Skip to main content

Advertisement

SpringerLink
Log in

Primary progressive aphasia as the initial manifestation of corticobasal degeneration and unusual tauopathies

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

Abstract

The clinical, neuroradiological, neuropathological and biochemical findings in four patients with primary progressive aphasia and tauopathy are described. The aphasic syndrome preceded by several years the appearance of other symptoms in every case. Asymmetrical apraxia with alien hand phenomenon occurred in one case. Frontotemporal symptoms occurred in three cases, but progressed to dramatic cognitive devastation in only one of these. Generalized dementia consistent with probable Alzheimer's disease (AD) developed with time in another. Cerebral computer tomography scans, magnetic resonance imaging and SPECT studies revealed marked asymmetries in one case, and showed nonspecific cerebral atrophy in the remaining ones. The neuropathological examination revealed typical corticobasal degeneration (CBD) in one case; CBD and AD in another; and atypical CBD, argyrophilic grain disease (AGD) and α-synucleinopathy consistent with Parkinson's disease in a third. Unique neuropathological findings were found in the remaining case. This was characterized by severe cerebral atrophy, marked neuronal loss in the cerebral cortex and abnormal tau deposition in neurons of the cerebral cortex, diencephalon and brain stem. Ballooned neurons, Pick bodies, generalized cortical neurofibrillary tangles and astrocytic plaques were absent. However, massive globular inclusions, containing phospho-tau, occurred in glial cells, mainly oligodendrocytes, in the white matter. Biochemical studies of frontal homogenates revealed four bands of 73/74, 68, 64 and 60 kDa of phosphorylated tau (using antibodies recognizing phospho-tau Thr181, Ser262 and Ser422) in the patient with AD and CBD, suggesting a predominant AD pattern in this case. Two bands of 68 and 64 kDa of phospho-tau were recovered in the sarkosyl-insoluble fraction in the other three cases. This pattern is similar to that found in CBD, progressive supranuclear palsy and AGD. Taken together, the present series further supports pure and combined CBD as causes of primary progressive aphasia, and they extend the hypothesis that primary progressive aphasia may be the initial symptom of distinct tauopathies.

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.
Fig. 7.
Fig. 8.
Fig. 9.
Fig. 10.
Fig. 11.

Similar content being viewed by others

References

  1. Arima K, Uesugi H, Fujita I, Sakurai Y, Oyanagi S, Andoh S, Izumiyama Y, Inose T (1994) Corticonigral degeneration with neuronal achromasia presenting with primary progressive aphasia: ultrastructural and immunocytochemical studies. J Neurol Sci 127:186–197

    Article  CAS  PubMed  Google Scholar 

  2. Arima K, Ueda K, Sunohara N, Arakawa K, Hirai S, Nakamura M, Tonozuka-Uehara H, Kawai M (1998) NACP/α-synuclein immunoreactivity in fibrillary components of neuronal and oligodendroglial cytoplasmic inclusions in the pontine nuclei in multiple system atrophy. Acta Neuropathol 96:439–444

    Article  PubMed  Google Scholar 

  3. Benson DF, Zaias BW (1991) Progressive aphasia: a case with post-mortem correlation. Neuropsychiatr Neuropsylchol Behav Neurol 4:215–223

    Google Scholar 

  4. Bergeron C, Pollanen M, Weyer L, Black S, Lang A (1996) Unusual clinical presentation of cortical-basal ganglionic degeneration. Ann Neurol 40:893–900

    PubMed  Google Scholar 

  5. Bergeron C, Davis A, Lang AE (1998) Corticobasal ganglionic degeneration and progressive supranuclear palsy presenting with cognitive decline. Brain Pathol 8:355–365

    CAS  PubMed  Google Scholar 

  6. Bigio EH, Lipton AM, Yen SH, Hutton ML, Baker M, Nacharaju P, White ChL, Davies P, Lin W, Dickson DW (2001) Frontal lobe dementia with novel tauopathy: sporadic multiple system tauopathy with dementia. J Neuropathol Exp Neurol 60:328–341

    Google Scholar 

  7. Boeve B, Dickson D, Duffy J, Bartleson J, Trenerry M, Petersen R (2003) Progressive nonfluent aphasia and subsequent aphasic dementia associated with atypical progressive supranuclear palsy. Eur Neurol 49:72–78

    Article  PubMed  Google Scholar 

  8. Braak H, Braak E (1989) Cortical and subcortical argyrophilic grains characterize a disease associated with adult onset dementia. Neuropathol Appl Neurobiol 15:13–26

    CAS  PubMed  Google Scholar 

  9. Braak H, Braak E (1998) Argyrophilic grain disease: frequency of occurrence in different age categories and neuropathological diagnostic criteria. J Neural Transm 105:801–819

    CAS  PubMed  Google Scholar 

  10. Braak H, Braak E (1999) Temporal sequence of Alzheimer's disease-related pathology. Cereb Cortex 14:475–512

    Google Scholar 

  11. Buée L, Delacourte A (1999) Comparative biochemistry of tau in progressive supranuclear palsy, corticobasal degeneration, FTDP-17 and Pick's disease. Brain Pathol 9:681–693

    PubMed  Google Scholar 

  12. Buée L, Bussière T, Buée-Scherrer V, Delacourte A, Hof PR (2000) Tau isoforms, phosphorylation and role in neurodegenerative disorders. Brain Res Rev 33:95–130

    PubMed  Google Scholar 

  13. Burn DJ, Jaros E (2001) Multiple system atrophy: cellular and molecular pathology. J Clin Pathol: Mol Pathol 54:419–426

    Google Scholar 

  14. Del Tredici K, Rüb U, Vos RAI de, Bohl JRE, Braak H (2002) Where does Parkinson disease pathology begin in the brain? J Neuropathol Exp Neurol 61:413–426

    PubMed  Google Scholar 

  15. Delisle MB, Murrell JR, Richardson R, Trofatter JA, Rascol O, Soulages X, Mohr M, Calvas P, Ghetti B (1999) A mutation at codon 279 (N279 K) in exon 10 of the tau gene causes tauopathy with dementia and supranuclear palsy. Acta Neuropathol 98:62–77

    Article  CAS  PubMed  Google Scholar 

  16. Dickson DW (2002) Progressive supranuclear palsy and corticobasal degeneration. In: Hof PR, Mobbs CV (eds) Functional neurobiology of aging. Academic Press, San Diego, pp 165–171

  17. Dickson DW, Yen SH, Suzuki KI, Davies P, Garcia JH, Hirano A (1986) Ballooned neurons in select neurodegenerative diseases contain phosphorylated neurofilament epitopes. Acta Neuropathol 71:216–223

    CAS  PubMed  Google Scholar 

  18. Dickson DW, Lin WL, Liu WK, Yen SH (1999) Multiple system atrophy: a sporadic synucleinopathy. Brain Pathol 9:721–732

    CAS  PubMed  Google Scholar 

  19. Dickson DW, Bergeron C, Chin SS, Duyckaerts C, Horoupian D, Ikeda K, Jellinger K, Lantos PL, Lippa CF, Mirra SS, Tabaton M, Vonsattel JP, Wakabayashi K, Litvan I (2002) Office of rare diseases neuropathologic criteria for corticobasal degeneration. J Neuropathol Exp Neurol 61:935–946

    CAS  PubMed  Google Scholar 

  20. Feany MB, Dickson DW (1995) Widespread cytoskeletal pathology characterizes corticobasal degeneration. Am J Pathol 146:1388–1396

    CAS  PubMed  Google Scholar 

  21. Feany MB, Dickson DW (1996) Neurodegenerative disorders with extensive tau pathology: a comparative study and review. Ann Neurol 40:139–148

    CAS  PubMed  Google Scholar 

  22. Ferrer I, Barrachina M, Tolnay M, Rey MJ, Vidal N, Carmona M, Blanco R, Puig B (2003) Phosphorylated protein kinases associated with neuronal and glial tau deposits in argyrophylic grain disease. Brain Pathol 13:62–78

    PubMed  Google Scholar 

  23. Ferrer I, Pastor P, Rey MJ, Muñoz E, Puig B, Pastor E, Oliva R, Tolosa E (2003) Tau phosphorylation and kinase activation in familial tauopathy linked to delN296 mutation. Neuropathol Appl Neurobiol 29:23–34

    Article  PubMed  Google Scholar 

  24. Gibb WRG, Luthert PJ, Marsden CD (1989) Corticobasal degeneration. Brain 116:281–302

    Google Scholar 

  25. Goedert M, Spillantini MG, Cairns NJ, Crowtler RA (1992) Tau proteins of Alzheimer paired helical filaments: abnormal phosphorylation of all six brain isoforms. Neuron 8:159–168

    CAS  PubMed  Google Scholar 

  26. Goedert M, Spillantini MG, Davis SW (1998) Filamentous nerve cell inclusions in neurodegenerative diseases. Curr Opin Neurobiol 8:619–632

    CAS  PubMed  Google Scholar 

  27. Graff-Radford NR, Damasio AR, Hyman BT, Hart MN, Tranel D, Damasio H, Van Hoesen GW (1990) Progressive aphasia in a patient with Pick's disease: neuropsychological, radiologic, and anatomic study. Neurology 40:620–626

    CAS  PubMed  Google Scholar 

  28. Green J, Moris JC, Sandson J, McKeel DW, Miller JW (1990) Progressive aphasia: a precursor of global dementia? Neurology 40:423–429

    PubMed  Google Scholar 

  29. Holland AL, McBurney DH, Moossy J, Reinmuth OM (1985) The dissolution of language in Pick's disease with neurofibrillary tangles: a case study. Brain Lang 24:36–58

    CAS  PubMed  Google Scholar 

  30. Hutton M, Lendon CL, Rizzu P, et al (1996) Association of a missense and 5'splice-site mutations in tau with the inherited dementia FTDP-17. Nature 393:702–705

    Article  Google Scholar 

  31. Ikeda K, Akiyama H, Kiondo H, Haga C (1995) A study of dementia with argyrophilic grains. Possible cytoskeletal abnormality in dendrospinal portion of neurons and oligodendroglia. Acta Neuropathol 89:409–414

    Article  CAS  PubMed  Google Scholar 

  32. Ikeda K, Akiyama H, Iritani S, Kase K, Arai T, Niizato K, Kuroki N, Kosaka K (1996) Corticobasal degeneration with primary progressive aphasia and accentuated cortical lesion in superior temporal gyrus: case report and review. Acta Neuropathol 92:534–539

    Article  CAS  PubMed  Google Scholar 

  33. Ingram EM, Spillantini MG (2002) Tau gene mutations: dissecting the pathogenesis of FTDP-17. Trends Mol Med 8:555–562

    Article  PubMed  Google Scholar 

  34. Iseki E, Matsumura T, Marui W, Hino H, Odawara T, Sugiyama N, Suzuki K, Sawada H, Arai T, Kosaka K (2001) Familial frontotemporal dementia and parkinsonism with a novel N296H mutation in exon 10 of the tau gene and a widespread tau accumulation in the glial cells. Acta Neuropathol 102:285–292

    PubMed  Google Scholar 

  35. Jellinger KA (1998) Dementia with grains (argyrophilic grain disease). Brain Pathol 8:377–386

    CAS  PubMed  Google Scholar 

  36. Judkins AR, Forman MS, Uryu K, Hinkle DA, Asbury AK, Lee VMY, Trojanowski JQ (2002) Co-occurrence of Parkinson's disease with progressive supranuclear palsy. Acta Neuropathol 103:526–530

    Article  PubMed  Google Scholar 

  37. Kempler D, Metter EJ, Riege WH, Jackson CA, Benson DF, Hanson WR (1990) Slowly progressive aphasia: three cases with language, memory, CT and PET data. J Neurol Neurosurg Psychiatry 53:987–993

    PubMed  Google Scholar 

  38. Kertesz A, Muñoz DG (2003) Primary progressive aphasia and Pick complex. J Neurol Sci 206:97–107

    Article  PubMed  Google Scholar 

  39. Kertesz A, Hudson L, Mackenzie IR, Muñoz DG (1994) The pathology and nosology of primary progressive aphasia. Neurology 44:2065–2072

    CAS  PubMed  Google Scholar 

  40. Kertesz A, Martínez-Lage P, Davidson W, Muñoz DG (2000) The corticobasal degeneration syndrome overlaps progressive aphasia and frontotemporal dementia. Neurology 55:1368–1375

    PubMed  Google Scholar 

  41. Kinoshita A, Tomimoto H, Tachibana N, Suenaga T, Kawamata T, Kimura T, Akiguchi I, Kimura J (1996) A case of primary progressive aphasia with abnormally ubiquitinated neurites in the cerebral cortex. Acta Neuropathol 92:520–524

    Article  CAS  PubMed  Google Scholar 

  42. Kirschner HS, Tanridag O, Thurman L, Whetsell WO (1987) Progressive aphasia without dementia: two cases with spongiform degeneration. Ann Neurol 22:527–532

    PubMed  Google Scholar 

  43. Knopman DS (1993) Overview of dementia lacking distinctive histology: pathological designation of a progressive dementia. Dementia 4:132–136

    CAS  PubMed  Google Scholar 

  44. Komori T (1999) Tau-positive glial inclusions in progressive supranuclear palsy, corticobasal degeneration and Pick's disease. Brain Pathol 9:663–679

    CAS  PubMed  Google Scholar 

  45. Lang AE (1992) Cortical basal ganglionic degeneration presenting with progressive loss of speech output and orofacial dyspraxia. J Neurol Neurosurg Psychiatry 55:1101

    PubMed  Google Scholar 

  46. Lantos PL (1998) The definition of multiple system atrophy: a review of recent developments. J Neuropathol Exp Neurol 57:1099–1111

    CAS  PubMed  Google Scholar 

  47. Lee VMY, Goedert M, Trojanowski JQ (2001) Neurodegenerative tauopathies. Annu Rev Neurosci 24:1121–1159

    CAS  PubMed  Google Scholar 

  48. Lippa CF, Cohen R, Smith TW, Drachman DA (1991) Primary progressive aphasia with focal neuronal achromasia. Neurology 41:882–886

    PubMed  Google Scholar 

  49. Litvan I, Grimes DA, Lang AE (2000) Phenotypes and prognosis: clinicopathological studies of corticobasal degeneration. Adv Neurol 82:183–196

    PubMed  Google Scholar 

  50. Lowe J, Errington DR, Lennox G, Pike I, Spendlove I, Landon M, Mayer RJ (1992) Ballooned neurons in several neurodegenerative diseases and stroke contain αB-crystallin. Neuropathol Appl Neurobiol 18:341–350

    CAS  PubMed  Google Scholar 

  51. Mandell AM, Alexander MP, Carpenter S (1989) Creutzfeldt-Jakob disease presenting as isolated aphasia. Neurology 39:55–58

    Google Scholar 

  52. Martinez-Lage P, Muñoz DG (1997) Prevalence of diseases associated with argyrophilic grains of Braak. J Neuropathol Exp Neurol 56:157–164

    CAS  PubMed  Google Scholar 

  53. Mesulam MM (1982) Slowly progressive aphasia without generalized dementia. Ann Neurol 11:592–598

    CAS  PubMed  Google Scholar 

  54. Mesulam MM (1987) Primary progressive aphasia—differentiation from Alzheimer's disease. Ann Neurol 22:533–534

    PubMed  Google Scholar 

  55. Mimura M, Oda T, Tsuchiya K, Kato M, Ikeda K, Hori K, Kashima H (2001) Corticobasal degeneration presenting with nonfluent primary progressive aphasia: a clinicopathological study. J Neurol Sci183:19–26

    Google Scholar 

  56. Mochizuki A, Ueda Y, Komatsuzaki Y, Tsuchiya K, Arai T, Shoji S (2003) Progressive supranuclear palsy presenting with primary progressive aphasia. Clinicopathological report of an autopsy. Acta Neuropathol 105:610–614

    PubMed  Google Scholar 

  57. Mori H, Yoshimura M, Tomonaga M, Yamanouchi H (1986) Progressive supranuclear palsy with Lewy bodies. Acta Neuropathol 71:344–346

    CAS  PubMed  Google Scholar 

  58. Neary D, Snowden JS, Mann DM (1993) Familial progressive aphasia: its relationship to other forms of lobar atrophy. J Neurol Neurosurg Psychiatry 56:1122–1125

    PubMed  Google Scholar 

  59. Novak M, Jakes PC, Edwards C, Milstein C, Wischik CM (1991) Difference between the tau protein of Alzheimer paired helical filament core and normal tau revealed by epitope analysis of monoclonal antibodies 423 and 7.51. Proc Natl Acad Sci USA 88:5837–5841

    CAS  PubMed  Google Scholar 

  60. Pogakar S, Williams RS (1984) Alzheimer's disease presenting as slowly progressive aphasia. RI Med J 67:181–185

    Google Scholar 

  61. Rebeiz JJ, Kolodny EH, Richardson EP (1968) Corticodentatonigral degeneration with neuronal achromasia. Arch Neurol 18:20–33

    CAS  PubMed  Google Scholar 

  62. Sakurai Y, Hashida H, Uesugi H, Arima K, Murayama S, Bando M, Iwata M, Momose T, Sakuta M (1996) A clinical profile of corticobasal degeneration presenting as primary progressive aphasia. Eur Neurol 36:134-137

    PubMed  Google Scholar 

  63. Scheltens P, David R, Kampshorst W (1994) Pathologic findings in a case of primary progressive aphasia. Neurology 44:279–282

    PubMed  Google Scholar 

  64. Schneider JA, Watts RL, Gearing M, Brewer RP, Mirra SS (1997) Corticobasal degeneration: neuropathological and clinical heterogeneity. Neurology 48:959–969

    PubMed  Google Scholar 

  65. Schwarz M, De Blesser R, Poeck K, Weis J (1998) A case of primary progressive aphasia. A 14-year follow-up study with neuropathological findings. Brain 121:115–126

    PubMed  Google Scholar 

  66. Shuttleworth EC, Yates AJ, Paltan-Ortiz JD (1985) Creutzfeldt-Jakob disease presenting as progressive aphasia. J Natl Med Assoc 77:649–655

    PubMed  Google Scholar 

  67. Snowden JS, Neary D, Mann DMA, Goulding PJ, Testa HJ (1992) Progressive language disorder due to lobar atrophy. Ann Neurol 31:174–183

    CAS  PubMed  Google Scholar 

  68. Spillantini MG, Goedert M, Crowther RA, Murrell JR, Farlow MR, Ghetti B (1997) Familial multiple system tauopathy with presenile dementia: a disease with abundant neuronal and glial tau filaments. Proc Natl Acad Sci USA 94:4113–4118

    Article  CAS  PubMed  Google Scholar 

  69. Spillantini MG, Crowther RA, Jakes R, Cairns NJ, Lantos PL, Goedert M (1998) Filamentous α-synuclein inclusions link multiple system atrophy with Parkinson's disease and dementia with Lewy bodies. Neurosci Lett 251:205–208

    PubMed  Google Scholar 

  70. Takahashi T, Amano N, Hanihara T, Nagatiomo H, Yagishita S, Itoh Y, Yamaoka K, Toda H, Tanabe T (1996) Corticobasal degeneration: widespread argentophilic threads and glia in addition to neurofibrillary tangles. Similarities of cytoskeletal abnormalities in corticobasal degeneration and progressive supranuclear palsy. J Neurol Sci 138:66–77

    Article  CAS  PubMed  Google Scholar 

  71. Togo T, Cookson N, Dickson DW (2001) Argyrophilic grain disease: neuropathology. Frequency in a dementia brain bank and lack of relationship with apolipoprotein E. Brain Pathol 12:45–52

    Google Scholar 

  72. Togo T, Sahara N, Yen SH, Cookson N, Ishizawa T, Hutton M, De Silva R, Lees A, Dickson DW (2002) Argyrophilic grain disease is a sporadic 4-repeat tauopathy. J Neuropathol Exp Neurol 61:547–556

    PubMed  Google Scholar 

  73. Tolnay M, Probst A (1998) Ballooned neurons expressing α-B-crystallin as a constant feature of the amygdala in argyrophilic grain disease. Neurosci Lett 246:165–168

    CAS  PubMed  Google Scholar 

  74. Tolnay M, Schwietert M, Monsch AU, Stahelin HB, Langui D, Probst A (1997) Argyrophilic grain disease: distribution of grains in patients with and without dementia. Acta Neuropathol 94:353–358

    Article  CAS  PubMed  Google Scholar 

  75. Tolnay M, Sergeant N, Ghestem A, Chalbot S, Vos RAI de, Jansen Steur ENH, Probst A, Delacourte A (2002) Argyrophylic grain disease and Alzheimer's disease are distinguished by their different distribution of tau protein isoforms. Acta Neuropathol 104:425–434

    CAS  PubMed  Google Scholar 

  76. Tu PH, Galvin JE, Baba M, Giasson B, Tomita T, Leigh S, Nakajo S, Iwatsuboto T, Trojanowski JQ, Lee VMY (1998) Glial cytoplasmic inclusions in white matter oligodendrocytes of multiple system atrophy brains contain insoluble α-synuclein. Ann Neurol 44:415–422

    CAS  PubMed  Google Scholar 

  77. Turner RS, Kenyon LC, Trojanowski JQ, Gonatas N, Grossman M (1996) Clinical, neuroimaging, and pathologic features of progressive nonfluent aphasia. Ann Neurol 39:166–173

    PubMed  Google Scholar 

  78. Uchihara T, Mitani K, Mori H, Kondo H, Yamada M, Ikeda K (1994) Abnormal cytoskeletal pathology peculiar to corticobasal degeneration is different from that of Alzheimer's disease or progressive supranuclear palsy. Acta Neuropathol 88:379–383

    Article  CAS  PubMed  Google Scholar 

  79. Wakabayashi K, Oyanagi K, Makifuchi T, Ikuta F, Honna A, Honna Y, Horikawa Y, Tokiguche S (1994) Corticobasal degeneration. Etiopathological significance of the cytoskeletal alterations. Acta Neuropathol 87:545–553

    CAS  PubMed  Google Scholar 

  80. Wakabayashi K, Hayashi S, Kakita A, Yamada M, Toyoshima Y, Yoshimoto M, Takahashi H (1998) Accumulation of α-synuclein/NACP is a cytopathological feature common to Lewy body disease and multiple system atrophy. Acta Neuropathol 96:445–452

    Article  CAS  PubMed  Google Scholar 

  81. Wechsler AF, Verity MA, Rosenschein S, Fried I, Scheibel AB (1982) Pick's disease. A clinical, computed tomographic, and pathological study with Golgi impregnation observations. Arch Neurol 39:287–290

    CAS  PubMed  Google Scholar 

  82. Yamanouchi H, Budka H, Vass K (1986) Unilateral Creutzfeldt-Jakob disease. Neurology 36:1517–1520

    PubMed  Google Scholar 

  83. Yoshimura I, Yoshimura N, Hayashi S, Tasaki H, Kukushima Y (1995) A clinicopathological study of senile dementia presenting as progressive sensory aphasia: a variant of corticobasal degeneration. Jpn J Clin Psychiatry 24:193–200

    Google Scholar 

  84. Zhukareva V, Vogelsberg-Ragaglia V, Van Deerlin VMD, Bruce J, Shuck T, Grossman M, Clark CM, Arnold SE, Masliah E, Galasko D, Trojanowski JQ, Lee VMY (2001) Loss of brain tau defines novel sporadic and familial tauopathies with frontotemporal dementia. Ann Neurol 49:165–175

    CAS  PubMed  Google Scholar 

Download references

Acknowledgements

This work was supported in part by EU contract QLRI-CT-2000-66, CICYT SAF-2001-4681-E and FIS P1020004. We greatly acknowledge Dr. J. Avila his comments and criticism. We wish to thank M. Barrachina for help with the figures and T. Yohannan for editorial assistance.

Author information

Authors and Affiliations

  1. Institut de Neuropatologia, Servei Anatomia Patològica, Hospital Princeps d'Espanya (Bellvitge CSUB), carrer Feixa Llarga sn, 08907, Hospitalet de Llobregat, Spain

    I. Ferrer & B. Puig

  2. Banc de Teixits Neurològics, Universitat de Barcelona/Hospital Clinic, Barcelona, Spain

    I. Ferrer, M. J. Rey, A. Cardozo & M. Ezquerra

  3. Fundaciò ACE, Institut Català de Neurociències Aplicades, Barcelona, Spain

    I. Hernández, M. Boada & A. Llorente

  4. SAP Nou Barris, ICS, CAPII Chafarinas Neurología, Barcelona, Spain

    I. Hernández

  5. Servei de Neurologia, Hospital General Universitari Valle Hebrón, Barcelona, Spain

    M. Boada

Authors
  1. I. Ferrer
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. I. Hernández
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. M. Boada
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. A. Llorente
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. M. J. Rey
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. A. Cardozo
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. M. Ezquerra
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. B. Puig
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to I. Ferrer.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Ferrer, I., Hernández, I., Boada, M. et al. Primary progressive aphasia as the initial manifestation of corticobasal degeneration and unusual tauopathies. Acta Neuropathol 106, 419–435 (2003). https://doi.org/10.1007/s00401-003-0756-4

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00401-003-0756-4

Keywords

Search

Navigation