Publication:
The relationship between clinical and pathological variables in Richardson's syndrome

dc.contributor.author Schofield, Emma C en_US
dc.contributor.author Hodges, John R en_US
dc.contributor.author Bak, Thomas H en_US
dc.contributor.author Xuereb, John H en_US
dc.contributor.author Halliday, Glenda en_US
dc.date.accessioned 2021-11-25T12:30:32Z
dc.date.available 2021-11-25T12:30:32Z
dc.date.issued 2012 en_US
dc.description.abstract In order to determine the relationship between regional neuropathology and severity of clinical features in Richardson’s syndrome (PSP-RS), the following hypotheses were tested; 1) executive dysfunction relates to prefrontal pathology; 2) language difficulties to pathology in Broca’s area and/or the perirhinal cortex and 3) visuospatial impairment to pathology in the supramarginal region. A prospectively studied case series of brain donors at a specialist clinic in Addenbrooke’s Hospital Cambridge, UK, were examined. All those fulfilling postmortem criteria for PSP-RS and their last cognitive assessment within 24 months of death (N=11/25) were included. The degree of regional neuronal loss and neuronal tau deposition across a number of cortical brain regions was performed and compared to 10 age and sex matched controls from the Sydney Brain Bank. Stepwise multiple linear regressions were used to determine the neuropathological correlates to cognitive scores and revealed the following. Executive dysfunction, as indexed by letter fluency, related to the degree of tau deposition in the superior frontal gyrus and supramarginal cortices (p<0.020), language deficits related to neuron loss in the perirhinal gyrus (p<0.001) and tau deposition in Broca’s area (p=0.020), while visuospatial dysfunction and global cognitive impairment related to tau deposition in the supramarginal gyrus (p<0.007). The severity of cognitive deficits relate to regional cortical tau deposition in PSP-RS, although language impairment related to neuronal loss in the perirhinal region. Global cognitive dysfunction related most to the severity of tau deposition in the supramarginal gyrus warranting further research on the role of this brain region in PSP-RS. en_US
dc.identifier.issn 0340-5354 en_US
dc.identifier.uri http://hdl.handle.net/1959.4/53779
dc.language English
dc.language.iso EN en_US
dc.rights CC BY-NC-ND 3.0 en_US
dc.rights.uri https://creativecommons.org/licenses/by-nc-nd/3.0/au/ en_US
dc.source Legacy MARC en_US
dc.subject.other neuropathology en_US
dc.subject.other Richardson’s syndrome en_US
dc.subject.other progressive supranuclear palsy en_US
dc.subject.other cognition en_US
dc.subject.other tau en_US
dc.title The relationship between clinical and pathological variables in Richardson's syndrome en_US
dc.type Journal Article en
dcterms.accessRights open access
dspace.entity.type Publication en_US
unsw.accessRights.uri https://purl.org/coar/access_right/c_abf2
unsw.description.publisherStatement The final publication is available at Springer via http://dx.doi.org/10.1007/s00415-011-6205-8 en_US
unsw.identifier.doiPublisher http://dx.doi.org/10.1007/s00415-011-6205-8 en_US
unsw.relation.faculty Medicine & Health
unsw.relation.ispartofissue 3 en_US
unsw.relation.ispartofjournal Journal of Neurology en_US
unsw.relation.ispartofpagefrompageto 482-490 en_US
unsw.relation.ispartofvolume 259 en_US
unsw.relation.originalPublicationAffiliation Schofield, Emma C, Neuroscience Research Australia, Faculty of Medicine, UNSW en_US
unsw.relation.originalPublicationAffiliation Hodges, John R, Neuroscience Research Australia, Faculty of Medicine, UNSW en_US
unsw.relation.originalPublicationAffiliation Bak, Thomas H, Human Cognitive Neuroscience, University of Edinburgh, UK & Centre for Clinical Brain Sciences, University of Edinburgh, UK en_US
unsw.relation.originalPublicationAffiliation Xuereb, John H, Department of Pathology, University of Cambridge, U.K. en_US
unsw.relation.originalPublicationAffiliation Halliday, Glenda, Neuroscience Research Australia, Faculty of Medicine, UNSW en_US
unsw.relation.school Neuroscience Research Australia *
unsw.subject.fieldofresearchcode 110903 Central Nervous System en_US
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