as a possible site of origin that triggers autoimmunity in myasthenia gravis (MG). Although several groups have suggested that the decrease in the number of regulatory T (Treg) cells contributes to the onset of MG, the exact role of Treg cells in MG remains unclear. To address this point, we examined the number and distribution of Treg cells in a large number of patients with MG. Immunohistofluorescence analysis of Foxp3 along with CD4 and CD8 was performed in thymic sections of MG (+) (n = 24) and MG (-) patients (n = 27). Circulating CD4(+)CD25(+) cells in the peripheral blood of patients with MG (n = 15) and age-matched healthy subjects (n = 15) were also analyzed. Foxp3(+)CD4(+)CD8(-) cells were predominantly found in the thymic medulla and their number declined with age. There was no significant difference in the number or the distribution of Foxp3(+)CD4(+)CD8(-) cells in the thymus between MG (+) and MG (-) patients. The number of circulating CD4(+)CD25(+) cells in the peripheral blood of patients with MG was not significantly altered compared to that in healthy subjects. The cellularity of Treg cells in the thymus and circulation is not diminished in patients with myasthenia gravis."},"publication_date":"2010-03-09","publication_name":{"en":"Neurology","ja":"Neurology"},"volume":"Vol.74","number":"No.10","starting_page":"816","ending_page":"820","languages":["eng"],"referee":true,"identifiers":{"doi":["10.1212/WNL.0b013e3181d31e47"],"issn":["1526-632X"]},"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"http://ci.nii.ac.jp/naid/40019066124/","label":"url"},{"@id":"https://cir.nii.ac.jp/crid/1523106605998613120/","label":"url"},{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=257216","label":"url"}],"paper_title":{"en":"Neurological manifestations in hereditary hemorrhagic telangiectasia type 1: a familial case in Japan","ja":"Neurological manifestations in hereditary hemorrhagic telangiectasia type 1: a familial case in Japan"},"authors":{"en":[{"name":"Matsui Naoko"},{"name":"Izumi Yuishin"},{"name":"Azuma Hiroyuki"},{"name":"Kaji Ryuji"}],"ja":[{"name":"松井 尚子"},{"name":"和泉 唯信"},{"name":"東 博之"},{"name":"梶 龍兒"}]},"publication_date":"2010-01","publication_name":{"en":"Journal of Tokushima National Hospital","ja":"Journal of Tokushima National Hospital"},"volume":"Vol.2010","number":"No.1","starting_page":"11","ending_page":"14","languages":["eng"],"referee":true,"identifiers":{"issn":["2185-3169"]},"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"https://cir.nii.ac.jp/crid/1523951030929400320/","label":"url"},{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=298033","label":"url"}],"paper_title":{"en":"A case of cryptococcal meningitis successfully treated with liposomal amphotericin-B, Fulcytosine, and Voriconazole","ja":"A case of cryptococcal meningitis successfully treated with liposomal amphotericin-B, Fulcytosine, and Voriconazole"},"authors":{"en":[{"name":"Miyazaki Yoshimichi"},{"name":"Miyashiro Ai"},{"name":"Simatani Yoshimitsu"},{"name":"Matsui Naoko"},{"name":"Asanuma Koutaro"},{"name":"Izumi Yuishin"},{"name":"Kaji Ryuji"}],"ja":[{"name":"宮﨑 由道"},{"name":"宮城 愛"},{"name":"Simatani Yoshimitsu"},{"name":"松井 尚子"},{"name":"浅沼 光太郎"},{"name":"和泉 唯信"},{"name":"梶 龍兒"}]},"description":{"en":"クリプトコッカス性髄膜炎の59歳男性症例について検討した.発熱と頭痛を主訴とし,風邪と診断さたが,嘔吐,意識障害,歩行困難の症状が出現し,発症から16日目に入院となった.入院時に意識障害と強い傾眠傾向が認められた.右眼の対光反射が鈍く,瞳孔径の左右差が認められた.顕著な頸部硬直も認められた.ウイルス関連抗原抗体は陰性であった.脳脊髄液はキサントクロミーを呈しており,脳脊髄液圧,脳脊髄液中の細胞数と蛋白濃度の上昇,脳脊髄液中の糖濃度低下が認められた.May-Giemsa染色および墨汁染色では莢膜を有する菌体が多数確認された.Cryptococcus抗原の256倍上昇が示された.頭部のガドリニウム造影MRI検査で脳溝に沿った造影像を認めた.クリプトコッカス性髄膜炎と診断し,liposomal amphotericin-Bおよびfulcytosineによる2剤療法を開始した.臨床検査結果および脳脊髄液検査結果は徐々に改善したが,入院45日目に左顔面麻痺が出現した.MRI検査から新しい病変が確認され,造影MRI検査で造影病変の拡大を認めた.Voriconazole追加による3剤療法を開始した.脳脊髄液検査結果および臨床症状の改善を示し,入院180日目に退院となった.左顔面麻痺も徐々に改善した.","ja":"クリプトコッカス性髄膜炎の59歳男性症例について検討した.発熱と頭痛を主訴とし,風邪と診断さたが,嘔吐,意識障害,歩行困難の症状が出現し,発症から16日目に入院となった.入院時に意識障害と強い傾眠傾向が認められた.右眼の対光反射が鈍く,瞳孔径の左右差が認められた.顕著な頸部硬直も認められた.ウイルス関連抗原抗体は陰性であった.脳脊髄液はキサントクロミーを呈しており,脳脊髄液圧,脳脊髄液中の細胞数と蛋白濃度の上昇,脳脊髄液中の糖濃度低下が認められた.May-Giemsa染色および墨汁染色では莢膜を有する菌体が多数確認された.Cryptococcus抗原の256倍上昇が示された.頭部のガドリニウム造影MRI検査で脳溝に沿った造影像を認めた.クリプトコッカス性髄膜炎と診断し,liposomal amphotericin-Bおよびfulcytosineによる2剤療法を開始した.臨床検査結果および脳脊髄液検査結果は徐々に改善したが,入院45日目に左顔面麻痺が出現した.MRI検査から新しい病変が確認され,造影MRI検査で造影病変の拡大を認めた.Voriconazole追加による3剤療法を開始した.脳脊髄液検査結果および臨床症状の改善を示し,入院180日目に退院となった.左顔面麻痺も徐々に改善した."},"publication_date":"2010","publication_name":{"en":"Journal of Tokushima National Hospital","ja":"Journal of Tokushima National Hospital"},"volume":"Vol.1","starting_page":"31","ending_page":"34","languages":["eng"],"referee":true,"identifiers":{"issn":["2185-3169"]},"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=271788","label":"url"}],"paper_title":{"en":"健常者に発症し治療に難渋したクリプトコッカス髄膜脳炎の1例","ja":"健常者に発症し治療に難渋したクリプトコッカス髄膜脳炎の1例"},"authors":{"en":[{"name":"Matsui Naoko"},{"name":"Nakane Shunya"},{"name":"Izumi Yuishin"},{"name":"Nagahiro Shinji"},{"name":"西田 善彦"},{"name":"Kaji Ryuji"}],"ja":[{"name":"松井 尚子"},{"name":"中根 俊成"},{"name":"和泉 唯信"},{"name":"永廣 信治"},{"name":"西田 善彦"},{"name":"梶 龍兒"}]},"publication_date":"2007","publication_name":{"en":"Neurological Therapeutics","ja":"神経治療学"},"volume":"Vol.24","starting_page":"497","ending_page":"502","languages":["jpn"],"referee":true,"identifiers":{"issn":["0916-8443"]},"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"https://www.ncbi.nlm.nih.gov/pubmed/14714966","label":"url"},{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=374570","label":"url"}],"paper_title":{"en":"Dermatomyositis with peripheral nervous system involvement: activation of vascular endothelial growth factor (VEGF) and VEGF receptor (VEGFR) in vasculitic lesions","ja":"Dermatomyositis with peripheral nervous system involvement: activation of vascular endothelial growth factor (VEGF) and VEGF receptor (VEGFR) in vasculitic lesions"},"authors":{"en":[{"name":"Matsui Naoko"},{"name":"Mitsui Takao"},{"name":"Endo Itsuro"},{"name":"Oshima Yasushi"},{"name":"Kunishige Makoto"},{"name":"Matsumoto Toshio"}],"ja":[{"name":"松井 尚子"},{"name":"三ツ井 貴夫"},{"name":"遠藤 逸朗"},{"name":"大島 康志"},{"name":"Kunishige Makoto"},{"name":"松本 俊夫"}]},"description":{"en":"We describe two cases of dermatomyositis (DM) with nervous system involvement. Polyneuropathy was observed in both patients, and cerebral vasculitis was suspected in one patient. Histological examination of biopsied specimens of skeletal muscles, skin and sural nerves revealed vasculitis. Furthermore, vascular endothelial growth factor (VEGF) and VEGF receptors (VEGFRs) were overexpressed in vasculitic lesions. Although, VEGF and VEGFRs were not detected in biopsied specimens of skeletal muscle from normal subjects, they were observed in one of two patients with DM who did not exhibit neuropathy. These findings suggest the possibility that VEGF overproduction is associated with development of vasculitis in some cases of DM complicated with peripheral neuropathy.","ja":"We describe two cases of dermatomyositis (DM) with nervous system involvement. Polyneuropathy was observed in both patients, and cerebral vasculitis was suspected in one patient. Histological examination of biopsied specimens of skeletal muscles, skin and sural nerves revealed vasculitis. Furthermore, vascular endothelial growth factor (VEGF) and VEGF receptors (VEGFRs) were overexpressed in vasculitic lesions. Although, VEGF and VEGFRs were not detected in biopsied specimens of skeletal muscle from normal subjects, they were observed in one of two patients with DM who did not exhibit neuropathy. These findings suggest the possibility that VEGF overproduction is associated with development of vasculitis in some cases of DM complicated with peripheral neuropathy."},"publication_date":"2003-12","publication_name":{"en":"Internal Medicine","ja":"Internal Medicine"},"volume":"Vol.42","number":"No.12","starting_page":"1233","ending_page":"1239","languages":["eng"],"referee":true,"identifiers":{"doi":["10.2169/internalmedicine.42.1233"],"issn":["0918-2918"]},"published_paper_type":"scientific_journal"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=345147","label":"url"}],"paper_title":{"en":"症例検討:妊婦に発症した脳梗塞の1例","ja":"症例検討:妊婦に発症した脳梗塞の1例"},"authors":{"en":[{"name":"細川 忍"},{"name":"戸根 沙織"},{"name":"Matsui Naoko"},{"name":"Kusunose Kenya"},{"name":"Kagawa Kumiko"},{"name":"山上 圭"},{"name":"廣島 裕也"},{"name":"田村 洋人"}],"ja":[{"name":"細川 忍"},{"name":"戸根 沙織"},{"name":"松井 尚子"},{"name":"楠瀬 賢也"},{"name":"賀川 久美子"},{"name":"山上 圭"},{"name":"廣島 裕也"},{"name":"田村 洋人"}]},"publication_date":"2017-10-10","publication_name":{"en":"The Journal of the Japanese Society of Internal Medicine","ja":"日本内科学会雑誌"},"volume":"Vol.106","number":"No.10","starting_page":"2265","ending_page":"2272","languages":["jpn"],"identifiers":{"doi":["10.2169/naika.106.2265"],"issn":["1883-2083"]},"published_paper_type":"research_institution"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=304013","label":"url"}],"paper_title":{"en":"脳室周囲および延髄背側の病変","ja":"脳室周囲および延髄背側の病変"},"authors":{"en":[{"name":"Miyazaki Yoshimichi"},{"name":"Matsui Naoko"},{"name":"Fujita Koji"},{"name":"Abe Takashi"},{"name":"高橋 利幸"},{"name":"Harada Masafumi"}],"ja":[{"name":"宮﨑 由道"},{"name":"松井 尚子"},{"name":"藤田 浩司"},{"name":"阿部 考志"},{"name":"高橋 利幸"},{"name":"原田 雅史"}]},"publication_date":"2014-10-10","publication_name":{"en":"Practical Currently","ja":"脳神経外科速報"},"volume":"Vol.24","number":"No.10","starting_page":"1100","ending_page":"1105","languages":["jpn"],"identifiers":{"issn":["0917-1495"]},"published_paper_type":"research_institution"},"priority":"input_data"} {"insert":{"user_id":"B000373105","type":"published_papers"},"similar_merge":{"see_also":[{"@id":"http://ci.nii.ac.jp/naid/130000142263/","label":"url"},{"@id":"https://www.ncbi.nlm.nih.gov/pubmed/20046006","label":"url"},{"@id":"https://cir.nii.ac.jp/crid/1390001204871227136/","label":"url"},{"@id":"https://web.db.tokushima-u.ac.jp/cgi-bin/edb_browse?EID=235418","label":"url"}],"paper_title":{"en":"Anti-neuronal antibodies in acute pandysautonomia.","ja":"Anti-neuronal antibodies in acute pandysautonomia."},"authors":{"en":[{"name":"Matsui Naoko"},{"name":"Mitsui Takao"},{"name":"Ohshima Yasushi"},{"name":"Yokoi Kenji"},{"name":"Kunishige Makoto"},{"name":"Yagi Fumikazu"},{"name":"Vernino Steven"},{"name":"Matsumoto Toshio"},{"name":"Kaji Ryuji"}],"ja":[{"name":"松井 尚子"},{"name":"三ツ井 貴夫"},{"name":"Ohshima Yasushi"},{"name":"Yokoi Kenji"},{"name":"Kunishige Makoto"},{"name":"Yagi Fumikazu"},{"name":"Vernino Steven"},{"name":"松本 俊夫"},{"name":"梶 龍兒"}]},"description":{"en":"We encountered two patients with acute pandysautonomia who subacutely exhibited extensive autonomic dysfunction after antecedent infections. Although these patients had been suffering from autonomic disturbance for several months, they both had a good clinical course after plasma exchange and intravenous immunoglobulin therapy. Thin-layer chromatography (TLC)-immunostaining did not demonstrate any antibodies against gangliosides, but immunoblot analysis showed antibodies against a neuroblastoma cell line, SH-SY5Y, in serum samples. Furthermore, ganglionic acetylcholine receptor autoantibodies were detected in one patient. These findings suggest that neuronal antibodies against the autonomic nervous system play an important role in the pathogenesis of acute pandysautonomia.","ja":"We encountered two patients with acute pandysautonomia who subacutely exhibited extensive autonomic dysfunction after antecedent infections. Although these patients had been suffering from autonomic disturbance for several months, they both had a good clinical course after plasma exchange and intravenous immunoglobulin therapy. Thin-layer chromatography (TLC)-immunostaining did not demonstrate any antibodies against gangliosides, but immunoblot analysis showed antibodies against a neuroblastoma cell line, SH-SY5Y, in serum samples. Furthermore, ganglionic acetylcholine receptor autoantibodies were detected in one patient. These findings suggest that neuronal antibodies against the autonomic nervous system play an important role in the pathogenesis of acute pandysautonomia."},"publication_date":"2010-01-01","publication_name":{"en":"Internal Medicine","ja":"Internal Medicine"},"volume":"Vol.49","number":"No.1","starting_page":"73","ending_page":"77","languages":["eng"],"identifiers":{"doi":["10.2169/internalmedicine.49.2788"],"issn":["1349-7235"]},"published_paper_type":"research_institution"},"priority":"input_data"}