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Research paper
Midcingulate involvement in progressive supranuclear palsy and tau positive frontotemporal dementia
  1. Wang Zheng Chiu1,
  2. Janne M Papma1,
  3. Inge de Koning2,
  4. Laura Donker Kaat1,
  5. Harro Seelaar1,
  6. Ambroos E M Reijs3,
  7. Roelf Valkema3,
  8. Djo Hasan4,
  9. Agnita J W Boon1,
  10. John Cornelis van Swieten1
  1. 1Department of Neurology, Erasmus University Medical Centre, Rotterdam, The Netherlands
  2. 2Department of Neuropsychology, Erasmus University Medical Centre, Rotterdam, The Netherlands
  3. 3Department of Nuclear Medicine, Erasmus University Medical Centre, Rotterdam, The Netherlands
  4. 4Department of Regional Intensive Care, Maasstad Hospital, Rotterdam, The Netherlands
  1. Correspondence to Professor J C van Swieten, Department of Neurology, Erasmus University Medical Centre Rotterdam, Room Hs 611, 's-Gravendijkwal 230, 3015 CE Rotterdam, The Netherlands; j.c.vanswieten{at}


Background Progressive supranuclear palsy (PSP) patients often exhibit cognitive decline and behavioural changes during the disease course. In a subset, these symptoms may be the presenting manifestation and can be similar to those in frontotemporal dementia (FTD). However, correlation studies between quantitative imaging measures and detailed neuropsychological assessment are scarce. The aim of this study was to investigate the functional role of affected brain regions in cognition in PSP compared with controls and subsequently examine these regions in FTD patients with known tau pathology (FTD tau).

Methods 21 PSP patients, 27 healthy controls and 11 FTD tau patients were enrolled. All participants underwent neuropsychological testing and technetium-99m-hexamethyl-propylenamine-oxime single photon emission CT. Regression slope analyses were performed in statistical parametric mapping to find significant associations between neuropsychological test results and brain perfusion.

Results PSP patients showed hypoperfusion in the midcingulate cortex (MCC) of which the posterior part correlated with Stroop III and Weigl. In FTD tau patients, MCC involvement was located more anterior and correlated with Stroop III and Wisconsin Card Sorting Test concepts. The degree of hypoperfusion in the anterior cortex and MCC in the disorders differed in the subgenual anterior cingulate cortex only.

Conclusions The posterior part of the MCC is prominently involved in the neurodegenerative process of PSP, and the severity of its hypoperfusion correlated with the extent of executive dysfunction. In FTD tau, this cognitive domain was associated with anterior MCC involvement. The degree of hypoperfusion in these regions did not differ between PSP and FTD tau. These observations provide insight into the role of the cingulate cortex in cognitive dysfunction in these neurodegenerative disorders and warrant further investigations.

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  • Funding This study received financial support from Fonds Nuts-Ohra, grant No 0801-69.

  • Competing interests None.

  • Ethics approval Ethics approval was provided by the Medical Ethics Committee of the Erasmus University Medical Centre Rotterdam.

  • Provenance and peer review Not commissioned; externally peer reviewed.