The Generative Brain

Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan

The Carlo Besta institute is a public research hospital in Milan for neurology and neurosurgery, founded in 1918, classed as an IRCCS (research hospital) in 1981 and run as a foundation since 2006. In prion disease it does three things in the opened sources. It runs a biosafety level 3 laboratory that tests brain biomarkers in people with suspected prion disease with seed amplification methods (RT-QuIC and PMCA). It has a dementia and degenerative disease unit that lists prion diseases in its clinical scope and offers genetic counselling for dementias. And its researchers have co-led prion trials and studies: the senior author of the Italian and French phase 2 doxycycline trial in CJD, co-authors on the Doxiff FFI prevention study with Mario Negri, the 2015 FFI mouse model, the 2014 urine test for variant CJD and the ProFFIle biomarker consortium. Its staff have also been regular speakers at AIEnP family meetings from 2010 to 2019.

Key facts

  • The legal name is Fondazione IRCCS Istituto Neurologico Carlo Besta, with its seat at Via Celoria 11, Milan, and tax code 01668320151.
  • The institute was founded in 1918, became an IRCCS in 1981 and a foundation in 2006, with the Ministry of Health, Lombardy Region and the City of Milan as founders.
  • The laboratory medicine service runs a BSL3 laboratory for brain biomarkers in suspected prion disease using seed amplification assays, RT-QuIC and PMCA; Fabio Moda is listed among its senior health staff.
  • The Neurologia 8 unit covers dementias, degenerative brain diseases and prion diseases, with inpatient care, a cognitive disorders and dementia outpatient centre and a genetic counselling clinic for dementias.
  • Fabrizio Tagliavini of Carlo Besta was senior and corresponding author of the phase 2 doxycycline trial in CJD, which enrolled 121 patients in Italy (2007 to 2010) and France (2009 to 2012) and was stopped for futility; six other Besta staff were co-authors.
  • Besta researchers are credited, with Mario Negri, for helping conceive and coordinate the Telethon funded Doxiff doxycycline prevention study in FFI carriers, and Tagliavini co-authored its 2015 design paper.
  • Besta neurologists are co-authors of the 2022 paper on how scientists and an at-risk family designed the FFI prevention trial together.
  • The 2015 FFI transgenic mouse model was built at Mario Negri with Besta neuropathology co-authors; the mice reproduced the sleep disorder without detectable prion infectivity.
  • A Besta led team with Houston colleagues found prions in urine of 13 of 14 people with variant CJD and in none of 224 controls (NEJM, August 2014).
  • A 2023 follow-up with Houston and Cleveland found urine prions in 36 percent of 81 people with sporadic CJD and in no controls, so urine testing is far less sensitive for the sporadic form.
  • Besta, through Fabio Moda, is one of six partners in the JPND funded ProFFIle consortium on early biomarkers in FFI carriers, coordinated by Mario Negri.
  • Fabio Moda of Besta sits on the AFIFF scientific committee.
  • Besta staff spoke at AIEnP family meetings in 2010 (Tagliavini on GSS), 2017 (with Besta thanked as collaborator), 2018 and 2019 (Moda on mechanisms, diagnosis and therapy prospects).
  • In 2025 and 2026 Besta prion staff published book chapters on PMCA and RT-QuIC in peripheral tissues and a CJD case report warning that early nerve involvement can be mistaken for an inflammatory neuropathy.

Timeline

  1. 1918Institute founded in Milan.
  2. 1981Recognised as an IRCCS research hospital.
  3. 2006Turned into a foundation of public participation.
  4. 2007Italian arm of the doxycycline CJD trial starts recruiting.
  5. 2010Tagliavini speaks on GSS at the second AIEnP family day in Milan.
  6. 2014Doxycycline CJD trial published in Lancet Neurology with a Besta senior author.
  7. 2014NEJM paper on prions in urine in variant CJD, Besta first author.
  8. 2015Design paper of the FFI doxycycline prevention study with Tagliavini as co-author.
  9. 2015FFI transgenic mouse model published in PLoS Pathogens with Besta co-authors.
  10. 2017AIEnP Bergamo family meeting with Besta speakers.
  11. 2018Centenary reported, with Tagliavini as scientific director.
  12. 2021ProFFIle recommended for JPND funding with Besta as partner.
  13. 2021Giuseppe Lauria Pinter named scientific director.
  14. 2023Sporadic CJD urine PMCA paper with a Besta co-author.
  15. 2024ProFFIle poster presented by a Besta researcher at the JPND symposium.
  16. 2026CJD case report from the Besta laboratory medicine unit.
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