{
  "abstract": "Background The PiPPIN study defines epidemiological and phenotypic variation of syndromes associated with frontotemporal lobar degeneration (FTLD): progressive supranuclear palsy, corticobasal syndrome, behavioural-variant frontotemporal dementia and primary progressive aphasiaMethodology PiPPIN is a prospective, observational study in Cambridgeshire and Norfolk (population 1.8million 2021) with three 2-year recruitment phases: 2013-14, 2017-18 and 2021-22. Multisource referral identified people with FTLD-associated diagnoses. Contemporary international consensus clinical diagnostic criteria were applied.Results 520 participants (M:F 257:252, unknown=11; mean age 71y[ 9.4].) were recruited with FTLD-related syndromes. The diagnoses at recruitment were: PSP n=187 (36%), bvFTD n=129 (25%), PPA n=111 (21%), CBS n=93 (18%). 72% of patients had died by the census date 30/6/2024.Direct age-adjusted prevalence peaked in the seventh decade at 33/100,000. The incidence (per 100,000 population/year) and midpoint prevalence (per 100,000) for each phase were (95% CI):Pippin 1: Incidence 2.1 (1.7-2.7), Prevalence 8.8 (7.5-10.3)Pippin 2: Incidence 1.9 (1.4-2.4), Prevalence 8.9 (7.6-10.4)Pippin 3: Incidence 1.9 (1.5-2.5), Prevalence 7.4 (6.2-8.7)Median survival from diagnosis was (years [IQR]): PSP 2.8 [2.6]; CBS 3.9 [3.6]; bvFTD 4.1 [4.1]; PPA 6.0 [5.3]Ongoing Cox-regression modelling is investigating blood, imaging and clinical predictors of survival.Conclusion The incidence and prevalence of FTLD-associated disorders is stable over ten years. Survival remains poor. Determining survival predictors will help to develop therapeutic strategies.katherinestockton@nhs.net",
  "authors": [
    {
      "affiliations": [
        "Department of Clinical Neurosciences, University of Cambridge, Cambridge, UK",
        "Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK"
      ],
      "name": "Stockton Katherine"
    },
    {
      "affiliations": [
        "Department of Clinical Neurosciences, University of Cambridge, Cambridge, UK",
        "Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK"
      ],
      "name": "Murley Alexander G"
    },
    {
      "affiliations": [
        "Department of Clinical Neurosciences, University of Cambridge, Cambridge, UK",
        "Norfolk and Norwich University Hospitals NHS Foundation Trust, Norwich, UK"
      ],
      "name": "Coyle-Gilchrist Ian"
    },
    {
      "affiliations": [
        "Department of Clinical Neurosciences, University of Cambridge, Cambridge, UK",
        "MRC Cognition and Brain Sciences Unit, University of Cambridge, Cambridge, UK"
      ],
      "name": "Rouse Matthew A"
    },
    {
      "affiliations": [
        "MRC Cognition and Brain Sciences Unit, University of Cambridge, Cambridge, UK"
      ],
      "name": "Naessens Michelle"
    },
    {
      "affiliations": [
        "Department of Clinical Neurosciences, University of Cambridge, Cambridge, UK"
      ],
      "name": "Dreyer Anna J"
    },
    {
      "affiliations": [
        "Cambridge Public Health, University of Cambridge, Cambridge, UK"
      ],
      "name": "Brayne Carol"
    },
    {
      "affiliations": [
        "MRC Cognition and Brain Sciences Unit, University of Cambridge, Cambridge, UK"
      ],
      "name": "Patterson Karalyn"
    },
    {
      "affiliations": [
        "Department of Clinical Neurosciences, University of Cambridge, Cambridge, UK",
        "Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK"
      ],
      "name": "Rittman Timothy"
    },
    {
      "affiliations": [
        "Department of Clinical Neurosciences, University of Cambridge, Cambridge, UK",
        "Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK",
        "MRC Cognition and Brain Sciences Unit, University of Cambridge, Cambridge, UK"
      ],
      "name": "Rowe James B"
    }
  ],
  "title": "234 The incidence, prevalence and survival of frontotemporal lobar degeneration syndromes: 10 years of ‘PiPPIN’",
  "uid": "3eb763e4-2306-51ef-8cd7-9b8720563f4e"
}