The Generative Brain

Danish Reference Center for Prion Diseases

The Danish Reference Center for Prion Diseases (DRCPD) is a unit of the Department of Pathology at Copenhagen University Hospital, Rigshospitalet, run within the public Capital Region hospital system rather than as a separate legal body. It runs a biosafety level 3 prion laboratory that handles CJD diagnostics on CSF, blood and brain samples from all of Denmark, and since about 2022 has run second generation (IQ) CSF RT-QuIC in house, validated with the Edinburgh surveillance unit. Its 2023 validation paper reports 100 percent sensitivity (30 microlitre CSF) and 100 percent specificity on 18 probable sCJD and 45 control samples, with full agreement with Edinburgh on 63 samples. National notification and surveillance is coordinated by Statens Serum Institut, with neuropathology at Copenhagen University Hospital. Since 2025 its research has widened to alpha-synuclein seed amplification. Whether it runs 14-3-3, clinical PRNP testing or accepts foreign samples is not public. Information only.

Key facts

  • DRCPD sits in the Department of Pathology, Copenhagen University Hospital Rigshospitalet, 2100 Copenhagen; no separate legal entity, mandate document or Sundhedsstyrelsen designation was found in the public sources opened.
  • It describes itself as a centre for advanced diagnostics and research of human neurodegenerative protein misfolding diseases, covering sporadic, genetic and acquired prion disease and prion-like diseases such as Parkinson and Alzheimer disease.
  • Its biosafety level 3 prion laboratory handles CJD and other prion disease diagnostics on CSF, blood and brain samples from all of Denmark; CSF RT-QuIC was introduced as a test not previously done in Denmark, replacing the sending of samples abroad.
  • RT-QuIC was developed and validated with support from the UK National CJD Research and Surveillance Unit, Edinburgh, whose staff co-author the validation paper.
  • The validation paper is Bsoul, Lund, Burns, Andrews, McKenzie, Green and Areškevičiūtė, International Journal of Molecular Sciences 24(7):6098, published online 23 March 2023.
  • The study tested 63 CSF samples (18 probable sCJD, 45 non-CJD; 48 from Danish neurology departments, 15 from Edinburgh); IQ RT-QuIC gave 100 percent sensitivity with 30 microlitres of CSF, 77 percent with 15 microlitres, and 100 percent specificity, with 100 percent concordance against Edinburgh's earlier generation assay.
  • Assay details: recombinant hamster PrP 90 to 231 substrate, FLUOstar Omega plate reader, about 48 hour runs, positivity threshold 900 RFU.
  • The often quoted figures of above 90 percent sensitivity and 100 percent specificity come from earlier international ring trials cited in the paper, not from DRCPD; DRCPD is not among the six laboratories of the 2020 second generation RT-QuIC ring trial.
  • National surveillance: suspected cases are notified on form 1515 to the SSI Department of Infectious Disease Epidemiology; neuropathology is done at Copenhagen University Hospital; 1997 to 2011 averaged seven sporadic CJD cases a year (range 4 to 12), 54 percent autopsy confirmed, with no hereditary, iatrogenic or variant CJD notified.
  • An analysis of 1997 to 2008 found 83 sporadic CJD patients, a mean incidence of 1.26 per million, and one GSS diagnosis.
  • National guidance (SSI, 2nd edition 2023) states CJD, vCJD and suspicion of them are notifiable, lists CSF 14-3-3 and RT-QuIC among probable CJD criteria, and reports no vCJD in Denmark as of end 2022; Eva Løbner Lund, a DRCPD senior author, sat on the working group for the Danish Society of Pathology.
  • A 40 year reclassification of definite cases referred to the centre found rare sCJD subtypes, a sporadic fatal insomnia case and three novel PRNP mutations, including two large octapeptide repeat insertions, in work with the Bologna prion group.
  • Named staff (professional only): Eva Løbner Lund, MD, PhD, consultant pathologist and senior author, and Aušrinė Areškevičiūtė, listed as prion laboratory manager in March 2023 and senior author on 2025 papers.
  • From 2025 the group publishes alpha-synuclein seed amplification work on CSF, skin, olfactory mucosa and urine (skin 94 percent sensitivity, 98 percent specificity in Lewy body dementia) and a standardised control fluid protocol, with Danish dementia and Italian (Verona, Bologna) collaborators.
  • No public page found lists CSF 14-3-3 or tau, clinical PRNP genotyping, sample forms, prices, or acceptance of foreign samples; the Danish clinician handbook names RT-QuIC and 14-3-3 but not where to send samples.

Timeline

  1. 1997Start of the Danish CJD surveillance period later analysed by SSI and Eurosurveillance.
  2. 2012SSI EPI-NEWS reports seven sporadic CJD cases a year on average for 1997 to 2011.
  3. 2019Danish prion cohort reclassification paper with three novel PRNP mutations published.
  4. 2022Department announces in-house CSF RT-QuIC at the prion laboratory (date inferred from post id, about June 2022).
  5. 2023SSI publishes the 2nd edition national prion infection hygiene guideline.
  6. 2023PhD position at DRCPD advertised by Region Hovedstaden.
  7. 2023RT-QuIC validation paper published in International Journal of Molecular Sciences.
  8. 2025Multi-tissue alpha-synuclein seed amplification paper published.
  9. 2025Microglia profiling study across CJD and Alzheimer disease published.
  10. 2026Further alpha-synuclein seeding papers in J Neurol and Mov Disord Clin Pract list DRCPD authors.
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