Clinical Spectrum of Tauopathies
- PMID: 35911892
- PMCID: PMC9329580
- DOI: 10.3389/fneur.2022.944806
Clinical Spectrum of Tauopathies
Erratum in
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Corrigendum: Clinical spectrum of tauopathies.Front Neurol. 2022 Sep 14;13:1015572. doi: 10.3389/fneur.2022.1015572. eCollection 2022. Front Neurol. 2022. PMID: 36188395 Free PMC article.
Abstract
Tauopathies are both clinical and pathological heterogeneous disorders characterized by neuronal and/or glial accumulation of misfolded tau protein. It is now well understood that every pathologic tauopathy may present with various clinical phenotypes based on the primary site of involvement and the spread and distribution of the pathology in the nervous system making clinicopathological correlation more and more challenging. The clinical spectrum of tauopathies includes syndromes with a strong association with an underlying primary tauopathy, including Richardson syndrome (RS), corticobasal syndrome (CBS), non-fluent agrammatic primary progressive aphasia (nfaPPA)/apraxia of speech, pure akinesia with gait freezing (PAGF), and behavioral variant frontotemporal dementia (bvFTD), or weak association with an underlying primary tauopathy, including Parkinsonian syndrome, late-onset cerebellar ataxia, primary lateral sclerosis, semantic variant PPA (svPPA), and amnestic syndrome. Here, we discuss clinical syndromes associated with various primary tauopathies and their distinguishing clinical features and new biomarkers becoming available to improve in vivo diagnosis. Although the typical phenotypic clinical presentations lead us to suspect specific underlying pathologies, it is still challenging to differentiate pathology accurately based on clinical findings due to large phenotypic overlaps. Larger pathology-confirmed studies to validate the use of different biomarkers and prospective longitudinal cohorts evaluating detailed clinical, biofluid, and imaging protocols in subjects presenting with heterogenous phenotypes reflecting a variety of suspected underlying pathologies are fundamental for a better understanding of the clinicopathological correlations.
Keywords: clinical; corticobasal; frontotemporal dementia; movement; neurodegenerative; primary progressive aphasia; progressive supranuclear palsy; tauopathy.
Copyright © 2022 Olfati, Shoeibi and Litvan.
Conflict of interest statement
The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. IL is the Field Editor of Frontiers in Neurology however was not involved in the review of this manuscript.
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