Increased tau burden in the cortices of progressive supranuclear palsy presenting with corticobasal syndrome

Yoshio Tsuboi, Keith Anthony Josephs, Bradley F Boeve, Irene Litvan, Richard John Caselli, John Nathaniel Caviness, Ryan J. Uitti, Allen D. Bott, Dennis W Dickson

Research output: Contribution to journalArticle

83 Citations (Scopus)

Abstract

The objective of this study is to better define the pathological characteristics of pathologically proven progressive supranuclear palsy (PSP) presenting with the corticobasal syndrome (CBS). PSP is characterized by early falls, vertical supranuclear ophthalmoplegia, and axial rigidity, whereas asymmetric limb features, including rigidity, bradykinesia, apraxia, alien limb phenomena, and cortical sensory loss are characteristic of CBS. We investigated clinicopathological characteristics of 5 cases of PSP that presented with CBS (CBS-PSP). Comprehensive pathological analysis was undertaken to determine the presence of concomitant pathological processes as well as quantitative tau burden in cortical regions of CBS-PSP, compared with 8 typical PSP cases (Typ-PSP). The clinical features in the CBS-PSP cases included asymmetrical features, apraxia, alien limb phenomena, and progressive aphasia. All cases had Parkinsonism, and vertical supranuclear ophthalmoplegia was noted in all but 1 case of CBS-PSP. Secondary neuropathological diagnoses included argyrophilic grain disease (AGD) in 1 of the 8 cases of Typ-PSP, whereas Alzheimer's disease (AD), Lewy body disease, AGD, and vascular disease was found in 3 cases of CBS-PSP. Image analysis of cortical tau burden performed in 8 Typ-PSP and 3 CBS-PSP cases revealed a significant increased tau burden in mid-frontal and inferior-parietal cortices in the CBS-PSP cases. This study demonstrates that when PSP presents as CBS, it is most likely due to either a concurrent cortical pathology from a secondary process such as AD or from the primary pathology of PSP extending into cortical areas that are primarily and commonly affected in CBD.

Original languageEnglish (US)
Pages (from-to)982-988
Number of pages7
JournalMovement Disorders
Volume20
Issue number8
DOIs
StatePublished - Aug 2005

Fingerprint

Progressive Supranuclear Palsy
Apraxias
Ophthalmoplegia
Extremities
Alzheimer Disease
Pathology
Lewy Body Disease
Hypokinesia
Parietal Lobe
Aphasia
Parkinsonian Disorders
Pathologic Processes
Vascular Diseases

Keywords

  • Alzheimer's disease
  • Asymmetrical features
  • Corticobasal syndrome
  • Progressive supranuclear palsy
  • Tau burden

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)

Cite this

Increased tau burden in the cortices of progressive supranuclear palsy presenting with corticobasal syndrome. / Tsuboi, Yoshio; Josephs, Keith Anthony; Boeve, Bradley F; Litvan, Irene; Caselli, Richard John; Caviness, John Nathaniel; Uitti, Ryan J.; Bott, Allen D.; Dickson, Dennis W.

In: Movement Disorders, Vol. 20, No. 8, 08.2005, p. 982-988.

Research output: Contribution to journalArticle

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