Acta Neuropathologica

, Volume 108, Issue 5, pp 379–385

Frontotemporal lobar degeneration with motor neuron disease-type inclusions predominates in 76 cases of frontotemporal degeneration

  • Anne M. Lipton
  • Charles L. WhiteIII
  • Eileen H. Bigio
Regular Paper

DOI: 10.1007/s00401-004-0900-9

Cite this article as:
Lipton, A.M., White, C.L. & Bigio, E.H. Acta Neuropathol (2004) 108: 379. doi:10.1007/s00401-004-0900-9


This report presents the largest series of consecutive, neuropathologically confirmed cases of frontotemporal degeneration (FTD). Prior studies have found dementia lacking distinctive histology (DLDH) to be the most common pathology underlying the clinical diagnosis of FTD. In this series of 76 cases, 29 (38%) were found to have frontotemporal lobar degeneration with motor neuron disease-type inclusions (FTLD-MND-type) or FTLD-MND (with ALS), the most common neuropathological classification in our series. Only eight (11%) were classified as Pick’s disease. Several cases originally designated as DLDH could be reclassified as FTLD-MND-type based on current recommendations for classification of FTD.


Frontotemporal dementiaFrontotemporal lobar degenerationMotor neuron diseaseUbiquitin

Copyright information

© Springer-Verlag 2004

Authors and Affiliations

  • Anne M. Lipton
    • 1
  • Charles L. WhiteIII
    • 2
  • Eileen H. Bigio
    • 3
  1. 1.Department of NeurologyUniversity of Texas Southwestern Medical SchoolDallasUSA
  2. 2.Department of PathologyUniversity of Texas Southwestern Medical SchoolDallasUSA
  3. 3.Department of PathologyNorthwestern University Medical SchoolChicagoUSA