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

Emma Schofield, John Hodges, Thomas Bak, John Xuereb, Glenda Halliday

Research output: Contribution to journalArticlepeer-review

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
Original languageEnglish
Pages (from-to)482-490
Number of pages9
JournalJournal of Neurology
Volume259
Issue number3
Early online date12 Aug 2011
DOIs
Publication statusPublished - Mar 2012

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