Abstract

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.

Keywords

Frontotemporal lobar degenerationFrontotemporal dementiaDegeneration (medical)PathologyDementiaDiseaseMotor neurone diseaseDegenerative diseaseMedicineAmyotrophic lateral sclerosis

MeSH Terms

DementiaHumansImmunohistochemistryInclusion BodiesMotor Neuron DiseaseNerve Degeneration

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Publication Info

Year
2004
Type
article
Volume
108
Issue
5
Pages
379-385
Citations
190
Access
Closed

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Citation Metrics

190
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12
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151
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Cite This

Anne M. Lipton, Charles L. White, Eileen H. Bigio (2004). Frontotemporal lobar degeneration with motor neuron disease-type inclusions predominates in 76 cases of frontotemporal degeneration. Acta Neuropathologica , 108 (5) , 379-385. https://doi.org/10.1007/s00401-004-0900-9

Identifiers

DOI
10.1007/s00401-004-0900-9
PMID
15351890

Data Quality

Data completeness: 86%