Journal
MOVEMENT DISORDERS
Volume 25, Issue 2, Pages 179-188Publisher
WILEY
DOI: 10.1002/mds.22946
Keywords
primary progressive aphasia; progressive supranuclear palsy; apraxia of speech
Categories
Funding
- Department of Health's NIHR Biomedical Research Centres
- Medical Research Council UK
- Brain Exit Scholarship
- Reta Lila Weston Trust
- Wellcome Trust
- PSP Association
- Weston Trust-The Reta Lila
- Medical Research Council [MC_U950770497, G0601846, G0600183, G0801306] Funding Source: researchfish
- National Institute for Health Research [NF-SI-0508-10123] Funding Source: researchfish
- MRC [G0801306, G0601846, G0600183, MC_U950770497] Funding Source: UKRI
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There is currently considerable interest in the clinical spectrum of progressive nonfluent aphasia (PNFA) and progressive supranuclear palsy (PSP) and the intersection of these two entities. Here, we undertook a detailed prospective clinical, neuropsychological, and neuroimaging analysis of 14 consecutive patients presenting with PNFA to identify cases meeting clinical criteria for PSP. These patients had further detailed assessment of extrapyramidal and oculomotor functions. All patients had high-resolution MR brain volumetry and a cortical thickness analysis was undertaken on the brain images. Four patients presenting with PNFA subsequently developed features of a PSP syndrome, including a typical oculomotor palsy. The neuropsychological profile in these cases was similar to other patients with PNFA, however, with more marked reduction in propositional speech, fewer speech errors, less marked impairment of literacy skills but more severe associated deficits of episodic memory and praxis. These PSP-PNFA cases had less prominent midbrain atrophy but more marked prefrontal atrophy than a comparison group of five patients with pathologically confirmed PSP without PNFA and more prominent midbrain atrophy but less marked perisylvian atrophy than other PNFA cases. In summary, although the PSP-PNFA syndrome overlaps with PNFA without PSP, certain neuropsychological and neuroanatomical differences may help predict the development of a PSP syndrome. (C) 2010 Movement Disorder Society
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