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ISCIII Centro Nacional de Epidemiologia, Spanish national registry of human TSE (RNEETH) logo

ISCIII Centro Nacional de Epidemiologia, Spanish national registry of human TSE (RNEETH)

The Centro Nacional de Epidemiologia (CNE, National Epidemiology Centre) of the Instituto de Salud Carlos III in Madrid coordinates national surveillance of human transmissible spongiform encephalopathies (prion diseases) in Spain and holds the national registry, known as RNEETH. The registry began collecting cases in 1995 under an EU concerted action, includes cases with onset from 1993 found retrospectively, and has taken part in the EUROCJD network since 1996. It is a public health registry, not a clinic or a laboratory: doctors in each of the 17 autonomous communities report suspected cases to their regional registry within 48 hours, and the regions send the cases on to the CNE. The registry staff publish the national figures and papers, most of them with a second affiliation to CIBERNED, which is a separate research network. Its own most recent abstract warns that notification, testing and autopsy have fallen sharply since 2017.

Key facts

  • The CNE at ISCIII coordinates national surveillance of human TSE and posts a surveillance and notification protocol and results reports for 2019 and 2021 and an annual report for 2016 on its public page.
  • The national registry is located at and run by the CNE; it was created in 1995 and records cases from 1993 reported by the autonomous communities.
  • The state Orden of 21 February 2001 set up the national surveillance network for human TSE, with population based national and regional registries classifying cases as possible or suspected, probable or confirmed on European criteria; doctors and laboratories report within 48 hours of suspicion and regional registries send new cases to the national registry monthly.
  • Surveillance started as an EU concerted action in 1995, Spain has been part of EUROCJD from 1996, EU network procedures were added from 2002 and the system was linked to ECDC networks covering 21 countries from 2005.
  • Each region has a public health and a clinical coordinator; nine regional neuropathology labs and a national reference centre in Madrid do autopsies, and a few EU validated labs at ISCIII and elsewhere run genetic and 14-3-3 tests.
  • From 1993 to 2018 the registry received 2,137 referrals, including 1,600 sporadic CJD, 168 genetic TSE (89 genetic CJD, 74 fatal familial insomnia, 5 GSS), 8 dura mater related and 5 variant CJD cases.
  • Age adjusted sporadic CJD incidence was 1.25 per million person years in 1998 to 2018, ranging from 0.50 to 1.87 by region; genetic cases cluster in the Basque Country at about four times the national rate, and the most frequent mutations were D178N (74) and E200K (65).
  • The five variant CJD cases in Spain were confirmed post mortem between 2005 and 2008, which the registry authors say makes Spain the third most affected country.
  • By March 2025 the registry held 2,618 notified cases, 81.3 percent of them CJD; RT-QuIC, part of the diagnostic criteria since 2017, was used in only 31 percent of 2024 cases, and autopsy fell from over 60 percent of cases in 2004 to near zero in the last decade.
  • Registry authors have published national survival analysis of 1,530 cases (2022), the EUROSURGYCJD studies on surgery and CJD risk, and an eleven country study of diagnostic patterns from 1993 to 2002 with surveillance centres in Italy, Germany, Australia, Canada, France and Austria.
  • Spanish ISCIII authors, listed as the Spanish National Reference Center for CJD, contributed cases to the 2019 international study of age at onset in 1,094 PRNP mutation carriers led from the Broad Institute and the MRC Prion Unit.
  • The CNE public page does not name EUROCJD, RNEETH or any staff in its body text, and gives no update date.

Timeline

  1. 1993Earliest onset year of cases included in the registry, identified retrospectively.
  2. 1995Registry created at the CNE and prospective collection begins under an EU concerted action.
  3. 1996Government order formally creates the CJD registry; Spain takes part in EUROCJD from this year.
  4. 2001State order sets up the national surveillance network for human TSE and makes notification compulsory.
  5. 2001Galicia issues its regional order linking the Galician registry to the national one at the CNE.
  6. 2002EU network procedures added to Spanish surveillance.
  7. 2005Surveillance linked with ECDC networks covering 21 countries.
  8. 2005to 2008 Five variant CJD cases confirmed in Spain.
  9. 2006Eleven country study of human TSE diagnostic patterns led by the registry published.
  10. 2017Diagnostic criteria revised, with RT-QuIC added to the criteria (search excerpt for the month).
  11. 2021National overview of surveillance from 1993 to 2018 published in Prion.
  12. 2025Registry authors present the national variant CJD review at the Spanish Epidemiology Society meeting.
  13. 2025Registry abstract at the Spanish Society of Neurology meeting warns of falling notification and diagnosis, 2,618 cases to March 2025.
  14. 2026Variant CJD surveillance paper for 1993 to 2021 published.
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