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The evolution and pathology of frontotemporal dementia
Andrew Kertesz1, Paul McMonagle, Mervin Blair
1Department of Cognitive Neurology, St Joseph's Hospital, London, Ontario N6A 4V2, Canada. Andrew.Kertesz@sjhc.london.on.ca
Brain : a Journal of Neurology
|July 22, 2005
Summary
This study examined 60 frontotemporal dementia (FTD) patients, linking clinical syndromes to autopsy-confirmed pathologies. Findings suggest clinical presentation predicts histology, but overlapping symptoms support a unified FTD classification.
Area of Science:
- Neurology
- Pathology
- Neuroscience
Background:
- Frontotemporal dementia (FTD) encompasses diverse clinical syndromes and underlying pathologies.
- Accurate clinicopathologic correlation is crucial for understanding FTD heterogeneity.
Purpose of the Study:
- To correlate clinical presentations of FTD syndromes with post-mortem histopathological findings.
- To investigate the relationship between clinical onset and specific FTD pathologies.
- To evaluate the utility of a unified classification for FTD.
Main Methods:
- Prospective clinic-based cohort study of 60 FTD patients followed to autopsy.
- Clinical diagnosis included behavioural variant FTD (FTD-bv), primary progressive aphasia (PPA), corticobasal degeneration syndrome (CBDS), and progressive supranuclear palsy (PSP).
- Histopathological analysis identified various pathologies including motor neurone disease type inclusion (MNDI), corticobasal degeneration (CBD), Pick's disease, and Alzheimer's disease.
Main Results:
- Motor neurone disease type inclusion (MNDI) and dementia lacking distinctive histology (DLDH) were most common tau-negative pathologies, often presenting as FTD-bv.
- Tau-positive pathologies (CBD, PSP, Pick's disease) were frequently associated with PPA onset or CBDS/PSP.
- Earlier age of onset was noted in tau-negative cases, with no significant differences in duration or gender distribution across variants.
Conclusions:
- Clinical onset partially predicts FTD histology, but significant overlap exists.
- The convergence of syndromes during disease progression supports classifying diverse clinical and pathological entities under a single FTD umbrella.
- This clinicopathologic study reinforces the complex nature of FTD and the need for integrated diagnostic approaches.
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