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Acta Neuropathologica

, Volume 117, Issue 1, pp 15–18 | Cite as

Nomenclature for neuropathologic subtypes of frontotemporal lobar degeneration: consensus recommendations

  • Ian R. A. MackenzieEmail author
  • Manuela Neumann
  • Eileen H. Bigio
  • Nigel J. Cairns
  • Irina Alafuzoff
  • Jillian Kril
  • Gabor G. Kovacs
  • Bernardino Ghetti
  • Glenda Halliday
  • Ida E. Holm
  • Paul G. Ince
  • Wouter Kamphorst
  • Tamas Revesz
  • Annemieke J. M. Rozemuller
  • Samir Kumar-Singh
  • Haruhiko Akiyama
  • Atik Baborie
  • Salvatore Spina
  • Dennis W. Dickson
  • John Q. Trojanowski
  • David M. A. Mann
Editorial

Introduction

The neuropathology associated with the clinical entities frontotemporal dementia (FTD, behavioral variant FTD), progressive non-fluent aphasia (PNFA) and semantic dementia (SD), is heterogeneous with the common feature being a relatively selective degeneration of the frontal and temporal lobes (frontotemporal lobar degeneration, FTLD). As in other neurodegenerative conditions, most pathological subtypes of FTLD are characterized by specific kinds of intracellular protein inclusions. In the past few decades, the biochemical composition of many of these inclusion bodies has been determined. There is a growing trend to classify FTLD based on the presumed molecular defect, in the belief that this most closely reflects the underlying pathogenic process and because many of the eponymous and descriptively named syndromes of the past are now known to have imperfect clinicopathological correlation.

A comprehensive consensus paper on the neuropathologic diagnostic and nosologic...

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Copyright information

© Springer-Verlag 2008

Authors and Affiliations

  • Ian R. A. Mackenzie
    • 1
    • 20
    Email author
  • Manuela Neumann
    • 2
  • Eileen H. Bigio
    • 3
  • Nigel J. Cairns
    • 4
  • Irina Alafuzoff
    • 5
  • Jillian Kril
    • 6
  • Gabor G. Kovacs
    • 7
  • Bernardino Ghetti
    • 8
  • Glenda Halliday
    • 9
  • Ida E. Holm
    • 10
  • Paul G. Ince
    • 11
  • Wouter Kamphorst
    • 12
  • Tamas Revesz
    • 13
  • Annemieke J. M. Rozemuller
    • 12
  • Samir Kumar-Singh
    • 14
  • Haruhiko Akiyama
    • 15
  • Atik Baborie
    • 16
  • Salvatore Spina
    • 8
  • Dennis W. Dickson
    • 17
  • John Q. Trojanowski
    • 18
  • David M. A. Mann
    • 19
  1. 1.Department of Pathology and Laboratory Medicine, Vancouver General HospitalUniversity of British ColumbiaVancouverCanada
  2. 2.Institute of NeuropathologyUniversity Hospital of ZurichZurichSwitzerland
  3. 3.Department of PathologyNorthwestern University Feinberg School of MedicineChicagoUSA
  4. 4.Department of Pathology and ImmunologyWashington University School of MedicineSt LouisUSA
  5. 5.Section of NeuropathologyKuopio UniversityKuopioFinland
  6. 6.Department of PathologyThe University of SydneySydneyAustralia
  7. 7.Institute of NeurologyMedical University of ViennaViennaAustria
  8. 8.Department of Pathology and Laboratory MedicineIndiana University School of MedicineIndianapolisUSA
  9. 9.Prince of Wales Medical Research InstituteUniversity of New South WalesSydneyAustralia
  10. 10.Department of Pathology, Aalborg HospitalAarhus University HospitalAalborgDenmark
  11. 11.Neuropathology GroupUniversity of Sheffield Medical SchoolSheffieldUK
  12. 12.Department of PathologyVrije University Medical CentreAmsterdamThe Netherlands
  13. 13.Department of Molecular NeuroscienceQueen SquareLondonUK
  14. 14.Department of Molecular GeneticsUniversity of AntwerpAntwerpBelgium
  15. 15.Tokyo Institute of PsychiatryTokyoJapan
  16. 16.NeuropathologyThe Walton Centre for Neurology and NeurosurgeryLiverpoolUK
  17. 17.Neuropathology LaboratoryMayo Clinic College of MedicineJacksonvilleUSA
  18. 18.Department of Pathology and Laboratory MedicineUniversity of Pennsylvania School of MedicinePhiladelphiaUSA
  19. 19.Clinical Neuroscience Research Group, Greater Manchester Neurosciences CentreUniversity of ManchesterSalfordUK
  20. 20.Department of PathologyVancouver General HospitalVancouverCanada

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