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Making the diagnosis of frontotemporal lobar degeneration
1Department of Pathology, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USA. e-bigio@northwestern.edu
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.
Insights
Frontotemporal dementia (FTD) autopsy evaluation is simplified by distinguishing FTLD-tau and FTLD-U subtypes. This guide aids pathologists in diagnosing frontotemporal lobar degeneration (FTLD) subtypes using histochemical and immunohistochemical methods.
Area of Science:
- Neuropathology
- Neurodegenerative Diseases
- Histopathology
Background:
- Frontotemporal dementia (FTD) autopsy evaluation presents challenges for general pathologists.
- Pick disease, a subtype of frontotemporal lobar degeneration-tau (FTLD-tau), is characterized by Pick bodies.
- FTLD encompasses tauopathies (FTLD-tau) and ubiquitinopathies (FTLD-U).
Purpose of the Study:
- To simplify the classification of FTLD subtypes.
- To provide a stepwise approach for differentiating FTLD-tau and FTLD-U in autopsy brains.
- To serve as a practical reference for pathologists performing FTD autopsies.
Main Methods:
- Review of previously published peer-reviewed articles.
- Utilizing histochemical and immunohistochemical techniques.
- Logical, stepwise diagnostic investigation of FTD autopsy brain tissue.
Main Results:
- A clear distinction between FTLD-tau and FTLD-U subtypes is achievable.
- Pick disease (FTLD-tau (PiD)) can be identified through characteristic morphologic features.
- The proposed methods facilitate accurate subtyping of FTLD.
Conclusions:
- This approach demystifies FTLD evaluation for general pathologists.
- The study provides a practical guide for FTD autopsy brain analysis.
- Accurate subtyping of FTLD is crucial for understanding FTD pathology.
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