Frontotemporal lobar degeneration with motor neuron disease-type inclusions predominates in 76 cases of frontotemporal degeneration
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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.
KeywordsFrontotemporal dementia Frontotemporal lobar degeneration Motor neuron disease Ubiquitin
We thank the UT Southwestern ADC Clinical Core for the evaluation of some of the patients. We are grateful for the excellent assistance of the UT Southwestern Immunohistochemistry Laboratory, under the direction of Christa Hladik, HT (ASCP) QIHC. We gratefully acknowledge Dr. Peter Davies for his generous gift of the PHF-1 antibody. This work was supported in part by NIH grant AG12300.
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