The Generative Brain

Austrian Reference Centre for Human Prion Diseases (OERPE), Vienna

OERPE is Austria's national reference centre for human prion disease. It sits in the Division of Neuropathology and Neurochemistry of the University Clinic of Neurology at the Medical University of Vienna (AKH Vienna) and works on behalf of the federal health ministry, which lists it as the national reference centre for Creutzfeldt-Jakob disease. Its core work since the 1990s has been to record and check suspected and confirmed cases, report to the ministry, and advise clinicians and pathologists. Austria has run active, autopsy-controlled prion surveillance since 1996. The Vienna group has been a steady partner in EUROCJD and European neuropathology work, and led the 2005 EUROCJD paper on genetic prion disease. Information only, not medical advice.

Key facts

  • The ministry's list of national reference centres (state 26 March 2026) names the Austrian reference centre for the recording and documentation of human prion diseases, at the Division of Neuropathology and Neurochemistry, University Clinic of Neurology, MedUni Wien, AKH, as the reference centre for CJD.
  • On that list the division head is Romana Hoeftberger and the named contact persons are Ellen Gelpi, Anika Simonovska-Serra and the division head; these are public professional roles.
  • The MedUni Wien neurology site says the centre is run on behalf of the federal ministry of social affairs, health, care and consumer protection; the division's prion work uses histology, immunohistochemistry, electron microscopy, genetics, ELISA and RT-QuIC.
  • Deaths from confirmed spongiform encephalopathy have been notifiable in Austria since April 1996 (regulation BGBl. Nr. 156/1996); suspected cases alone were not notifiable under that rule.
  • In the 1990s the centre recorded confirmed deaths and clinically suspected cases and sent the ministry a report every three months.
  • By 2001 the centre was checking suspected cases on referral, had sent autopsy and brain removal safety guidance to Austrian pathology institutes, and was open to questions from care staff and pathologists.
  • A 2008 autopsy-controlled study from the centre analysed more than 900 referrals since 1996 and found mean yearly mortality of 1.39 per million from 1996 to mid 2006, highest in Vienna at 2.37 per million, with about 85 percent of definite cases sporadic.
  • The same study found the clinical surveillance criteria had 82.7 percent sensitivity and 80.0 percent specificity for probable CJD against autopsy.
  • The 2005 EUROCJD paper on genetic prion disease, with OERPE Vienna as first author affiliation, pooled 1993 to 2002 European data and found genetic forms make up 10 to 15 percent of cases, with mutation frequencies that differ by country.
  • Austrian authors from Vienna took part in the 2006 eleven country study of diagnostic patterns from 1993 to 2002 led from the Spanish national epidemiology centre.
  • A Vienna neuropathologist with Toronto ties sat on the international group behind the 2021 sporadic CJD biomarker and diagnostic criteria that admitted RT-QuIC.
  • Recent Vienna papers include the 2021 study of oligodendroglial PrP in the M2C type, the 2022 description of the VM1 subtype with Barcelona, Bologna, ISS Rome and Toronto, and the April 2026 report of two new VPSPr cases with CIC bioGUNE and Bellvitge authors.
  • A 2025 JAMA Neurology study by centre and Vienna neurology authors linked 129 autopsy-confirmed sporadic CJD cases to Austrian insurance prescription data and found higher odds of SSRI prescription in the year before onset (odds ratio 2.86), rising from about three years before.
  • A 2026 paper by centre authors re-read 51 EEGs from 16 sporadic CJD patients with the 2021 ACNS criteria and found periodic patterns mainly in cortical histotypes.
  • An affiliation search found 46 prion papers listing the Vienna reference centre from 1997 to 2026.
  • The division hosted a Vienna symposium on prion diseases for the international CJD Awareness Day on 6 November 2023 (seen only as a search excerpt).

Timeline

  1. 1996Active Austrian prion surveillance starts.
  2. 1996Deaths from confirmed spongiform encephalopathy become notifiable in Austria; OERPE reports to the ministry quarterly.
  3. 2001Ministry answer to parliament describes OERPE case checks and autopsy safety guidance.
  4. 2005EUROCJD genetic prion disease paper led from OERPE Vienna published online.
  5. 2006Eleven country diagnostic pattern paper with Austrian co-authors published online.
  6. 2008Autopsy-controlled study of CJD in Austria, 1996 to 2006, published online.
  7. 2022VM1 sporadic CJD subtype paper with Vienna and OERPE authors published online.
  8. 2023CJD Awareness Day symposium in Vienna hosted by the reference centre division.
  9. 2025Prodromal mood study published.
  10. 2025Two new VPSPr cases with Vienna reference centre authors published online.
  11. 2026Ministry list of national reference centres confirms OERPE as the CJD reference centre.
  12. 2026EEG study with ACNS 2021 criteria published online.
Sources for this page