脳神経内科学講座【研究プロジェクト名および概要】
Ⅰ．アミロイドーシスの病態解析と治療法の開発
Ⅰ-1. 遺伝性トランスサイレチンアミロイドーシスのアミロイド沈着機構の解析と治療法開発に関する研究
Ⅰ-2. アミロイドーシス全般の診断法の確立と病態解析
Ⅰ-3. 野生型トランスサイレチンアミロイドーシスの病態解析
Ⅰ-4. 質量分析法によるアミロイドーシス病型診断法の開発
Ⅰ-5. 新規アミロイドーシスの病態解析
I-6. アルツハイマー病、脳アミロイドアンギオパチーの病態解析
Ⅱ．脳血管障害の病態解析と治療法開発
Ⅱ-1. CADASIL 並びに遺伝性脳小血管病の実態調査、診断、病態解析に関する研究
Ⅱ-2. 脳血管障害の MRI/CT・神経超音波・SPECT を用いた臨床解析並びに超急性期治療に関する研究
Ⅱ-3. Drip and ship システムによる脳卒中急性期診療体制の構築
Ⅱ-4. 抗血栓薬、脳保護薬、降圧薬および脂質異常症治療薬の臨床病型別治療効果の研究
Ⅲ．神経難病の病態解析と治療法に関する開発
Ⅲ-1. IRUD 拠点病院活動を基盤とした遺伝性希少疾患の診断体制構築
Ⅲ-2. パーキンソン病、多系統萎縮症の病態解析
Ⅲ-3. 疾患レジストリを基盤とした疾患バイオマーカーの開発
Ⅲ-4. 神経免疫疾患の病態解析
Ⅲ-5. 筋疾患の病態解析
Ⅳ．てんかんの病態解析
Ⅳ-1. てんかんの診断における定量的バイオマーカーに関する研究
Ⅳ-2. 急性期疾患における脳波解析
Ⅳ-3. 神経難病における脳波検査の有用性の検証
Ⅴ．中毒性神経疾患の病態解析と治療開発
Ⅴ-1. 有機水銀中毒（水俣病）の長期経過例の臨床像に関する研究
Ⅴ-2. 熊本地区におけるスモン患者の現状調査【教職員および大学院学生】
教 授
植田 光晴
特任教授
中島 誠
准教授
三隅 洋平
診療講師
植田 明彦
講師
中原 圭一
特任助教
進藤 誠悟
助 教
松原 崇一朗
助 教
野村 隼也
特任助教
水谷 浩徳
医 員
池ノ下 侑
医 員
村上 慶高
医 員
俵 明恵
医 員
今村 美智恵医 員
原田 しずか医 員
宮野 遼太朗
医 員
詹 翔叡
医 員
井村 真男
医 員
寺﨑 晃平
医 員
津川 貴博
特別研究員
田崎 雅義
大学院学生（博士課程） 山川 詩織【メールアドレス】【研究プロジェクト】
mitt@rb3.so-net.ne.jp
研究の統括nakazima04@gmail.com
Ⅱmisumiy1@kuh.kumamoto-u.ac.jp
Ⅰ, Ⅲ, Ⅴakihiko-shima@k4.dion.ne.jp
Ⅱ, Ⅴ97056kn@gmail.com
Ⅲ, Ⅴ
seigoshindou@gmail.com
Ⅱmatsubara.soichiro@kuh.kumamoto-u.ac.jp
Ⅱ, Ⅳ
caramelbox106@gmail.com
Ⅰ, Ⅲ
hironori03240325@gmail.com
Ⅰ, Ⅲ
s.ikenoshita1986@gmail.com
Ⅲyoshi.m.562@gmail.com
Ⅲ, ⅤⅢⅢ
Ⅱ, ⅢⅡ, Ⅲ, Ⅳ
Ⅱ, Ⅲ
Ⅱ, Ⅲ
Ⅱ, Ⅲ
Ⅱ, Ⅲ
Ⅰ, Ⅲ
Ⅰ, Ⅲ, Ⅴ

大学院学生（博士課程） 田口
社会人大学院学生
波止
社会人大学院学生
永沼
社会人大学院学生
嶋本社会人大学院学生
北川智朗
聡司
雅基
稔也
敬資

Ⅰ, Ⅱ, ⅢⅡ
Ⅱ
Ⅲ
Ⅰ, Ⅲ

【連絡先】
Tel: 096-373-5893
Fax: 096-373-5895【ホームページ】 http://kumadai-neurology.com/【特殊技術・特殊装置】
1. アミロイドーシスの診断と病態解析
2. ATTR アミロイドーシスの実験モデル
3. 質量分析装置（LC-MS/MS）
4. 自律神経機能検査
5． Notch3 解析

6. リアルタイム PCR 装置
7. 交差型超音波装置（アミロイド形成促進装置）
8. 神経難病レジストリ、バイオバンク
9. 自動免疫染色装置
10．神経難病のエクソーム解析【英文論文】
1. Benson MD, Berk JL, Dispenzieri A, Damy T, Gillmore JD, Hazenberg BP, Lavatelli F, Picken MM,Röcken C, Schönland S, Ueda M, Westermark P. Tissue biopsy for the diagnosis of amyloidosis:
experience from some centres. Amyloid, 29: 8-13, 2022
2. Tahara N, Lairez O, Endo J, Okada A, Ueda M, Ishii T, Kitano Y, Lee H, Russo E, Kubo T.
99mTechnetium-pyrophosphate scintigraphy: a practical guide for early diagnosis of transthyretin
amyloid cardiomyopathy. ESC Heart Fail, 9: 251-262, 2022
3. Ueda M. Transthyretin: its function and amyloid formation. Neurochem Int. 155: 105313, 2022
4. Misumi Y, Tabata Y, Tasaki M, Obayashi K, Yamakawa S, Nomura T, Ueda M. Binding of serum-derived
amyloid-associated proteins to amyloid fibrils. Amyloid, 2022 (in press)
5. Ando Y, Adams D, Benson MD, Berk J, Planté-Bordeneuve V, Coelho T, Conceição I, Ericzon B, Obici
L, Rapezzi C, Sekijima Y, Ueda M, Palladini G, Merlini G. Guidelines and new directions in thetherapy and monitoring of ATTRv amyloidosis. Amyloid, 29: 143-155, 2022
6. Nakajima M, Watari M, Ando Y, Ueda M. Asymptomatic deep venous thrombosis identified on routine
screening in patients with hospitalized neurological diseases. J Clin Neurosci, 102:13-20, 2022
7. Naganuma M, Inatomi Y, Yonehara T, Nakajima M, Ueda M. Underdosed direct oral anticoagulants inatrial fibrillation patients reduce stroke severity and improve outcome. Cerebrovasc Dis, 51:473480, 2022
8. Tasaki M, Oishi T, Ueda M. EGF-containing fibulin-like extracellular matrix protein 1 (EFEMP1)
Ueda A, Nakajima M, Misumi Y, Nakahara K, Shinriki S, Tasaki M, Matsui H, Ueda M. Detection ofVascular Notch3 deposits in Unfixed Frozen Skin Biopsy Sample in CADASIL. Front Neurol, 3:881528,2022
9. amyloid deposits in the lower rectum from aged patient with bloody stools. Pathol Int, 72: 217218, 2022
10. Tasaki M, Nomura T, Uchiyama K, Misumi Y, Nakahara K, Oyama Y, Uesugi N, Ueda M. Fibrinogen
Aalpha-chain amyloidosis associated with a rare frameshift pathogenic variant p.
Arg547GlyfsTer21. Amyloid, 29: 276-277, 2022
11. Yamakawa M, Nakahara K, Nakanishi T, Nomura T, Ueda M. Miller Fisher Syndrome following
vaccination against SARS-CoV-2: a case report. Intern Med, 61: 1067-1069, 2022
12. Misumi Y, Ando Y, Ueda M. Early transverse tubule involvement in cardiomyocytes in hereditarytransthyretin amyloidosis; A possible cause of cardiac events. Cardiovasc Pathol, 61:107458, 2022
13. Inoue Y, Tasaki M, Masuda T, Misumi Y, Nomura T, Yukio Ando Y, Ueda M. α-Enolase reduces
cerebrovascular Aβ deposits by protecting Aβ amyloid formation. Cell Mol Life Sci, 79: 462,2022.
14. Yi K, Nakajima M, Ikeda T, Yoshigai M, Ueda M. Modified Rankin Scale assessment on telephonesurvey using a simple questionnaire. J Stroke Cerebrovasc Dis, 31: 106695, 2022
15. Shimamoto T, Uchino K, Mori A, Nojima K, Iyama J, Misumi Y, Ueda M, Uchino M. Effects ofintensive exercise on cognitive dysfunction in patients with pure cerebellar degeneration: asingle-arm pilot study. Ann Rehabil Med, 46: 263-273, 2022
16. Mizutani H, Sato Y *, Yamazaki M, Yoshizawa T, Ando Y, Ueda M, Yamagata K.* SIRT7 deficiency
protects against Aβ42-induced apoptosis through the regulation of NOX4-derived reactive oxygenspecies production in SH-SY5Y cells. Int J Mol Sci, 23: 9027, 2022
17. Nakahara K, Takamatsu K, Kudo N, Ito T, Ueda M. Histopathological features of Gerhardt syndrome
in a patient with multiple system atrophy: An autopsy case report. Cureus, 14: e30415, 2022

18. Takeuchi Y, Nomura T, Nakahara K, Ueda M. Autoimmune polyglandular syndrome with refractory gait
disturbance. Intern Med, 2022 (in press)
19. Singu T, Nakajima M, Ueda A, Nakahara K, Nomura T, Kojima S, Inoue T, Ueda M. Transient optic
neuritis and perineuritis associated with anti-MOG antibody after SARS-CoV-2 mRNA vaccination.
Neuroimmunology Reports, 2: 100077, 2022
20. Kinoshita Y, Ikeda T, Miyamura T, Ueda Y, Yoshida Y, Ueda M, Haraoka S, Kushima H, Ishii H.
Nodular Pulmonary Amyloidosis Associated with Sjögren's Syndrome. Intern Med, 61: 877-881, 2022
21. Takashio S, Yamada T, Nishi M, Morioka M, Nakashima N, Hirakawa K, Hanatani S, Usuku H, Yamamoto
E, Ueda M, Matsushita K, Kaikita K, Tsujita K. Sex-related differences in the clinicalcharacteristics of wild-type transthyretin amyloidosis cardiomyopathy. J Cardiol, 79: 50-57, 2022
22. Takahashi K, Sasaki D, Yamashita M, Sakaue T, Enomoto D, Morioka H, Uemura S, Okura T, Ikeda S,Kono T, Hiratsuka Y, Saijo A, Yamamura N, Kitazawa S, Yamashita T, Ueda M. Amyloid deposit
corresponds to technetium-99mpyrophosphate accumulation in abdominal fat of patients withtransthyretin cardiac amyloidosis. J Nucl Cardiol, 29: 3126-3136, 2022
23. Hayashi H, Oda S, Kidoh M, Nakaura T, Morita K, Nagayama Y, Yoneda T, Takashio S, Misumi Y, Ueda
M, Tsujita K, Hirai T. Can myocardial susceptibility quantification be an imaging biomarker forcardiac amyloidosis? Jpn J Radiol, 40: 500-507. 2022
24. Yumoto S, Doi K, Higashi T, Shimao Y, Ueda M, Ishihara A, Adachi Y, Ishiodori H, Honda S, Baba H.
Intra-abdominal bleeding caused by amyloid transthyretin amyloidosis in the gastrointestinal
tract: a case report. Clin J Gastroenterol, 15: 140-145, 2022
25. Takashio S, Tokitsu T, Matsukawa M, Sakaino N, Fujimoto K, Sakamoto T, Noda K, Tsunoda R, Misumi
I, Hirai N, Doi H, Koide S, Mizuno Y, Hirose T, Kurokawa H, Kajiwara I, Ohba K, Miyamoto S, Araki
S, Yamamoto E, Matsushita K, Ueda M, Tsujita K; Kumamoto Cardiac Amyloid Survey Investigators.
Incidence, clinical characteristics, and diagnostic approach in transthyretin amyloid
cardiomyopathy: The Kumamoto Cardiac Amyloidosis Survey. J Cardiol, 80: 49-55, 2022
26. Moriyama H, Kitakata H, Endo J, Ikura H, Sano M, Tasaki M, Sakai S, Ueda M, Fukuda K. Step-bystep typing for the accurate diagnosis of concurrent light chain and transthyretin cardiac
amyloidosis. ESC Heart Fail, 9: 1474-1477, 2022
27. Ikura H, Kitakata H, Endo J, Moriyama H, Sano M, Tsujikawa H, Sawano M, Masuda T, Ohki T, Ueda M,Kosaki K, Fukuda K. Three patients of transthyretin amyloidosis in a Japanese family withamyloidogenic transthyretin Thr49Ser (p.Thr69Ser) variant. Eur J Med Genet, 65: 104451, 2022
28. Usuku H, Takashio S, Yamamoto E, Yamada T, Egashira K, Morioka M, Nishi M, Komorita T, Oike F,Tabata N, Ishii M, Yamanaga K, Fujisue K, Sueta D, Arima Y, Araki S, Oda S, Misumi Y, Kawano H,Matsushita K, Ueda M, Matsui H, Tsujita K. Prognostic value of right ventricular global
longitudinal strain in transthyretin amyloid cardiomyopathy. J Cardiol, 80: 56-63, 202229. Inoue Y, Nakajima M, Inatomi Y, Masuda T, Misumi Y, Nomura T, Uetani H, Hirai T, Ueda M.
Reversible periventricular hyperintensity lesions in cerebral amyloid angiopathy (CAA): A casemimicking CAA-related inflammation. Intern Med, 61:3581-3584, 2022
30. Oike F, Usuku H, Yamamoto E, Marume K, Takashio S, Ishii M, Tabata N, Fujisue K, Yamanaga K,Sueta D, Hanatani S, Arima Y, Araki S, Oda S, Kawano H, Soejima H, Matsushita K, Ueda M, Fukui T,Tsujita K. Utility of left atrial and ventricular strain for diagnosis of transthyretin amyloid
cardiomyopathy in aortic stenosis. ESC Heart Fail, 9: 1976-1986, 2022
31. Nakashima N, Takashio S, Morioka M, Nishi M, Yamada T, Hirakawa K, Ishii M, Tabata N, Yamanaga K,Fujisue K, Sueta D, Kanazawa H, Hoshiyama T, Hanatani S, Araki S, Usuku H, Yamamoto E, Ueda M,
Matsushita K, Tsujita K. A simple staging system using biomarkers for wild-type transthyretin
amyloid cardiomyopathy in Japan. ESC Heart Fail, 9: 1731-1739, 2022
32. Takahashi Y, Date H, Oi H, Adachi T, Imanishi N, Kimura E, Takizawa H, Kosugi S, Matsumoto N,Kosaki K, Matsubara Y; IRUD Consortium, Mizusawa H. Six years' accomplishment of the Initiativeon Rare and Undiagnosed Diseases: nationwide project in Japan to discover causes, mechanisms, andcures. J Hum Genet, 67: 505-513, 2022
33. Kawano Y, Hata H, Takashio S, Tsujita K, Ueda M, Matsuoka M. Daratumumab, lenalidomide anddexamethasone in newly diagnosed systemic light chain amyloidosis patients associated withmultiple myeloma. Br J Haematol, 198: e38-e41, 2022
34. Nishi M, Takashio S, Morioka M, Fujiyama A, Nakashima N, Hirakawa K, Hanatani S, Usuku H,Yamamoto E, Kidoh M, Oda S, Gushima R, Matsushita K, Fukushima S, Ueda M, Tsujita K. Extracardiac
biopsy sensitivity in transthyretin amyloidosis cardiomyopathy patients with positive 99 mTclabeled pyrophosphate scintigraphy findings. Circ J, 86:1113-1120, 2022
35. Morioka M, Takashio S, Nakashima N, Nishi M, Fujiyama A, Hirakawa K, Hanatani S, Usuku H,Yamamoto E, Kidoh M, Oda S, Matsushita K, Ueda M, Tsujita K. Correlation between cardiac images,biomarkers, and amyloid load in wild-type transthyretin amyloid cardiomyopathy. J Am Heart Assoc,
11:e024717, 2022
36. Tahara N, Honda A, Ueda M, Fukumoto Y. Popeye’s sign and transthyretin amyloidosis. Eur Heart J,
43:3000, 2022

37. Sato K, Oda S, Kidoh M, Ishiuchi S, Kamimoto M, Kanaya H, Nomura T, Kuyama N, Ueda M, Tsujita K,Hirai T. Myocarditis in myasthenia gravis: Multiparametric evaluation using cardiac magnetic
resonance imaging. Circ J, 86:1788, 2022
38. Matsushita H, Misumi Y, Masuda T, Okada M, Inoue F, Ueda M, Ando Y. Urinary transthyretin as abiomarker in ATTRv amyloidosis. Pathophysiology, 29: 333-343, 2022
39. Tasaki M, Misumi Y, Nomura T, Kamba T, Ueda M. Detection of semenogelin 1 amyloidosis throughimmunohistochemical staining with novel antibodies developed based on mass spectrometric peptide
mapping analysis. Amyloid, 29: 278-280, 2022
40. Iwahashi N, Ikezaki M, Komohara Y, Fujiwara Y, Noguchi T, Nishioka K, Sakai K, Nishio K, Ueda M,Ihara Y, Uchimura K, Ino K, Nishitsuji K. Cytoplasmic p53 aggregates accumulated in p53-mutatedcancer correlate with poor prognosis. PNAS Nexus, 1: pgac128, 2022
41. Inoue M, Muta K, Fouad A, Mohammed A, Onodera R, Higashi T, Ueda M, ,Ando Y, Arima H, Jono H,Motoyama K. Potential use of Dendrimer-based TTR-CRISPR pDNA polyplex for ocular amyloidosis.
Biol Pharm Bull, 45: 1660-1668, 2022
42. Inoue M, Higashi T, Hayashi Y, Onodera R, Fujisawa K, Taharabaru T, Yokoyama R, Ouchi K, Misumi
Y, Ueda M, Inoue Y, Mizuguchi M, Saito T, Saido T, Ando Y, Arima H, Motoyama K, Jono H.
Multifunctional therapeutic cyclodextrin-appended dendrimer complex for treatment of systemic andlocalized amyloidosis. ACS Appl Mater Interfaces, 14: 40599-40611, 2022
43. Kidoh M, Oda S, Takashio S, Hirakawa K, Kawano Y, Shiraishi S, Hayashi H, Nakaura T, Nagayama Y,Funama Y, Ueda M, Tsujita K, Hirai T. Diagnostic performance of CT-derived extracellular volume
fraction versus myocardium-to-lumen signal ratio for detecting cardiac amyloidosis. Radiology,2022 (in press)
44. Yagita K, Noguchi H, Koyama S, Hamasaki H, Komori T, Aishima S, Kosaka T, Ueda M, Komohara Y,Watanabe A, Sasagasako N, Ninomiya T, Oda Y, Honda H. Chronological changes in the expression
pattern of hippocampal prion proteins during disease progression in sporadic Creutzfeldt-Jakob
disease MM1 subtype. J Neuropathol Exp Neurol, 81: 900-909, 2022
45. Shinriki S, Hirayama M, Nagamachi A, Yokoyama A, Kawamura T, Kanai A, Kawai H, Iwakiri J, Riu L,Maeshiro M, Tungalag S, Tasaki M, Ueda M, Tomizawa K, Kataoka N, Ideue T, Suzuki Y, Asai K, Tani
T, Inaba T, Matsui H. DDX41 coordinates RNA splicing and transcriptional elongation to prevent
DNA replication stress in hematopoietic cells. Leukemia, 36: 2605-2620, 2022
46. Takahashi K, Morioka H, Sasaki D, Yamamura N, Kitazawa S, Ueda M, Sakaue T, Enomoto D, Uemura S,Okura T, Ikeda S. Two autopsy cases of wild-type transthyretin cardiac amyloidosis who died 10days after technetium-99mpyrophosphate scintigraphy. J Nucl Cardiol, 2022 (in press)
47. Maeda-Ogata S, Tahara N, Tahara A, Bekki M, Honda A, Sugiyama Y, Igata S, Abe T, Sekijima Y, Ueda
M, Ando Y, Fukumoto Y. Treatment response to Tafamidis quantitatively assessed by serial bone
scintigraphy in transthyretin amyloid cardiomyopathy. J Nucl Cardiol, 2022 (in press)
48. Yamakawa M, Nakane S, Ihara E, Tawara N, Ikeda H, Igarashi Y, Komohara Y, Takamatsu K, Ikeda T,Tomita Y, Murai S, Ando Y, Mukaino A, Ogawa Y, Ueda M. A novel murine model of autoimmune
dysautonomia by α3 nicotinic AChR immunization. Front Neurosci, 16: 1006923, 2022
49. Usuku H, Yamamo E, Sueta D, Noguchi M, Fujisaki T, Egashira K, Morioka M, Komorita T, Oike F,
Fujisue K, Hanatani S, Arima Y, Takashio S, Oda S, Kawano H, Matsushita K, Ueda M, Matsui H,Tsujita K. Time-dependent change of relative apical longitudinal strain index in patients withwild-type transthyretin amyloid cardiomyopathy. Int J Cardiol Heart Vasc, 43, 101146, 2022
50. Matsushita H, Sameshima G, Fukunari A, Okada M, Inoue F, Ueda M, Ando Y. Suppression of amyloid
fibril formation by UV irradiation. Journal of Radiation Research and Applied Sciences, 2022 (inpress)
51. Kuyama N, Takashio S, Nagakura T, Imamura M, Takamatsu K, Takae M, Hirakawa K, Yamanaga K,Hanatani S, Yamamoto E, Matsushita K, MD, Ueda M, Tsujita K. Anti-Kv1.4 antibody positivemyocarditis and myositis despite absence of thymoma and anti-acetylcoline receptor antibody. JACC
Case Rep, 2022 (in press)
52. Goto M, Tasaki M, Ueda M. Early onset Congo red-positive fibrillary glomerulonephritis associatedwith glomerular DNAJB9 deposits mimicking renal amyloidosis. Pathol Int, 2022 (in press)
53. Takahashi K, Hiratsuka Y, Sasaki D, Sakaue T, Enomoto D, Morioka H, Uemura S, Okura T, Ikeda S,Kono T, Iwamura T, Yamamura N, Kitazawa S, Ueda M. 99mTc-pyrophosphate scintigraphy can image
tracer uptake in skeletal trunk muscles of transthyretin cardiac amyloidosis Brief title:
Extracardiac PYP uptake in ATTR-CA. Clin Nucl Med, 48:18-24, 2023
54. Hirai T, Abe O, Nakamura M, Inui S, Uetani H, Ueda M, Azuma M. Brain structural changes inpatients with chronic methylmercury poisoning in Minamata. Brain Res, 2023 (in press)
55. Naiki H, Yamaguchi A, Sekijima Y, Ueda M, Ohashi K, Hatakeyama K, Ikeda Y, Hoshii Y, ShintaniDomoto Y, Miyagawa-Hayashino A, Tsujikawa H, Endo J, Arai T, Ando Y. Steep increase in the numberof transthyretin-positive cardiac biopsy cases in Japan: evidence obtained by the nation-wide
pathology consultation for the typing diagnosis of amyloidosis. Amyloid, 2023 (in press)
56. Yamashita S, Nagatoshi A, Takeuchi Y, Nishino I, Ueda M. Myopathic changes caused by protein
aggregates in adult-onset spinal muscular atrophy. Neuropathology, 2023 (in press)