Skip to main content

Advertisement

SpringerLink
Log in

Clinicopathological characterization of Pick’s disease versus frontotemporal lobar degeneration with ubiquitin/TDP-43-positive inclusions

  • Original Paper
  • Published:
Acta Neuropathologica Aims and scope Submit manuscript

Abstract

Although frontotemporal lobar degeneration with ubiquitin/TDP-43-positive inclusions (FTLD-TDP) and Pick’s disease are common pathological substrates in sporadic FTLD, clinical differentiation of these diseases is difficult. We performed a retrospective review of medical records and semiquantitative examination of neuronal loss of 20 sporadic FTLD-TDP and 19 Pick’s disease cases. Semantic dementia as the first syndrome developed only in FTLD-TDP patients. Impaired speech output in the early stage was five times more frequent in Pick’s disease than in FTLD-TDP. The total frequency of asymmetric motor disturbances (e.g., parkinsonism, pyramidal signs, and contracture) during the course was significantly more frequent in FTLD-TDP (78%) than in Pick’s disease cases (14%). Asymmetric pyramidal signs were found in 7 of 13 FTLD-TDP cases with corticospinal tract degeneration similar to primary lateral sclerosis. Frontotemporal dementia as the first syndrome was noted in both FTLD-TDP (28%) and Pick’s disease cases (64%); however, only FTLD-TDP cases subsequently developed asymmetric motor disturbances, and some of the cases further exhibited hemineglect. Concordant with these clinical findings, degeneration in the temporal cortex, caudate nucleus, putamen, globus pallidus, substantia nigra, and corticospinal tract was significantly more severe in FTLD-TDP, and degeneration in the frontal cortex tended to be more severe in Pick’s disease. Given these findings, the initial impairment of semantic memory or comprehension and subsequent asymmetric motor disturbances in sporadic FTLD patients predict sporadic FTLD-TDP rather than Pick’s disease, while initial behavioral symptoms or non-fluent aphasia without subsequent asymmetric motor disturbances predict Pick’s disease rather than sporadic FTLD-TDP.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6

Similar content being viewed by others

References

  1. Arai T, Hasegawa M, Akiyama H, Ikeda K, Nonaka T, Mori H, Mann D, Tsuchiya K, Yoshida M, Hashizume Y, Oda T (2006) 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

    Article  PubMed  CAS  Google Scholar 

  2. Arima K, Miyasaka Y (1985) An autopsy case of Pick’s disease, with special reference to light and electron microscopical findings of Pick bodies and ballooned neurons. Neuropathology 6:325–339 in Japanese with English abstract

    Google Scholar 

  3. Baker M, Mackenzie IR, Pickering-Brown SM, Gass J, Rademakers R, Lindholm C, Snowden J, Adamson J, Sadovnick AD, Rollinson S, Cannon A, Dwosh E, Neary D, Melquist S, Richardson A, Dickson D, Berger Z, Eriksen J, Robinson T, Zehr C, Dickey CA, Crook R, McGowan E, Mann D, Boeve B, Feldman H, Hutton M (2006) Mutations in progranulin cause tau-negative frontotemporal dementia linked to chromosome 17. Nature 442:916–919

    Article  PubMed  CAS  Google Scholar 

  4. Beck J, Rohrer JD, Campbell T, Isaacs A, Morrison KE, Goodall EF, Warrington EK, Stevens J, Revesz T, Holton J, Al-Sarraj S, King A, Scahill R, Warren JD, Fox NC, Rossor MN, Collinge J, Mead S (2008) A distinct clinical, neuropsychological and radiological phenotype is associated with progranulin gene mutations in a large UK series. Brain 131:706–720

    Article  PubMed  Google Scholar 

  5. Boeve BF, Baker M, Dickson DW, Parisi JE, Giannini C, Josephs KA, Hutton M, Pickering-Brown SM, Rademakers R, Tang-Wai D, Jack CR Jr, Kantarci K, Shiung MM, Golde T, Smith GE, Geda YE, Knopman DS, Petersen RC (2006) Frontotemporal dementia and parkinsonism associated with the IVS1+1G->A mutation in progranulin: a clinicopathologic study. Brain 129:3103–3114

    Article  PubMed  Google Scholar 

  6. Braak H, Braak E (1991) Neuropathological stageing of Alzheimer-related changes. Acta Neuropathol 82:239–259

    Article  PubMed  CAS  Google Scholar 

  7. Broe M, Hodges JR, Schofield E, Shepherd CE, Kril JJ, Halliday GM (2003) Staging disease severity in pathologically confirmed cases of frontotemporal dementia. Neurology 60:1005–1011

    PubMed  CAS  Google Scholar 

  8. Cairns NJ, Perry RH, Jaros E, Burn D, McKeith IG, Lowe JS, Holton J, Rossor MN, Skullerud K, Duyckaerts C, Cruz-Sanchez FF, Lantos PL (2003) Patients with a novel neurofilamentopathy: dementia with neurofilament inclusions. Neurosci Lett 341:177–180

    Article  PubMed  CAS  Google Scholar 

  9. Cairns NJ, Bigio EH, Mackenzie IR, Neumann M, Lee VM, Hatanpaa KJ, White CL 3rd, Schneider JA, Grinberg LT, Halliday G, Duyckaerts C, Lowe JS, Holm IE, Tolnay M, Okamoto K, Yokoo H, Murayama S, Woulfe J, Munoz DG, Dickson DW, Ince PG, Trojanowski JQ, Mann DM, Consortium for Frontotemporal Lobar Degeneration (2007) Neuropathologic diagnostic and nosologic criteria for frontotemporal lobar degeneration: consensus of the Consortium for Frontotemporal Lobar Degeneration. Acta Neuropathol 114:5–22

    Article  PubMed  Google Scholar 

  10. Cruts M, Gijselinck I, van der Zee J, Engelborghs S, Wils H, Pirici D, Rademakers R, Vandenberghe R, Dermaut B, Martin JJ, van Duijn C, Peeters K, Sciot R, Santens P, De Pooter T, Mattheijssens M, Van den Broeck M, Cuijt I, Vennekens K, De Deyn PP, Kumar-Singh S, Van Broeckhoven C (2006) Null mutations in progranulin cause ubiquitin-positive frontotemporal dementia linked to chromosome 17q21. Nature 442:920–924

    Article  PubMed  CAS  Google Scholar 

  11. Davies RR, Hodges JR, Kril JJ, Patterson K, Halliday GM, Xuereb JH (2005) The pathological basis of semantic dementia. Brain 128:1984–1995

    Article  PubMed  Google Scholar 

  12. Davion S, Johnson N, Weintraub S, Mesulam MM, Engberg A, Mishra M, Baker M, Adamson J, Hutton M, Rademakers R, Bigio EH (2007) Clinicopathologic correlation in PGRN mutations. Neurology 69:1113–1121

    Article  PubMed  CAS  Google Scholar 

  13. Dickson DW, Josephs KA, Amador-Ortiz C (2007) TDP-43 in differential diagnosis of motor neuron disorders. Acta Neuropathol 114:71–79

    Article  PubMed  CAS  Google Scholar 

  14. Forman MS, Farmer J, Johnson JK, Clark CM, Arnold SE, Coslett HB, Chatterjee A, Hurtig HI, Karlawish JH, Rosen HJ, Van Deerlin V, Lee VM, Miller BL, Trojanowski JQ, Grossman M (2006) Frontotemporal dementia: clinicopathological correlations. Ann Neurol 59:952–962

    Article  PubMed  Google Scholar 

  15. Fukui T, Sugita K, Kawamura M, Shiota J, Nakano I (1996) Primary progressive apraxia in Pick’s disease: a clinicopathologic study. Neurology 47:467–473

    PubMed  CAS  Google Scholar 

  16. Gass J, Cannon A, Mackenzie IR, Boeve B, Baker M, Adamson J, Crook R, Melquist S, Kuntz K, Petersen R, Josephs K, Pickering-Brown SM, Graff-Radford N, Uitti R, Dickson D, Wszolek Z, Gonzalez J, Beach TG, Bigio E, Johnson N, Weintraub S, Mesulam M, White CL 3rd, Woodruff B, Caselli R, Hsiung GY, Feldman H, Knopman D, Hutton M, Rademakers R (2006) Mutations in progranulin are a major cause of ubiquitin-positive frontotemporal lobar degeneration. Hum Mol Genet 15:2988–3001

    Article  PubMed  CAS  Google Scholar 

  17. Grimes DA, Bergeron CB, Lang AE (1999) Motor neuron disease-inclusion dementia presenting as cortical-basal ganglionic degeneration. Mov Disord 14:674–680

    Article  PubMed  CAS  Google Scholar 

  18. Guerreiro RJ, Santana I, Bras JM, Revesz T, Rebelo O, Ribeiro MH, Santiago B, Oliveira CR, Singleton A, Hardy J (2008) Novel progranulin mutation: screening for PGRN mutations in a Portuguese series of FTD/CBS cases. Mov Disord 23:1269–1273

    Article  PubMed  Google Scholar 

  19. Hasegawa M, Arai T, Nonaka T, Kametani F, Yoshida M, Hashizume Y, Beach TG, Buratti E, Baralle F, Morita M, Nakano I, Oda T, Tsuchiya K, Akiyama H (2008) Phosphorylated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Ann Neurol 64:60–70

    Article  PubMed  CAS  Google Scholar 

  20. Higaki F, Yokota O, Ohishi M (2008) Factors predictive of survival after percutaneous endoscopic gastrostomy in the elderly: is dementia really a risk factor? Am J Gastroenterol 103:1011–1016

    Article  PubMed  Google Scholar 

  21. Hodges JR, Davies RR, Xuereb JH, Casey B, Broe M, Bak TH, Kril JJ, Halliday GM (2004) Clinicopathological correlates in frontotemporal dementia. Ann Neurol 56:399–406

    Article  PubMed  Google Scholar 

  22. Ikeda K (2000) Neuropathological discrepancy between Japanese Pick’s disease without Pick bodies and frontal lobe degeneration type of frontotemporal dementia proposed by Lund and Manchester Group. Neuropathology 20:76–82

    Article  PubMed  CAS  Google Scholar 

  23. Ikeda K, Akiyama H, Arai T, Ueno H, Tsuchiya K, Kosaka K (2002) Morphometrical reappraisal of motor neuron system of Pick’s disease and amyotrophic lateral sclerosis with dementia. Acta Neuropathol 104:21–28

    Article  PubMed  Google Scholar 

  24. Ikeda M, Ikeda K, Endo M, Haga C, Mizutani Y (1994) An autopsied case of slowly progressive Pick’s disease. Seishin Igaku (Clinical Psychiatry) 36:1167–1171 in Japanese with English abstract

    Google Scholar 

  25. Ikeda M, Ishikawa T, Tanabe H (2004) Epidemiology of frontotemporal lobar degeneration. Dementia Geriatr Cogn Disord 17:265–268

    Article  Google Scholar 

  26. Ishino H, Ikeda H (1972) Four autopsy cases of Pick’s disease—clinicopathological findings (in Japanese with English abstract). Kyushu Neuro psychiatry 18:82–86

    Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  28. Josephs KA, Holton JL, Rossor MN, Braendgaard H, Ozawa T, Fox NC, Petersen RC, Pearl GS, Ganguly M, Rosa P, Laursen H, Parisi JE, Waldemar G, Quinn NP, Dickson DW, Revesz T (2003) Neurofilament inclusion body disease: a new proteinopathy? Brain 126:2291–2303

    Article  PubMed  Google Scholar 

  29. Josephs KA, Holton JL, Rossor MN, Godbolt AK, Ozawa T, Strand K, Khan N, Al-Sarraj S, Revesz T (2004) Frontotemporal lobar degeneration and ubiquitin immunohistochemistry. Neuropathol Appl Neurobiol 30:369–373

    Article  PubMed  CAS  Google Scholar 

  30. Josephs KA, Ahmed Z, Katsuse O, Parisi JF, Boeve BF, Knopman DS, Petersen RC, Davies P, Duara R, Graff-Radford NR, Uitti RJ, Rademakers R, Adamson J, Baker M, Hutton ML, Dickson DW (2007) Neuropathologic features of frontotemporal lobar degeneration with ubiquitin-positive inclusions with progranulin gene (PGRN) mutations. J Neuropathol Exp Neurol 66:142–151

    Article  PubMed  CAS  Google Scholar 

  31. Josephs KA, Lin WL, Ahmed Z, Stroh DA, Graff-Radford NR, Dickson DW (2008) Frontotemporal lobar degeneration with ubiquitin-positive, but TDP-43-negative inclusions. Acta Neuropathol 116:159–167

    Article  PubMed  CAS  Google Scholar 

  32. Kawamura M, Mochizuki S (1999) Primary progressive apraxia. Neuropathology 19:249–258

    Article  Google Scholar 

  33. Kertesz A, McMonagle P, Blair M, Davidson W, Munoz DG (2005) The evolution and pathology of frontotemporal dementia. Brain 128:1996–2005

    Article  PubMed  Google Scholar 

  34. Knibb JA, Xuereb JH, Patterson K, Hodges JR (2006) Clinical and pathological characterization of progressive aphasia. Ann Neurol 59:156–165

    Article  PubMed  Google Scholar 

  35. Knopman DS, Mastri AR, Frey WH 2nd, Sung JH, Rustan T (1990) Dementia lacking distinctive histologic features: a common non-Alzheimer degenerative dementia. Neurology 40:251–256

    PubMed  CAS  Google Scholar 

  36. Kosaka K (1976) On aphasia of Pick’s disease—a review of our own 3 cases and 49 autopsy cases in Japan (in Japanese). Seishin Igaku (Clinical Psychiatry) 18:1181–1189

    Google Scholar 

  37. Kosaka K, Matsushita M, Oyanagi S, Hato K, Mehraein P (1980) Pick’s disease with concomitant traumatic brain damages–Five autopsied cases and a critical review of literatures (in Japanese with English abstract). Seishin Shinkeigaku Zasshi (Psychiatria et Neurologia Japonica) 82:33–47

    CAS  Google Scholar 

  38. Kosaka K, Ikeda K, Kobayashi K, Hamamoto J, Matsushita M (1985) On pyramidal tract lesions in Pick’s disease (in Japanese). Clinical Psychiatry 27:1171–1178

    Google Scholar 

  39. Le Ber I, Camuzat A, Hannequin D, Pasquier F, Guedj E, Rovelet-Lecrux A, Hahn-Barma V, van der Zee J, Clot F, Bakchine S, Puel M, Ghanim M, Lacomblez L, Mikol J, Deramecourt V, Lejeune P, de la Sayette V, Belliard S, Vercelletto M, Meyrignac C, Van Broeckhoven C, Lambert JC, Verpillat P, Campion D, Habert MO, Dubois B, Brice A, French research network on FTD/FTD-MND (2008) Phenotype variability in progranulin mutation carriers: a clinical, neuropsychological, imaging and genetic study. Brain 131:732–746

    Article  PubMed  Google Scholar 

  40. Mackenzie IR (2007) The neuropathology and clinical phenotype of FTD with progranulin mutations. Acta Neuropathol 114:49–54

    Article  PubMed  Google Scholar 

  41. Mackenzie IR, Foti D, Woulfe J, Hurwitz TA (2008) Atypical frontotemporal lobar degeneration with ubiquitin-positive, TDP-43-negative neuronal inclusions. Brain 131:1282–1293

    Article  PubMed  Google Scholar 

  42. Mackenzie IR, Neumann M, Bigio EH, Cairns NJ, Alafuzoff I, Kril J, Kovacs GG, Ghetti B, Halliday G, Holm IE, Ince PG, Kamphorst W, Revesz T, Rozemuller AJ, Kumar-Singh S, Akiyama H, Baborie A, Spina S, Dickson DW, Trojanowski JQ, Mann DM (2009) Nomenclature for neuropathologic subtypes of frontotemporal lobar degeneration: consensus recommendations. Acta Neuropathol 117:15–18

    Article  PubMed  Google Scholar 

  43. Masaki K, Maeda M, Furuhashi N, Kowa H (1996) Slowly-progressive aphasia and its symptoms—considered in reference to its related literature (in Japanese with English abstract). Kitasato Med 26:397–411

    Google Scholar 

  44. Masellis M, Momeni P, Meschino W, Heffner R Jr, Elder J, Sato C, Liang Y, St George-Hyslop P, Hardy J, Bilbao J, Black S, Rogaeva E (2006) Novel splicing mutation in the progranulin gene causing familial corticobasal syndrome. Brain 129:3115–3123

    Article  PubMed  Google Scholar 

  45. Mii T, Sudo K, Hayashi Y (1969) Ein Fall der Pickschen Krankheit (in Japanese with German abstract). Kyushu Neuro-psychiatry 15:106–113

    Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  47. Munoz DG, Dickson DW, Bergeron C, Mackenzie IR, Delacourte A, Zhukareva V (2003) The neuropathology and biochemistry of frontotemporal dementia. Ann Neurol 54(Suppl 5):S24–S28

    Article  PubMed  Google Scholar 

  48. Neary D, Snowden JS, Gustafson L, Passant U, Stuss D, Black S, Freedman M, Kertesz A, Robert PH, Albert M, Boone K, Miller BL, Cummings J, Benson DF (1998) Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria. Neurology 51:1546–1554

    PubMed  CAS  Google Scholar 

  49. Neumann M, Sampathu DM, Kwong LK, Truax AC, Micsenyi MC, Chou TT, Bruce J, Schuck T, Grossman M, Clark CM, McCluskey LF, Miller BL, Masliah E, Mackenzie IR, Feldman H, Feiden W, Kretzschmar HA, Trojanowski JQ, Lee VM (2006) Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science 314:130–133

    Article  PubMed  CAS  Google Scholar 

  50. Obi K, Akiyama H, Kondo H, Shimomura Y, Hasegawa M, Iwatsubo T, Mizuno Y, Mochizuki H (2008) Relationship of phosphorylated alpha-synuclein and tau accumulation to Abeta deposition in the cerebral cortex of dementia with Lewy bodies. Exp Neurol 210:409–420

    Article  PubMed  CAS  Google Scholar 

  51. Oda T, Tsuchiya K, Arai T, Togo T, Uchikado H, de Silva R, Lees A, Akiyama H, Haga C, Ikeda K, Kato M, Kato Y, Hara T, Onaya M, Hori K, Teramoto H, Tominaga I (2007) Pick’s disease with Pick bodies: an unusual autopsy case showing degeneration of the pontine nucleus, dentate nucleus, Clarke’s column, and lower motor neuron. Neuropathology 27:81–89

    Article  PubMed  Google Scholar 

  52. Onari K, Spatz H (1926) Anatomische Beiträge zur Lehre von Pickschen umschriebenen Größhirnrinden Atrophie (Picksche Krankheit). Z Ges Neurol Psychiatr 101:470–511

    Article  Google Scholar 

  53. Ota S, Tsuchiya K, Anno M, Akiyama H (2005) An autopsy case of dementia lacking distinctive histologic features showing semantic dementia. No To Shinkei 57:233–238

    PubMed  Google Scholar 

  54. Oyanagi S, Tanaka M, Omori T, Matsuchita M, Ishii T (1979) Regular arrangements of tubular structures in Pick bodies formed in an autopsied case of Pick’s disease (in Japanese with English abstract). Shinkei Kenkyū no Shimpo (Adv in Neurol Sci) 23:441–451

    Google Scholar 

  55. Paviour DC, Lees AJ, Josephs KA, Ozawa T, Ganguly M, Strand C, Godbolt A, Howard RS, Revesz T, Holton JL (2004) Frontotemporal lobar degeneration with ubiquitin-only-immunoreactive neuronal changes: broadening the clinical picture to include progressive supranuclear palsy. Brain 127:2441–2451

    Article  PubMed  CAS  Google Scholar 

  56. Pick A (1892) Ueber die Beziehungen der senilen Hirnatrophie zur Aphasie. Prager Med Wochenschr 17:165–167

    Google Scholar 

  57. Pickering-Brown SM (2007) Progranulin and frontotemporal lobar degeneration. Acta Neruopathol 114:39–47

    Article  Google Scholar 

  58. Pickering-Brown SM, Rollinson S, Du Plessis D, Morrison KE, Varma A, Richardson AM, Neary D, Snowden JS, Mann DM (2008) Frequency and clinical characteristics of progranulin mutation carriers in the Manchester frontotemporal lobar degeneration cohort: comparison with patients with MAPT and no known mutations. Brain 131:721–731

    Article  PubMed  Google Scholar 

  59. Ratnavalli E, Brayne C, Dawson K, Hodges JR (2002) The prevalence of frontotemporal dementia. Neurology 58:1615–1621

    PubMed  CAS  Google Scholar 

  60. Rabinovici GD, Furst AJ, O’Neil JP, Racine CA, Mormino EC, Baker SL, Chetty S, Patel P, Pagliaro TA, Klunk WE, Mathis CA, Rosen HJ, Miller BL, Jagust WJ (2007) 11C-PIB PET imaging in Alzheimer disease and frontotemporal lobar degeneration. Neurology 68:1205–1212

    Article  PubMed  CAS  Google Scholar 

  61. Roeber S, Mackenzie IR, Kretzschmar HA, Neumann M (2008) TDP-43-negative FTLD-U is a significant new clinico-pathological subtype of FTLD. Acta Neuropathol 116:147–157

    Article  PubMed  CAS  Google Scholar 

  62. Rossor MN, Revesz T, Lantos PL, Warrington EK (2000) Semantic dementia with ubiquitin-positive tau-negative inclusion bodies. Brain 123:267–276

    Article  PubMed  Google Scholar 

  63. Shi J, Shaw CL, Du Plessis D, Richardson AM, Bailey KL, Julien C, Stopford C, Thompson J, Varma A, Craufurd D, Tian J, Pickering-Brown S, Neary D, Snowden JS, Mann DM (2005) Histopathological changes underlying frontotemporal lobar degeneration with clinicopathological correlation. Acta Neuropathol 110:501–512

    Article  PubMed  Google Scholar 

  64. Snowden JS, Pickering-Brown SM, Mackenzie IR, Richardson AM, Varma A, Neary D, Mann DM (2006) Progranulin gene mutations associated with frontotemporal dementia and progressive non-fluent aphasia. Brain 129:3091–3102

    Article  PubMed  CAS  Google Scholar 

  65. Snowden J, Neary D, Mann D (2007) Frontotemporal lobar degeneration: clinical and pathological relationships. Acta Neuropathol 114:31–38

    Article  PubMed  Google Scholar 

  66. Spina S, Murrell JR, Huey ED, Wassermann EM, Pietrini P, Grafman J, Ghetti B (2007) Corticobasal syndrome associated with the A9D progranulin mutation. J Neuropathol Exp Neurol 66:892–900

    Article  PubMed  CAS  Google Scholar 

  67. Tsuchiya K, Ishizu H, Nakano I, Kita Y, Sawabe M, Haga C, Kuyama K, Nishinaka T, Oyanagi K, Ikeda K, Kuroda S (2001) Distribution of basal ganglia lesions in generalized variant of Pick’s disease: a clinicopathological study of four autopsy cases. Acta Neuropathol 102:441–448

    PubMed  CAS  Google Scholar 

  68. Tsuchiya K, Ikeda M, Hasegawa K, Fukui T, Kuroiwa T, Haga C, Oyanagi S, Nakano I, Matsushita M, Yagishita S, Ikeda K (2001) Distribution of cerebral cortical lesions in Pick’s disease with Pick bodies: a clinicopathological study of six autopsy cases showing unusual clinical presentations. Acta Neuropathol 102:553–571

    PubMed  CAS  Google Scholar 

  69. Tsuchiya K, Piao YS, Oda T, Mochizuki A, Arima K, Hasegawa K, Haga C, Kakita A, Hori K, Tominaga I, Yagishita S, Akiyama H, Takahashi H (2006) Pathological heterogeneity of the precentral gyrus in Pick’s disease: a study of 16 autopsy cases. Acta Neuropathol 112:29–42

    Article  PubMed  Google Scholar 

  70. Uchihara T, Tsuchiya K (2008) Neuropathology of Pick body disease. In: Duyckaerts C, Litvan I (eds) Handbook of clinical neurology, vol 89 (Third series), Dementias. Elsevier, Amsterdam, pp 415–430

  71. Yokota O, Sasaki K, Fujisawa Y, Takahashi J, Terada S, Ishihara T, Nakashima H, Kugo A, Ata T, Ishizu H, Kuroda S (2005) Frequency of early and late-onset dementias in a Japanese memory disorders clinic. Eur J Neurol 12:782–790

    Article  PubMed  CAS  Google Scholar 

  72. Yokota O, Tsuchiya K, Oda T, Ishihara T, de Silva R, Lees AJ, Arai T, Uchihara T, Ishizu H, Kuroda S, Akiyama H (2006) Amyotrophic lateral sclerosis with dementia: an autopsy case showing many Bunina bodies, tau-positive neuronal and astrocytic plaque-like pathologies, and pallido-nigral degeneration. Acta Neuropathol 112:633–645

    Article  PubMed  Google Scholar 

  73. Yokota O, Tsuchiya K, Itoh Y, Ishizu H, Akiyama H, Ikeda M, Kuzuhara S, Otomo E (2006) Frontotemporal lobar degeneration with ubiquitin pathology: an autopsy case presenting with semantic dementia and upper motor neuron signs with a clinical course of 19 years. Acta Neuropathol 112:739–749

    Article  PubMed  Google Scholar 

  74. Yokota O, Tsuchiya K, Terada S, Ishizu H, Uchikado H, Ikeda M, Oyanagi K, Nakano I, Murayama S, Kuroda S, Akiyama H (2008) Basophilic inclusion body disease and neuronal intermediate filament inclusion disease: a comparative clinicopathological study. Acta Neuropathol 115:561–575

    Article  PubMed  Google Scholar 

Download references

Acknowledgments

We would like to thank Ms. C. Haga (Department of Neuropathology, Tokyo Institute of Psychiatry) and Mr. A. Sasaki for help with the production of the manuscript. This work was supported by a grant-in-aid for scientific research from the Ministry of Education, Culture, Sports, Science and Technology (14570957) and a research grant from the Zikei Institute of Psychiatry. This work was supported by a grant-in-aid for scientific research from the Ministry of Education, Culture, Sports, Science and Technology (14570957) and a research grant from the Zikei Institute of Psychiatry.

Author information

Authors and Affiliations

  1. Department of Neuropathology, Tokyo Institute of Psychiatry, 2-1-8 Kamikitazawa, Setagaya-ku, Tokyo, 156-8585, Japan

    Osamu Yokota, Kuniaki Tsuchiya, Tetsuaki Arai & Haruhiko Akiyama

  2. Department of Neuropsychiatry, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, 2-5-1 Shikata-cho, Okayama, 700-8558, Japan

    Osamu Yokota, Hidetoshi Yoshida, Seishi Terada, Hideki Ishizu & Shigetoshi Kuroda

  3. Department of Laboratory Medicine and Pathology, Tokyo Metropolitan Matsuzawa Hospital, Tokyo, Japan

    Kuniaki Tsuchiya

  4. Department of Neurology, Tokyo Metropolitan Matsuzawa Hospital, Tokyo, Japan

    Kuniaki Tsuchiya

  5. Department of Pathology, Kanagawa Rehabilitation Center, Kanagawa, Japan

    Saburo Yagishita

  6. Department of Pathology, National Defense Medical College, Saitama, Japan

    Osamu Matsubara

  7. Department of Neurology, Doctoral Program in Medical Sciences for Control of Pathological Processes, Graduate School of Comprehensive Human Sciences, University of Tsukuba, Ibaraki, Japan

    Akihide Mochizuki & Akira Tamaoka

  8. Department of Neurology, Showa University School of Medicine, Tokyo, Japan

    Mitsuru Kawamura

  9. Department of Laboratory Medicine, Zikei Institute of Psychiatry, Okayama, Japan

    Hideki Ishizu

Authors
  1. Osamu Yokota
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Kuniaki Tsuchiya
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Tetsuaki Arai
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Saburo Yagishita
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Osamu Matsubara
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Akihide Mochizuki
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Akira Tamaoka
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Mitsuru Kawamura
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Hidetoshi Yoshida
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Seishi Terada
    View author publications

    You can also search for this author in PubMed Google Scholar

  11. Hideki Ishizu
    View author publications

    You can also search for this author in PubMed Google Scholar

  12. Shigetoshi Kuroda
    View author publications

    You can also search for this author in PubMed Google Scholar

  13. Haruhiko Akiyama
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Osamu Yokota.

Electronic supplementary material

Below is the link to the electronic supplementary material.

Supplementary material 1 (XLS 45 kb)

Supplementary material 2 (XLS 41.5 kb)

Rights and permissions

Reprints and permissions

About this article

Cite this article

Yokota, O., Tsuchiya, K., Arai, T. et al. Clinicopathological characterization of Pick’s disease versus frontotemporal lobar degeneration with ubiquitin/TDP-43-positive inclusions. Acta Neuropathol 117, 429–444 (2009). https://doi.org/10.1007/s00401-009-0493-4

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00401-009-0493-4

Keywords

Search

Navigation