【原著論文・症例報告】
1.

Ando T, Nakamura R, Kuru S, Yokoi D, Atsuta N, Koike H, Suzuki M, Hara K, Iguchi Y, Harada Y,Yoshida Y, Hattori M, Murakami A, Noda S, Kimura S, Sone J, Nakamura T, Goto Y, Mano K, Okada
H, Okuda S, Nishino I, Ogi T, Sobue G and Katsuno M. The wide-ranging clinical and genetic featuresin Japanese families with valosin-containing protein proteinopathy. Neurobiol Aging. 2021; 100:120.e121-120.e126.

2.

Ando T, Sato T, Kurahashi S, Kawaguchi Y, Kagaya Y, Ozawa Y, Hirano S, Goto Y, Mano K, Yokoi S,Nakamura T, Murakami A, Noda S, Kimura S, Sone J, Kuru S, Sobue G and Katsuno M. A case ofsporadic late-onset nemaline myopathy with monoclonal gammopathy of undetermined significance:long-term observation of neurological symptoms after autologous stem-cell transplantation. Nagoya J

Med Sci. 2021; 83(3): 641-647.
3.

Ando T, Yokoi F, Riku Y, Akagi A, Miyahara H, Hasegawa M, Katsuno M, Yoshida M and Iwasaki Y.
The hot cross bun sign in corticobasal degeneration. Neuropathology. 2021; 41(5): 376-380.

4.

Azuma F, Nokura K, Kako T, Yoshida M and Tatsumi S. An Autopsy Confirmed Neuromyelitis Optica
Spectrum Disorder with Extensive Brain White Matter Lesion and Optic Neuritis but Intact SpinalCord, Clinically Mimicking a Secondary Progressive Multiple Sclerosis-like Course. Intern Med. 2021.
7635-21.

5.

Boivin M, Deng J, Pfister V, Grandgirard E, Oulad-Abdelghani M, Morlet B, Ruffenach F, Negroni L,Koebel P, Jacob H, Riet F, Dijkstra A A, McFadden K, Clayton W A, Hong D, Miyahara H, Iwasaki Y,Sone J, Wang Z and Charlet-Berguerand N. Translation of GGC repeat expansions into a toxic
polyglycine protein in NIID defines a novel class of human genetic disorders: The polyG diseases.

Neuron. 2021; 109(11): 1825-1835.e1825.
6.

Dong T T, Akagi A, Nonaka T, Nakagaki T, Mihara B, Takao M, Iwasaki Y, Nishida N and Satoh K.
Formalin RT-QuIC assay detects prion-seeding activity in formalin-fixed brain samples from sporadic
Creutzfeldt-Jakob disease patients. Neurobiol Dis. 2021; 159: 105504.

7.

Fukuda H, Yamaguchi D, Nyquist K, Yabuki Y, Miyatake S, Uchiyama Y, Hamanaka K, Saida K,Koshimizu E, Tsuchida N, Fujita A, Mitsuhashi S, Ohbo K, Satake Y, Sone J, Doi H, Morihara K,
Okamoto T, Takahashi Y, Wenger A M, Shioda N, Tanaka F, Matsumoto N and Mizuguchi T. Fatherto-offspring transmission of extremely long NOTCH2NLC repeat expansions with contractions:
genetic and epigenetic profiling with long-read sequencing. Clin Epigenetics. 2021; 13(1): 204.

8.

Hayashi N, Sone J, Fukami Y, Yoshida Y, Kuno S, Shimada K, Atsuta N, Nakamura T, Higuchi O andKatsuno M. Severe myasthenia gravis with anti-LRP4 antibodies and Hodgkin lymphoma. MuscleNerve. 2021; 63(1): E2-e4.
9.

Hayashi Y, Iwasaki Y, Yoshikura N, Yamada M, Kimura A, Inuzuka T, Miyahara H, Goto Y, Nishino I,Yoshida M and Shimohata T. Clinicopathological findings of a mitochondrial encephalopathy, lactic
acidosis, and stroke-like episodes/Leigh syndrome overlap patient with a novel m.3482A>G mutationin MT-ND1. Neuropathology. 2021; 41(1): 84-90.

10. Iwasaki Y, Mori K, Ito M, Kawai Y, Akagi A, Riku Y, Miyahara H, Kobayashi A, Kitamoto T and Yoshida
M. System degeneration in an MM1-type sporadic Creutzfeldt-Jakob disease case with an unusually
prolonged akinetic mutism state. Prion. 2021; 15(1): 12-20.
11. Iwase T, Yoshida M, Iwasaki Y, Suzuki S, Yabata H, Koizumi R, Moriyoshi H and Yazawa I. Selective
extension of cerebral vascular calcification in an autopsy case of Fahr's syndrome associated withasymptomatic hypoparathyroidism. Neuropathology. 2021; 41(5): 387-395.
12. Kikumoto M, Nezu T, Shiga Y, Motoda A, Toko M, Kurashige T, Ueno H, Takahashi T, Morino H,Sone J, Iwasaki Y, Sobue G and Maruyama H. Case of Neuronal Intranuclear Inclusion Disease With
Dynamic Perfusion Changes Lacking Typical Signs on Diffusion-Weighted Imaging. Neurol Genet.
2021; 7(4): e601.
13. Kinoshita M, Oyanagi K, Kondo Y, Ishizawa K, Ishihara K, Yoshida M, Inoue T, Mitsuyama Y, Yoshida
K, Yamada M, Sekijima Y and Ikeda S I. Pathologic basis of the preferential thinning of thecorpus
callosum in adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP).
eNeurologicalSci. 2021; 22: 100310.
14. Kotani S, Fukazawa R, Takezawa H, Banba M, Sone J and Fujii A. [A comparative study of three casesof neuronal intranuclear inclusion disease (NIID)]. Rinsho Shinkeigaku. 2021; 61(3): 194-199.
15. Kuzuya M, Shi R Q, Yanagawa M, Watanabe K, Samizo S, Ando R, Miyahara H, Iwasaki Y and Yoshida
M. Cerebral pathological findings in long-lived patient with Werner syndrome and dementia. Geriatr

Gerontol Int. 2021; 21(8): 743-745.
16. Lövestam S, Schweighauser M, Matsubara T, Murayama S, Tomita T, Ando T, Hasegawa K, Yoshida
M, Tarutani A, Hasegawa M, Goedert M and Scheres S H W. Seeded assembly in vitro does not
replicate the structures of α-synuclein filaments from multiple system atrophy. FEBS Open Bio. 2021;
11(4): 999-1013.

17. Miwa A, Hirano M, Torii Y, Sekiguchi H, Habuchi C, Fujishiro H, Yoshida M, Iwai K, Kawashima K
and Iritani S. Clinicopathological investigation of the background of cognitive decline in elderly
schizophrenia. Acta Neuropsychiatr. 2021; 33(2): 85-91.
18. Miyahara H, Natsumeda M, Kanemura Y, Yamasaki K, Riku Y, Akagi A, Oohashi W, Shofuda T,Yoshioka E, Sato Y, Taga T, Naruke Y, Ando R, Hasegawa D, Yoshida M, Sakaida T, Okada N,Watanabe H, Ozeki M, Arakawa Y, Yoshimura J, Fujii Y, Suenobu S, Ihara K, Hara J, Kakita A, Yoshida
M and Iwasaki Y. Topoisomerase IIβ immunoreactivity (IR) co-localizes with neuronal marker-IR but
not glial fibrillary acidic protein-IR in GLI3-positive medulloblastomas: an immunohistochemicalanalysis of 124 medulloblastomas from the Japan Children's Cancer Group. Brain Tumor Pathol. 2021;
38(2): 109-121.
19. Natsumeda M, Miyahara H, Yoshimura J, Nakata S, Nozawa T, Ito J, Kanemaru Y, Watanabe J,
Tsukamoto Y, Okada M, Oishi M, Hirato J, Wataya T, Ahsan S, Tateishi K, Yamamoto T, Rodriguez
F J, Takahashi H, Hovestadt V, Suva M L, Taylor M D, Eberhart C G, Fujii Y and Kakita A. GLI3 Is
Associated With Neuronal Differentiation in SHH-Activated and WNT-Activated Medulloblastoma. JNeuropathol Exp Neurol. 2021; 80(2): 129-136.
20. Nihonmatsu-Kikuchi N, Yu X J, Matsuda Y, Ozawa N, Ito T, Satou K, Kaname T, Iwasaki Y, Akagi A,Yoshida M, Toru S, Hirokawa K, Takashima A, Hasegawa M, Uchihara T and Tatebayashi Y. Essential
roles of plexin-B3(+) oligodendrocyte precursor cells in the pathogenesis of Alzheimer's disease.

Commun Biol. 2021; 4(1): 870.
21. Oda R, Fujikura M, Hayashi T, Matsuya M, Sone J and Shimohama S. [A case of neuronal intranuclear
inclusion disease with serial MRI changes observed from before onset of forgetfulness]. Rinsho

Shinkeigaku. 2021; 61(11): 727-732.
22. Ohno Y, Ikeda T, Sakurai K, Yamada K, Tomonari T, Iwasaki Y, Yoshida M and Matsukawa N. Rapid
Progression of White Matter Signal Changes and Frontotemporal Atrophy in Globular Glial Tauopathy.

J Neuropathol Exp Neurol. 2021; 80(5): 480-483.
23. Riku Y, Seilhean D, Duyckaerts C, Boluda S, Iguchi Y, Ishigaki S, Iwasaki Y, Yoshida M, Sobue G andKatsuno M. Pathway from TDP-43-Related Pathology to Neuronal Dysfunction in Amyotrophic
Lateral Sclerosis and Frontotemporal Lobar Degeneration. Int J Mol Sci. 2021; 22(8): 3843. (Review)
24. Riku Y, Yoshida M, Tamura T, Kamijo M, Yasui K, Kameyama T, Katsuno M, Sobue G and Iwasaki Y.
Unexpected postmortem diagnoses in cases of clinically diagnosed amyotrophic lateral sclerosis.

Neuropathology. 2021; 41(6): 457-467. (Review)
25. Sano K, Iwasaki Y, Yamashita Y, Irie K, Hosokawa M, Satoh K and Mishima K. Tyrosine 136
phosphorylation of α-synuclein aggregates in the Lewy body dementia brain: involvement of serine
129 phosphorylation by casein kinase 2. Acta Neuropathol Commun. 2021; 9(1): 182.
26. Shi Y, Zhang W, Yang Y, Murzin A G, Falcon B, Kotecha A, van Beers M, Tarutani A, Kametani F,Garringer H J, Vidal R, Hallinan G I, Lashley T, Saito Y, Murayama S, Yoshida M, Tanaka H, KakitaA, Ikeuchi T, Robinson A C, Mann D M A, Kovacs G G, Revesz T, Ghetti B, Hasegawa M, Goedert Mand Scheres S H W. Structure-based classification of tauopathies. Nature. 2021; 598(7880): 359-363.
27. Tachi K, Takata T, Kume K, Sone J, Kobara H, Deguchi K, Kawakami H and Masaki T. Long-term
MRI findings of adult-onset neuronal intranuclear inclusion disease. Clin Neurol Neurosurg. 2021;
201: 106456.
28. Tamura A, Fujino Y, Sone J and Shiga K. Temporal Changes in Brain Magnetic Resonance Imaging
Findings over 16 Years in a Patient with Neuronal Intranuclear Inclusion Disease. Intern Med. 2021;
60(15): 2483-2486.
29. Tarutani A, Miyata H, Nonaka T, Hasegawa K, Yoshida M, Saito Y, Murayama S, Robinson A C, Mann
D M A, Tomita T and Hasegawa M. Human tauopathy-derived tau strains determine the substrates
recruited for templated amplification. Brain. 2021; 144(8): 2333-2348.
30. Toko M, Ohshita T, Kurashige T, Morino H, Kume K, Yamashita H, Sobue G, Iwasaki Y, Sone J,Kawakami H and Maruyama H. FXTAS is difficult to differentiate from neuronal intranuclear inclusion
disease through skin biopsy: a case report. BMC Neurol. 2021; 21(1): 396.
【商業誌】
1. 安藤 孝志, 中村 亮一, 吉田 眞理, 勝野 雅央．
【多系統蛋白質症に関する最近の進歩】多系統蛋白質症の概念．脳神経内科 (2434-3285)．95 巻 1 号，Page 94-103，2021 年．
2. Andre M. Mansoor（著），田中竜馬（翻訳），安藤 孝志（分担翻訳）．フレームワークで考える内科診断（Frameworks for Internal medicine），Chapter 41，多発ニューロパチー．Page 467-480，2021 年．
3. 安藤 孝志，勝野 雅央，吉田 眞理，岩崎 靖．知っておきたい脊髄炎とトピックス—帯状疱疹に伴う髄節性運動麻痺 Segmental zoster paresis．脊椎脊髄ジャーナル．34 巻 5 号，Page 305-312，2021 年．

4. 曽根 淳．神経核内封入体病の診断の進歩．脳神経疾患最新の治療 2021-2023．南江堂．Page 9~13，2021 年．
5. 曽根 淳．
【神経難病の今～疫学・成因・治療の研究最前線～】神経核内封入体病．Pharma Medica．
39 巻 3 号，Page 55-61．
6. 陸 雄一. 筋萎縮性側索硬化症（ALS）と類縁疾患とを見分ける．難病と在宅ケア．27 巻 7 号，Page39-43，2021 年．
7. 陸 雄一．Babinski-Nageotte 症候群と Cestan-Chenais 症候群．脳神経内科．94 巻 1 号，Page 50-54，2021 年．
【受賞】
1.

赤木 明生．ヒトプリオン病における異常プリオン蛋白質の非中枢神経組織への広がりの解明．優秀日本語展示賞．第 62 回日本神経病理学会学術集会，東京，2021.5.27-29．

2.

Takashi Ando. Hippocampal dominant-variant of multiple system atrophy. 一般演題優秀ポスター賞
セッション（基礎部門）．第 62 回日本神経学会学術大会，京都，2021.5.19-22．

3.

Hiroyuki Yabata. Transitional pathology of TDP-43 from nucleus to cytoplasm: An observation of 23
ALS autopsy cases. 一般演題優秀ポスター賞セッション（基礎部門）．第 62 回日本神経学会学術大会，京都，2021.5.19-22．