Iatrogenic Creutzfeldt–Jakob disease at the millennium

Abstract

The causes and geographic distribution of 267 cases of iatrogenic Creutzfeldt-Jakob disease (CJD) are here updated at the millennium. Small numbers of still-occurring cases result from disease onsets after longer and longer incubation periods following infection by cadaveric human growth hormone or dura mater grafts manufactured and distributed before the mid-1980s. The proportion of recipients acquiring CJD from growth hormone varies from 0.3 to 4.4% in different countries, and acquisition from dura mater varies between 0.02 and 0.05% in Japan (where most cases occurred). Incubation periods can extend up to 30 years, and cerebellar onsets predominate in both hormone and graft recipients (in whom the site of graft placement had no effect on the clinical presentation). Homozygosity at codon 129 of the PRNP gene is over-represented in both forms of disease; it has no effect on the incubation period of graft recipients, but may promote shorter incubation periods in hormone cases. Knowledge about potential high-risk sources of contamination gained during the last quarter century, and the implementation of methods to circumvent them, should minimize the potential for iatrogenic contributions to the current spectrum of CJD.

MRI and clinical syndrome in dura materrelated Creutzfeldt-Jakob disease

“…However, in DM-CJD cases, no such influence was found [60]. Compared to other reports, the incubation periods observed in our patients were significantly prolonged (mean 18 years vs. 8 years), which may be best explained by the fact that German CJD surveillance was only first established in 1993 [11].…”

Risikofaktoren der sporadischen Creutzfeldt-Jakob-Krankheit

“…Eine Übertragung des Krankheits-verursachenden Agens von Mensch zu Mensch ist bei Kuru durch den Verzehr von infiziertem Hirngewebe bekannt, darüber hinaus ist sie allerdings nur auf iatrogenem Weg beschrieben (Brown P et al 2000, Poser und Zerr 2002. Die iCJK wurde bisher akzidentell durch direkte Exposition mit dem infektiösen Agens verursacht, das heißt durch kontaminierte Gewebe, die mit dem ZNS in Verbindung standen (Will 2003).…”

“…Während der MM-Genotyp bei zerebraler Infektion durch Dura mater dominiert, kommt der VV-Genotyp bei peripherer Applikation von hGH überpropor-tional häufig vor (Brown P et al 2000, Brandel et al 2003. (Pattison 1965).…”

Propagation of scrapie in peripheral nerves after footpad infection in normal and neurotoxin exposed hamsters

“…Known sources of iatrogenic CJD in humans are transmission via infected grafts (cornea or meninx), via neurosurgical devices or transmission by the administration of human pituitary hormones [10]. There is some evidence that TSE can be transmitted by blood transfusion from infected donors [33,40].…”