title: Progressive supranuclear palsy syndrome presenting as progressive nonfluent aphasia: a neuropsychological and neuroimaging analysis.
abstract: 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.
authors.affiliation: Dementia Research Centre, Department of Neurodegenerative Disease, UCL Institute of Neurology, University College London, Queen Square, London, WC1N 3BG, UK.
authors.forename: Jonathan D
authors.initials: JD
authors.lastname: Rohrer
authors.affiliation: Dementia Research Centre, Department of Neurodegenerative Disease, UCL Institute of Neurology, University College London, Queen Square, London, WC1N 3BG, UK.|Reta Lila Weston Institute, UCL Institute of Neurology, University College London, Queen Square, London, WC1N 1PJ, UK.
authors.forename: Dominic
authors.initials: D
authors.lastname: Paviour
authors.affiliation: Department of Clinical Neuroscience, Charing Cross Hospital, Imperial College, London, UK.
authors.forename: Adolfo M
authors.initials: AM
authors.lastname: Bronstein
authors.affiliation: Reta Lila Weston Institute, UCL Institute of Neurology, University College London, Queen Square, London, WC1N 1PJ, UK.
authors.forename: Sean S
authors.initials: SS
authors.lastname: O'Sullivan
authors.affiliation: Reta Lila Weston Institute, UCL Institute of Neurology, University College London, Queen Square, London, WC1N 1PJ, UK.
authors.forename: Andrew
authors.initials: A
authors.lastname: Lees
authors.affiliation: Dementia Research Centre, Department of Neurodegenerative Disease, UCL Institute of Neurology, University College London, Queen Square, London, WC1N 3BG, UK.
authors.forename: Jason D
authors.initials: JD
authors.lastname: Warren
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D001284:Atrophy; D001921:Brain / Q000473:pathology*; D003071:Cognition; D005116:Extrapyramidal Tracts / Q000503:physiopathology; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008568:Memory; D008636:Mesencephalon / Q000473:pathology; D009483:Neuropsychological Tests; D009801:Oculomotor Muscles / Q000503:physiopathology; D017397:Prefrontal Cortex / Q000473:pathology; D057178:Primary Progressive Nonfluent Aphasia / Q000209:etiology / Q000503:physiopathology / Q000523:psychology*; D011446:Prospective Studies; D013494:Supranuclear Palsy, Progressive / Q000150:complications / Q000209:etiology / Q000473:pathology* / Q000503:physiopathology*; D013577:Syndrome; D014705:Verbal Behavior