National Prion Disease Registry, Erasmus MC, Rotterdam (Netherlands)

Key facts

• The registry is described as part of Erasmus University Medical Centre, Rotterdam, and aims to ascertain all definite or probable CJD patients in the Netherlands; staff often classify patients by international criteria before death.
• The national CJD surveillance programme was set up in 1997.
• Treating physicians, mainly neurologists, were required by law to notify clinically suspected patients to the reference centre within two days.
• Autopsies on notified patients, where permission was given, are done at the Dutch Surveillance Centre for Prion Diseases at UMC Utrecht, so the registry (Rotterdam) and the autopsy centre (Utrecht) are two linked units of one programme.
• For 1998 to 2009 the registry logged 451 clinically suspected patients (295 notified prospectively, 156 reported after death); 318 (70.5%) were autopsied; 214 were definite or probable; 162 were definite.
• Definite cases by type in 1998 to 2009: sporadic CJD 144, iatrogenic 5, inherited CJD 3, GSS 4, PrP cerebral amyloid angiopathy 1, FFI 1, variant CJD 3, VPSPr 1.
• Definite cases per year ranged from 6 to 22 and mortality from 0.63 to 1.53 per million inhabitants per year, using a population of about 16 million with data from Erasmus epidemiology.
• PRNP mutations were found in 9 of 161 tested patients (5.6%); codon 129 VV was under-represented (7.0%) and MV over-represented (22.4%) in sporadic CJD compared with other Western cohorts.
• Among suspects, post-mortem examination found another diagnosis in 156 patients, most often Alzheimer disease (21.2%) or vascular dementia (19.9%).
• In the 1993 to 2002 EUROCJD comparison, the Dutch register had begun prospective collection in 1993, centralised all information in Rotterdam, and counted 142 cases (136 sporadic, 3 iatrogenic, 1 genetic, 2 insertion mutation) and no variant CJD, about 3% of 4,441 pooled patients.
• A 2006 RIVM protocol, read only as a search excerpt, states that CJD and vCJD became notifiable on 1 September 2002 (group B) and that specialists report classic CJD within 24 hours to the national registry at the Erasmus MC epidemiology department.
• The Utrecht centre reported 280 autopsies on probable or possible CJD in 1997 to 2007 with prion disease in 146 (52%), and 384 autopsies in 1998 to 2011 with definite CJD in 203 (search excerpt).

Timeline

1. 1993The Dutch national register starts prospective data collection, with data centralised in Rotterdam, as part of the EUROCJD countries.
2. 1997National CJD surveillance programme established.
3. 2002CJD and vCJD become notifiable diseases in the Netherlands (group B), per the 2006 RIVM protocol.
4. 2009Nederlands Tijdschrift voor Geneeskunde article lists Landelijk Registratie Prionziekten, Erasmus MC, as an author affiliation (search excerpt).
5. 2011Doctoral thesis on the 162 definite cases of 1998 to 2009 awarded at Utrecht University.
6. 2012Registry-based national report published in PLoS ONE covering 1998 to 2009.