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Acta Neuropathologica

, Volume 122, Issue 1, pp 111–113 | Cite as

A harmonized classification system for FTLD-TDP pathology

  • Ian R. A. Mackenzie
  • Manuela Neumann
  • Atik Baborie
  • Deepak M. Sampathu
  • Daniel Du Plessis
  • Evelyn Jaros
  • Robert H. Perry
  • John Q. Trojanowski
  • David M. A. Mann
  • Virginia M. Y. Lee
Consensus Letter
In 2006, two papers were published, each describing pathological heterogeneity in cases of frontotemporal lobar degeneration (FTLD) with ubiquitin-positive, tau-negative inclusions (FTLD-U) [ 7, 11]. In both studies, large series of cases were evaluated and the investigators felt that they could recognize three distinct histological patterns, based on the morphology and anatomical distribution of ubiquitin immunoreactive neuronal inclusions. The findings of Sampathu et al. were further supported by differential labelling of the pathology, using a panel of novel monoclonal antibodies; whereas, Mackenzie et al. found relatively specific clinicopathological correlations. Most importantly, the pathological features that defined the subtypes in these two studies were almost identical, providing powerful validation of the results. However, because the studies were conducted simultaneously and independently, the numbering of the subtypes, used in the respective papers, did not match (Table  1).

Keywords

Amyotrophic Lateral Sclerosis Dystrophic Neurites Sporadic Amyotrophic Lateral Sclerosis Neuronal Cytoplasmic Inclusion Neuronal Intranuclear Inclusion 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Notes

Acknowledgments

The authors wish to thank their clinical colleagues, in particular Dr. William Seeley (University of California San Francisco), for their support and encouragement in moving this endeavour forward. Studies reviewed here from the Center for Neurodegenerative Disease Research were supported by AG-10124 and AG-17586.

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Copyright information

© Springer-Verlag 2011

Authors and Affiliations

  • Ian R. A. Mackenzie
    • 1
  • Manuela Neumann
    • 2
  • Atik Baborie
    • 3
  • Deepak M. Sampathu
    • 4
  • Daniel Du Plessis
    • 5
  • Evelyn Jaros
    • 6
  • Robert H. Perry
    • 6
  • John Q. Trojanowski
    • 4
  • David M. A. Mann
    • 7
  • Virginia M. Y. Lee
    • 4
  1. 1.Department of PathologyUniversity of British Columbia and Vancouver General HospitalVancouverCanada
  2. 2.Institute of NeuropathologyUniversity Hospital ZurichZurichSwitzerland
  3. 3.Department of NeuropathologyWalton Center for Neurology and NeurosurgeryLiverpoolUK
  4. 4.Department of Pathology and Laboratory MedicineUniversity of Pennsylvania School of MedicinePennsylvaniaUSA
  5. 5.Department of PathologyHope HospitalSalfordUK
  6. 6.Department of NeuropathologyNewcastle General HospitalNewcastle-Upon-TyneUK
  7. 7.Greater Manchester Neurosciences CentreUniversity of ManchesterManchesterUK

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