Progressive supranuclear palsy presenting with dynamic aphasia


T. Esmonde
Bok Engelsk 1996
Utgitt
1996
Omfang
Side 403- 410
Opplysninger
Background-Progressive supranuclear palsy (PSP) is an akinetic-rigidsyndrome of unknown aetiology which usually presents with acombination of unsteadiness, bradykinesia, and disordered eyemovement. Speech often becomes dysarthric but language disorders arenot well recognised.Methods-Three patients with PSP (pathologically confirmed in two) arereported in which the presenting symptoms were those of difficultywith language output.Results-Neuropsychological testing showed considerable impairment ona range of single word tasks which require active initiation andsearch strategies (letter and category fluency, sentence completion),and on tests of narrative language production. By contrast, namingfrom pictures and from verbal descriptions, and word and sentencecomprehension were largely intact. The degree of semantic memoryimpairment was also slight.Conclusions-Relatively selective involvement of cognitive processescritical for planning and initiating language output may occur insome patients with PSP. This presentation resembles the phenomenon of''verbal adynamia'' or ''dynamic aphasia'' seen in patients withfrontal lobe damage. Although definite cortical changes were presentat postmortem examination, it is likely that the neuropsychologicaldeficits reflect functional frontal deafferentation secondary tointerruption of frontostriatal feedback loops.
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