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Article type: Research Article
Authors: Burrell, James R.a; b; e; * | Ballard, Kirrie J.d; e | Halliday, Glenda M.b; c; d; e | Hodges, John R.b; e
Affiliations: [a] Concord General Hospital, Sydney, Australia | [b] Brain and Mind Centre, University of Sydney Medical School, Sydney, Australia | [c] Faculty of Medicine, University of New South Wales, Sydney, Australia | [d] Neuroscience Research Australia, Sydney, Australia | [e] The University of Sydney, Sydney, Australia
Correspondence: [*] Correspondence to: James R. Burrell, PhD, FRACP, Neurosciences 5West, Concord General Hospital, Sydney NSW 2139, Australia. Tel.: +61 2 9767 5000; Fax: +61 2 8244 1950; E-mail: james.burrell@sydney.edu.au.
Abstract: Background:Adynamic speech is characteristic of progressive supranuclear palsy (PSP), but higher language deficits have been reported inconsistently, in the context of clinical and pathological overlaps with progressive non-fluent aphasia (PNFA). Objective:The present study tested two hypotheses: 1) PSP and PNFA display impaired single word repetition, object naming, semantic knowledge, and syntactic comprehension; and 2) PSP have reduced speed on timed cognitive tasks. Methods:Structured clinical and neuropsychological assessments of language were performed on patients with clinically defined PSP and PNFA. Language was tested using the Sydney Language Battery (SYDBAT) and the Test of Reception of Grammar (TROG). Results:In total, 144 participants were studied (PSP 22, PNFA 29, and Control 93). PSP patients had prominent eye movement abnormalities, parkinsonism, and falls. All 4 PSP patients who underwent postmortem examination had 4-Repeat tauopathy, with PSP pathology in 3. The frequency and severity of impairment on the SYDBAT (naming, word comprehension, semantic association), and TROG (syntactic comprehension) did not differ between PSP and PNFA, but PSP were significantly slower on timed non-language cognitive tests. Conclusion:Tested formally, aphasia may be seen in PSP, with a severity similar to that seen in PNFA.
Keywords: Clinicopathological correlation, primary progressive aphasia, progressive non-fluent aphasia, progressive supranuclear palsy
DOI: 10.3233/JAD-170743
Journal: Journal of Alzheimer's Disease, vol. 61, no. 2, pp. 705-715, 2018
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