Dataset Information

Four-Repeat Tauopathies: Current Management and Future Treatments.

ABSTRACT: Four-repeat tauopathies are a neurodegenerative disease characterized by brain parenchymal accumulation of a specific isoform of the protein tau, which gives rise to a wide breadth of clinical syndromes encompassing diverse symptomatology, with the most common syndromes being progressive supranuclear palsy-Richardson's and corticobasal syndrome. Despite the lack of effective disease-modifying therapies, targeted treatment of symptoms can improve quality of life for patients with 4-repeat tauopathies. However, managing these symptoms can be a daunting task, even for those familiar with the diseases, as they span motor, sensory, cognitive, affective, autonomic, and behavioral domains. This review describes current approaches to symptomatic management of common clinical symptoms in 4-repeat tauopathies with a focus on practical patient management, including pharmacologic and nonpharmacologic strategies, and concludes with a discussion of the history and future of disease-modifying therapeutics and clinical trials in this population.

SUBMITTER: VandeVrede L

PROVIDER: S-EPMC7851277 | biostudies-literature | 2020 Oct

REPOSITORIES: biostudies-literature

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Publications

Four-Repeat Tauopathies: Current Management and Future Treatments.

VandeVrede Lawren L Ljubenkov Peter A PA Rojas Julio C JC Welch Ariane E AE Boxer Adam L AL

Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics 20201001 4

Four-repeat tauopathies are a neurodegenerative disease characterized by brain parenchymal accumulation of a specific isoform of the protein tau, which gives rise to a wide breadth of clinical syndromes encompassing diverse symptomatology, with the most common syndromes being progressive supranuclear palsy-Richardson's and corticobasal syndrome. Despite the lack of effective disease-modifying therapies, targeted treatment of symptoms can improve quality of life for patients with 4-repeat tauopat ...[more]

PMID: 32676851

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Project description:PurposeThe aim of this study was to use acoustic and kinematic speech measures to characterize type of motor speech impairment-apraxia of speech (AOS) versus dysarthria-in individuals with four-repeat tauopathy (4RT)-associated syndromes, including nonfluent variant primary progressive aphasia (nfvPPA), primary progressive AOS (PPAOS), corticobasal syndrome (CBS), and progressive supranuclear palsy syndrome (PSPs).MethodTwenty patient participants were recruited and stratified into two groups: (a) a motor-speech-impaired group of individuals with nfvPPA, PPAOS, CBS, or PSPs and suspected 4RT pathology ("MSI+") and (b) a non-motor-speech-impaired group of individuals with logopenic variant primary progressive aphasia ("MSI-"). Ten healthy, age-matched controls also participated in the study. Participants completed a battery of speech tasks, and 15 acoustic and kinematic speech measures were derived. Quantitative speech measures were grouped into feature categories ("AOS features," "dysarthria features," "shared features"). In addition to quantitative speech measures, two certified speech-language pathologists made independent, blinded auditory-perceptual ratings of motor speech impairment. A principal component analysis (PCA) was conducted to investigate the relative contributions of quantitative features.ResultsQuantitative speech measures were generally concordant with independent clinician ratings of motor speech impairment severity. Hypothesis-driven groupings of quantitative measures differentiated predominantly apraxic from predominantly dysarthric presentations within the MSI+ group. PCA results provided additional evidence for differential profiles of motor speech impairment in the MSI+ group; heterogeneity across individuals is explained in large part by varying levels of overall severity-captured by the shared feature variable group-and degree of apraxia severity, as measured by the AOS feature variable group.ConclusionsQuantitative features reveal heterogeneity of MSI in the 4RT group in terms of both overall severity and subtype of MSI. Results suggest the potential for acoustic and kinematic speech assessment methods to inform characterization of motor speech impairment in 4RT-associated syndromes.Supplemental materialhttps://doi.org/10.23641/asha.21401778.

Dataset Information

Four-Repeat Tauopathies: Current Management and Future Treatments.

Publications

Four-Repeat Tauopathies: Current Management and Future Treatments.

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