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Extrapyramidal syndromes in frontotemporal degeneration.
Andrew Kertesz1, Paul McMonagle, Sarah Jesso
1Department of Neurology, University of Western Ontario, London, ON, Canada. andrew.kertesz@sjhc.london.on.ca
Journal of Molecular Neuroscience : MN
|September 3, 2011
Summary
Extrapyramidal symptoms are common in frontotemporal dementia (FTD), including Corticobasal Degeneration (CBD) and Progressive Supranuclear Palsis (PSP). These conditions often present with cognitive and behavioral changes, including aphasia, and are linked to tau pathology.
Area of Science:
- Neurology
- Neuroscience
- Pathology
Background:
- Extrapyramidal (EP) symptoms have been described in frontotemporal dementia (FTD) for 80 years.
- Corticobasal Degeneration (CBD) pathology is a common cause of primary progressive aphasia (PPA).
- Progressive Supranuclear Palsis (PSP) and CBD share considerable overlap, challenging previous classifications of dementia.
Purpose of the Study:
- To investigate the spectrum of extrapyramidal symptoms (EPS) in FTD, specifically within Corticobasal Degeneration (CBD) and Progressive Supranuclear Palsis (PSP).
- To analyze the relationship between motor and cognitive onset presentations of CBD/PSP and the development of language and behavioral deficits.
- To explore the underlying pathology, particularly tau-positive findings, in patients with overlapping CBD/PSP, PPA, and behavioral variant FTD (bvFTD).
Main Methods:
- Review of a cohort of FTD patients with EPS, categorizing them by initial presentation (motor vs. cognitive).
- Assessment of language and behavioral changes using standardized measures, including the Frontal Behavioral Inventory (FBI).
- Analysis of neuropathological findings, including tau pathology, in autopsied cases.
Main Results:
- EPS were identified as a first syndrome in 22 FTD patients and occurred after cognitive onset in 48 others.
- Aphasia developed in most CBD/PSP patients, regardless of initial presentation.
- Cognitive onset cases showed worse language scores, and significant behavioral changes like apathy and disinhibition were documented.
- Tau pathology was present in 15 out of 18 autopsied cases.
Conclusions:
- CBD and PSP, along with PPA and bvFTD, represent a spectrum of related tau-positive neurodegenerative diseases.
- The presentation of CBD/PSP can vary, with motor or cognitive symptoms appearing first, but aphasia is a common feature.
- Longitudinal studies are crucial to understand the full evolution of language and behavioral deficits in these overlapping conditions.
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