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Dementia lacking distinctive histology (DLDH) revisited
Ian R A Mackenzie1, Jing Shi, Catherine L Shaw
1Department of Pathology, Vancouver General Hospital, Vancouver, BC, Canada.
Improved ubiquitin immunostaining reclassified most "dementia lacking distinctive histology" (DLDH) cases as frontotemporal lobar degeneration with ubiquitin-positive inclusions (FTLD-U). This suggests DLDH is rare, with pathology often missed due to less sensitive staining methods.
Area of Science:
- Neuropathology
- Neurodegenerative Diseases
Background:
- Immunohistochemistry is crucial for classifying frontotemporal lobar degeneration (FTLD).
- Some FTLD cases lack distinctive histology (DLDH), posing diagnostic challenges.
Purpose of the Study:
- To re-evaluate "dementia lacking distinctive histology" (DLDH) cases using an improved ubiquitin immunostaining method.
- To determine the prevalence of ubiquitin pathology in previously unclassified FTLD cases.
Main Methods:
- Automated immunostaining for ubiquitin protein (UBQ) was performed on 41 FTLD patient samples.
- Samples were compared based on previous staining (Manchester) and a newer method (Vancouver).
Main Results:
- The newer staining method significantly increased the detection of ubiquitin-immunoreactive (UBQ-ir) pathology.
- 13 of 16 previously DLDH cases were reclassified as definite or probable FTLD with ubiquitin-positive inclusions (FTLD-U).
- Two remaining DLDH cases had clinical uncertainties, suggesting DLDH is rare.
Conclusions:
- The "dementia lacking distinctive histology" (DLDH) diagnosis is likely rare.
- Insensitivity of older UBQ staining methods may have led to misclassification of FTLD-U cases as DLDH.
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