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

Acta Neuropathol. 2004 Nov;108(5):379-85. doi: 10.1007/s00401-004-0900-9. Epub 2004 Sep 4.


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.

Publication types

  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Dementia / classification*
  • Dementia / metabolism
  • Dementia / pathology*
  • Humans
  • Immunohistochemistry
  • Inclusion Bodies / metabolism
  • Inclusion Bodies / pathology*
  • Motor Neuron Disease / classification
  • Motor Neuron Disease / metabolism
  • Motor Neuron Disease / pathology*
  • Nerve Degeneration / metabolism
  • Nerve Degeneration / pathology*