Progressive supranuclear palsy syndrome presenting as progressive nonfluent aphasia: a neuropsychological and neuroimaging analysis.
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.
Rohrer
Jonathan D
JD
Dementia Research Centre, Department of Neurodegenerative Disease, UCL Institute of Neurology, University College London, Queen Square, London, WC1N 3BG, UK.
Paviour
Dominic
D
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.
Bronstein
Adolfo M
AM
Department of Clinical Neuroscience, Charing Cross Hospital, Imperial College, London, UK.
O'Sullivan
Sean S
SS
Reta Lila Weston Institute, UCL Institute of Neurology, University College London, Queen Square, London, WC1N 1PJ, UK.
Lees
Andrew
A
Reta Lila Weston Institute, UCL Institute of Neurology, University College London, Queen Square, London, WC1N 1PJ, UK.
Warren
Jason D
JD
Dementia Research Centre, Department of Neurodegenerative Disease, UCL Institute of Neurology, University College London, Queen Square, London, WC1N 3BG, UK.
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