BRAIN and NERVE－神経研究の進歩72巻6号

2020年06月発行

特集 前頭側頭葉変性症の今日的理解

前頭側頭葉変性症概念の歴史的変遷

著者： 川勝忍¹ 小林良太² 坂本和貴³ 大谷浩一²

所属機関： ¹福島県立医科大学会津医療センター精神医学講座 ²山形大学医学部医学科精神医学講座 ³山形県立保健医療大学大学院作業療法学分野

ページ範囲：P.561 - P.573

文献概要

前頭側頭葉変性症（FTLD）の概念は，ピックが側頭葉や前頭葉の限局性脳萎縮で局在的な症状を呈すると指摘し，のちにそれがピック病と命名されたことに始まる。その後長年，ピック球の有無はピック病の診断には無関係とされてきたが，現在ではピック球を有する症例のみをピック病と呼ぶ。ピックの症例は左側頭葉萎縮例が多く，実はピック病ではなく意味性認知症に相当しTDP-43病理が疑われる。当初のピック病は現在のFTDと同義で多様な背景病理を持っている。

参考文献

文献1）日本神経学会（監修）, 「認知症疾患治療ガイドライン」作成委員会（編）: 前頭側頭型認知症. 認知症疾患治療ガイドライン2017. 医学書院, 東京, 2017, pp263-280

2）Rascovsky K, Hodges JR, Knopman D, Mendez MF, Kramer JH, et al: Sensitivity of revised diagnostic criteria for the behavioral variant of frontotemporal dementia. Brain 134: 2456-2477, 2011

3）Neary D, Snowden JS, Gustafson L, Passant U, Stuss D, et al: Frontotemporal lobar degeneration. A consensus on clinical diagnostic criteria. Neurology 51: 1546-1554, 1998

4）Boeve BF: Links between frontotemporal lobar degeneration, corticobasal degeneration, progressive supranuclear palsy, and amyotrophic lateral sclerosis. Alzheimer Dis Assoc Disord 21: S31-S38, 2007

5）Gorno-Tempini ML, Hillis AE, Weintraub S, Kertesz A, Mendez M, et al: Classification of primary progressive aphasia and its variants. Neurology 76: 1006-1014, 2011

6）Kertesz A: Introductory nosology. Kertesz A, Munoz DG (eds): Pick's Disease and Pick Complex. Wiley, Hoboken, 1998, pp1-11

7）Pick A: Uber die Beziehungen der senilen Hirnatrophie zur Aphasie. Prag Med Wchnschr 17: 165-167, 1892

8）池村義明: ピック病—短い報告で始まった長い物語. ドイツ精神医学の原典を読む. 医学書院, 2008, pp31-57

9）山縣 博: A Pick—Senile Hirnatrophie als Grundlage von Herderscheinungen. 精神医17: 1313-1317, 1975

10）田辺敬貴, 池田 学, 中川賀嗣, 山本晴子, 池尻義隆, 他: 語義失語と意味記憶障害. 失語症研12: 153-167, 1992

11）Kobayashi R, Hayashi H, Tokairin T, Kawakatsu S, Otani K: Suicide as a result of stereotypic behaviour in a case with semantic dementia. Psychogeriatrics 19: 80-82, 2019

12）Onari K, Spatz H: Anatomische Beiträge zur Lehre von Pickschen umschribenen Gro ßhirnrinden-Atrophie (“Picksche Krankheit”). Z Ges Neurol Psychiatr 101: 470-511, 1926

13）内原俊記: Pick病の病理像. 神経内科50: 342-348, 1999

14）Ikeda K: Basic pathology of corticobasal degeneration. Neuropathology 17: 127-133, 1997

15）Snowden JS, Rollinson S, Thompson JC, Harris JM, Stopford CL, et al: Distinct clinical and pathological characteristics of frontotemporal dementia associated with C9ORF72 mutations. Brain 135: 693-708, 2012

16）Ikeda K, Akiyama H, Iritani S, Kase K, Arai T, et al: Corticobasal degeneration with primary progressive aphasia and accentuated cortical lesion in superior temporal gyrus: case report and review. Acta Neuropathol 92: 534-539, 1996

17）Karenberg A: Zur Frühgeschichte der Pickschen Erkrankung. Fortschr Neurol Psychiatr 69: 545-550, 2001

18）松下正明: 大成潔—その生涯と業績. 老年精医4: 824-834, 1987

19）土谷邦秋: Pick病の歴史と概念の変遷. 神経内科50: 321-328, 1999

20）池田研二: FTLD-UとPick球のないPick病—臨床と病理. Brain Nerve 61: 1328-1336, 2009

21）松下正明: Pick病再考. 池田 学（責任編集）: 前頭側頭型認知症の臨床. 中山書店, 東京, 2010, pp82-90

22）Constantinidis J, Richard J, Tissot R: Pick's disease. Histological and clinical correlations. Eur Neurol 11: 208-217, 1974

23）The Lund and Manchester groups: Clinical and neuropathological criteria for frontotemporal dementia. J Neurol Neurosurg Psychiatry 57: 416-418, 1994

24）Mitsuyama Y: Presenile dementia with motor neuron disease in Japan: clinico-pathological review of 26 cases. J Neurol Neurosurg Psychiatry 47: 953-959, 1984

25）Okamoto K, Murakami N, Kusaka H, Yoshida M, Hashizume Y, et al: Ubiquitin-positive intraneuronal inclusions in the extramotor cortices of presenile dementia patients with motor neuron disease. J Neurol 239: 426-430, 1992

26）Jackson M, Lennox G, Lowe J: Motor neuron disease-inclusion dementia. Neurodegeneration 5: 339-350, 1996

27）Arai T, Hasegawa M, Akiyama H, Ikeda K, Nonaka T, et al: TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Biochem Biophys Res Commun 351: 602-611, 2006

28）Neumann M, Sampathu DM, Kwong LK, Truax AC, Micsenyi MC, et al: Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science 314: 130-133, 2006

29）McKhann GM, Albert MS, Grossman M, Miller B, Dickson D, et al: Clinical and pathological diagnosis of frontotemporal dementia: report of the Work Group on Frontotemporal Dementia and Pick's Disease. Arch Neurol 58: 1803-1809, 2001

30）横田 修, 土谷邦秋: Pick病の臨床と病理. 臨床神経49: 235-248, 2009

31）Kawakami I, Kobayashi Z, Arai T, Yokota O, Nonaka T, et al: Chorea as a clinical feature of the basophilic inclusion body disease subtype of fused-in-sarcoma-associated frontotemporal lobar degeneration. Acta Neuropathol Commun 4: 36, 2016［doi: 10.1186/s40478-016-0304-9］

32）Seo SW, Thibodeau MP, Perry DC, Hua A, Sidhu M, et al: Early vs late age at onset frontotemporal dementia and frontotemporal lobar degeneration. Neurology 90: e1047-e1056, 2018［doi: 10.1212/WNL.0000000000005163］

33）Armstrong MJ, Litvan I, Lang AE, Bak TH, Bhatia KP, et al: Criteria for the diagnosis of corticobasal degeneration. Neurology 80: 496-503, 2013

34）日本神経学会（監修）, 「認知症疾患治療ガイドライン」作成委員会（編）: 大脳皮質基底核変性症. 認知症疾患治療ガイドライン2017. 医学書院, 東京, 2017, pp289-294

35）Ikeda C, Yokota O, Nagao S, Ishizu H, Morisada Y, et al: Corticobasal degeneration initially developing motor versus non-motor symptoms: a comparative clinicopathological study. Psychogeriatrics 14: 152-164, 2014

36）Höglinger GU, Respondek G, Stamelou M, Kurz C, Josephs KA, et al: Clinical diagnosis of progressive supranuclear palsy: The Movement Disorder Society Criteria. Mov Disord 32: 853-864, 2017

37）Mackenzie IR, Neumann M, Baborie A, Sampathu DM, Du Plessis D, et al: A harmonized classification system for FTLD-TDP pathology. Acta Neuropathol 122: 111-113, 2011

38）川勝 忍, 小林良太, 林 博史: 運動ニューロン疾患を伴う前頭側頭型認知症（湯浅・三山病）の病態と診断. 精神医57: 849-856, 2015

39）Majounie E, Renton AE, Mok K, Dopper EG, Waite A, et al: Frequency of the C9orf72 hexanucleotide repeat expansion in patients with amyotrophic lateral sclerosis and frontotemporal dementia: a cross-sectional study. Lancet Neurol 11: 323-330, 2012

40）Munoz-Garcia D, Ludwin SK: Classic and generalized variants of Pick's disease: a clinicopathological, ultrastructural, and immunocytochemical comparative study. Ann Neurol 16: 467-480, 1984

41）Mesulam MM: Slowly progressive aphasia without generalized dementia. Ann Neurol 11: 592-598, 1982

42）Mesulam MM: Primary progressive aphasia- Differentiation from Alzheimer's disease. Ann Neurol 22: 533-534, 1987

43）Chawluk JB, Mesulam MM, Hurtig H, Kushner M, Weintraub S, et al: Slowly progressive aphasia without generalized dementia: studies with positron emission tomography. Ann Neurol 19: 68-74, 1986

44）Mesulam MM: Primary progressive aphasia. Ann Neurol 49: 425-432, 2001

45）Gorno-Tempini ML, Brambati SM, Ginex V, Ogar J, Dronkers NF, et al: The logopenic/phonological variant of primary progressive aphasia. Neurology 71: 1227-1234, 2008

46）Bergeron D, Gorno-Tempini ML, Rabinovici GD, Santos-Santos MA, Seely W, et al: Prevalence of amyloid-β pathology in distinct variants of primary progressive aphasia. Ann Neurol 84: 729-740, 2018

47）小阪憲司: Pick病—日本における報告例を中心として. 臨精医11: 693-703, 1982

48）井村恒郎: 失語—日本語に於ける特性. 精神経誌47: 196-218, 1943

49）山鳥 重: 語義失語. Brain Nerve 63: 811-820, 2011

50）倉知正佳, 松原三郎: Pick病の臨床・病理と画像診断所見. 神経心理7: 10-18, 1991