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Article type: Research Article
Authors: Parjane, Nataliaa | Cho, Sunghyeb | Ash, Sharona | Cousins, Katheryn A.Q.a | Shellikeri, Sanjanaa | Liberman, Markb | Shaw, Leslie M.c | Irwin, David J.a; d | Grossman, Murraya | Nevler, Naomia; *
Affiliations: [a] Penn Frontotemporal Degeneration Center, Department of Neurology, University of Pennsylvania, Philadelphia, PA, USA | [b] Linguistic Data Consortium, University of Pennsylvania, Philadelphia, PA, USA | [c] Department of Pathology and Laboratory Medicine, Hospital of the University of Pennsylvania, Philadelphia, PA, USA | [d] Penn Digital Neuropathology Laboratory, Department of Neurology, University of Pennsylvania, Philadelphia, PA, USA
Correspondence: [*] Correspondence to: Naomi Nevler, MD, Department of Neurology, 3 West Gates, Hospital of the University of Pennsylvania, 3400 Spruce Street, Philadelphia, PA 19104, USA. Tel.: +1 2675422568; E-mail: naomine@pennmedicine.upenn.edu.
Abstract: Background:Progressive supranuclear palsy syndrome (PSPS) and corticobasal syndrome (CBS) as well as non-fluent/agrammatic primary progressive aphasia (naPPA) are often associated with misfolded 4-repeat tau pathology, but the diversity of the associated speech features is poorly understood. Objective:Investigate the full range of acoustic and lexical properties of speech to test the hypothesis that PSPS-CBS show a subset of speech impairments found in naPPA. Methods:Acoustic and lexical measures, extracted from natural, digitized semi-structured speech samples using novel, automated methods, were compared in PSPS-CBS (n = 87), naPPA (n = 25), and healthy controls (HC, n = 41). We related these measures to grammatical performance and speech fluency, core features of naPPA, to neuropsychological measures of naming, executive, memory and visuoconstructional functioning, and to cerebrospinal fluid (CSF) phosphorylated tau (pTau) levels in patients with available biofluid analytes. Results:Both naPPA and PSPS-CBS speech produced shorter speech segments, longer pauses, higher pause rates, reduced fundamental frequency (f0) pitch ranges, and slower speech rate compared to HC. naPPA speech was distinct from PSPS-CBS with shorter speech segments, more frequent pauses, slower speech rate, reduced verb production, and higher partial word production. In both groups, acoustic duration measures generally correlated with speech fluency, measured as words per minute, and grammatical performance. Speech measures did not correlate with standard neuropsychological measures. CSF pTau levels correlated with f0 range in PSPS-CBS and naPPA. Conclusion:Lexical and acoustic speech features of PSPS-CBS overlaps those of naPPA and are related to CSF pTau levels.
Keywords: Corticobasal syndrome, language, non-fluent primary progressive aphasia, progressive supranuclear palsy, speech, tauopathy
DOI: 10.3233/JAD-201132
Journal: Journal of Alzheimer's Disease, vol. 82, no. 1, pp. 33-45, 2021
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