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Behavioural symptoms in progressive supranuclear palsy and frontotemporal dementia
  1. Thomas H Bak1,2,
  2. Linda M Crawford2,
  3. German Berrios3,
  4. John R Hodges4,5
  1. 1Department of Psychology, Edinburgh University, Edinburgh, UK
  2. 2Medical Research Council—Cognition and Brain Sciences Unit, Cambridge, UK
  3. 3University Department of Psychiatry, Addenbrooke's Hospital, Cambridge, UK
  4. 4University Department of Clinical Neuroscience, Addenbrooke's Hospital, Cambridge, UK
  5. 5Prince of Wales Medical Research Institute, Sydney, Australia
  1. Correspondence to Professor John Hodges, Prince of Wales Medical Research Institute, Cnr Barker Street and Easy Street, Randwick, New South Wales, Australia, 2031; j.hodges{at}powmri.edu.au

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Within the spectrum of frontotemporal dementia (FTD), the behavioural variant (bvFTD) presents with prominent changes in personality, most notably disinhibition, loss of empathy, stereotypic behaviour, alteration of food preference and apathy.1 These features have been linked to orbitofrontal and mesial-cingulate pathology.2

In comparison, less is known about the behavioural changes in progressive supranuclear palsy (PSP) which remain in the shadow of the motor, oculo-motor and cognitive symptoms. In one study, PSP patients were found to be apathetic and disinhibited, while the Parkinson's disease patients had a higher frequency of hallucinations, delusions and depression.3 Imaging studies in PSP have demonstrated frontal-lobe atrophy that correlates with the degree of executive dysfunction and behavioural change on the frontal behavioural inventory.4 We compared large cohorts of patients with PSP and bvFTD using a recently validated instrument, the Cambridge Behavioural Inventory.5

A total of 185 patients with PSP participated (100 men, 85 women, mean age: 69.3±6.6 years). Two hundred and ten care-givers of patients with PSP were contacted by the PSP Association; 135 agreed to participate (a response rate of 64%). A further 50 patients were diagnosed in Cambridge. For those ascertained via the PSP Association, we included a symptom checklist which was reviewed to confirm that the clinical profile was …

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