Regular Paper

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. LiptonAffiliated withDepartment of Neurology, University of Texas Southwestern Medical School Email author 
  • , Charles L. WhiteIIIAffiliated withDepartment of Pathology, University of Texas Southwestern Medical School
  • , Eileen H. BigioAffiliated withDepartment of Pathology, Northwestern University Medical School

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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 dementia Frontotemporal lobar degeneration Motor neuron disease Ubiquitin