Making the diagnosis of frontotemporal lobar degeneration

The 3 most common pathologic diagnoses in neurodegenerative disorders are Alzheimer disease (AD), dementia with Lewy bodies (DLB or LBD), and frontotemporal lobar degeneration (FTLD). Almost 20 years ago, Mirra [...]
Context.--Autopsy evaluation of the brain of a patient with frontotemporal dementia (FTD) can be daunting to the general pathologist. At some point in their training, most pathologists learn about Pick disease, and can recognize Pick bodies, the morphologic hallmark of Pick disease. Pick disease is a type of frontotemporal lobar degeneration (FTLD), the general category of pathologic process underlying most cases of FTD. The 2 major categories of pathologic FTLD are tauopathies (FTLD-tau) and ubiquitinopathies (FTLD-U). Pick disease is one of the FTLD-tau subtypes and is termed FTLD-tau (PiD). Objective.--To 'demystify' FTLDs, and to demonstrate that subtypes of FTLD-tau and FTLD-U can be easily determined by following a logical, stepwise, histochemical, and immunohistochemical investigation of the FTD autopsy brain. Data Sources.--Previously published peer-reviewed articles. Conclusions.--The hope is that this article will be a useful reference for the general pathologist faced with performing a brain autopsy on a decedent with frontotemporal dementia. (Arch Pathol Lab Med. 2013;137:314-325; doi: 10.5858/arpa.2012-0075-RA)