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Progressive Supranuclear Palsy and Corticobasal Degeneration
David G Coughlin1, Dennis W Dickson2, Keith A Josephs3
1UC San Diego, Department of Neurosciences, La Jolla, CA, USA.
Advances in Experimental Medicine and Biology
|January 12, 2021
Summary
Progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) are 4R tau protein disorders. Recognizing diverse clinical signs is key for accurate diagnosis and future therapeutic trials.
Area of Science:
- Neuroscience
- Neuropathology
- Neurodegenerative Diseases
Background:
- Progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) are distinct neurodegenerative tauopathies.
- Both conditions feature neuronal and glial lesions primarily composed of 4R tau protein.
- Pathological overlap exists, but differences in lesion distribution, morphology, and comorbid pathology distinguish PSP and CBD.
Purpose of the Study:
- To highlight the phenotypic diversity in PSP and CBD.
- To emphasize the impact of phenotypic diversity on antemortem diagnostic accuracy.
- To discuss current and future management strategies for PSP and CBD.
Main Methods:
- Review of neuropathological features of PSP and CBD.
- Analysis of clinical manifestations and diagnostic challenges.
- Evaluation of current treatment standards and future therapeutic directions.
Main Results:
- PSP and CBD exhibit a wide spectrum of clinical presentations affecting movement and cognition.
- Diagnostic accuracy is high for typical PSP (Richardson syndrome) but low for characteristic CBD (corticobasal syndrome).
- Molecular and imaging biomarkers are needed to improve antemortem diagnosis.
Conclusions:
- Recognizing phenotypic variability is crucial for improving antemortem diagnosis of PSP and CBD.
- Multidisciplinary symptomatic and supportive care is the current standard.
- Experimental therapeutic trials are essential for future disease modification.
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