Creutzfeldt-Jakob disease in Germany: a prospective 12-year surveillance

Abstract

Creutzfeldt-Jakob disease (CJD) is a rare and fatal neurodegenerative disorder with a worldwide incidence of 1-1.5 per million. As in other countries, a CJD surveillance unit with a clinical and neuropathological approach was established in Goettingen (Germany) in 1993. Here we report the epidemiological data from a prospective 12-year surveillance. Since 1993, there has been an increasing incidence of CJD, from 0.7 in 1993 to 1.6 in 2005 with a quite stable level since 1998. During this period, the proportion of patients with MV and VV codon 129 genotype rose, possibly because of better identification of atypical subtypes. Six percent of all patients had a PRNP mutation, mainly D178N-129M (FFI), E200K and V210I. Iatrogenic CJD was a rare phenomenon. No patient infected by cadaveric growth hormone extracts was reported. Furthermore, no variant CJD patient has yet been identified in Germany. Differential diagnoses revealed a variety of neurodegenerative diseases, with Alzheimer's disease in the lead. One-third of the non-CJD patients included in this study suffered from a potentially treatable disorder such as metabolic or inflammatory diseases. The incidence and mortality rates in Germany are similar to those in other European countries. In contrast, however, acquired forms, such as iatrogenic and variant CJD are still rare in Germany or have not yet been identified.

Diagnostic profiles of patients with late-onset Creutzfeldt–Jakob disease differ from those of younger Creutzfeldt–Jakob patients: a historical cohort study using data from the German National Reference Center

“…The latter hypothesis is supported by the observation that age-specific sCJD incidence rates have been increasing in these age groups in those countries that started an active CJD surveillance program [2,4,14].…”

“…Agespecific incidence rates increase with age in most neurodegenerative diseases (e.g. Alzheimer's disease, Parkinson's disease) [9]; however, sCJD incidence rates are highest between 60 and 75 years of age and drop down thereafter [1,4,5]. The drop was located at a cut-off of 75 years in a USbased study by Holman et al, whereas it seemed to be slightly higher in two European studies available.…”

Cerebrospinal fluid tau levels are a marker for molecular subtype in sporadic Creutzfeldt-Jakob disease

“…This diagnostic study is based on data from an ongoing surveillance study of the German national reference centre for Transmissible Spongiform Encephalopathies (Heinemann, et al, 2007). control group of patients diagnosed with Alzheimer's disease (AD) in the same centre (using Dubois' revised research criteria (Dubois, et al, 2007)) and enrolled in a longitudinal observational study (Schmidt, et al, 2013) were tested for their codon 129 genotype and added to the study population.…”

“…Creutzfeldt-Jakob disease, tau, biomarker, subtype, neurodegeneration 2 31 32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 47 48Introduction Creutzfeldt-Jakob disease (CJD) is the most common human prion disease affecting about 1 in 1,000,000 individuals per year (Heinemann, et al, 2007). There are genetic and iatrogenic causes of CJD; however, the majority of patients suffers from a sporadic form of Creutzfeldt-Jakob disease (sCJD) (Kovacs, et al, 2005,Puoti, et al, 2012.…”

Risikofaktoren der sporadischen Creutzfeldt-Jakob-Krankheit

“…Die altersspezifische Inzidenz ist in der 7. Lebensdekade am höchsten (Heinemann et al 2007a). Das mittlere Alter bei Krankheitsbeginn beträgt bei der sCJK 66 Jahre (Min-Max: 19-91 Jahre), die Krankheit dauert im Durchschnitt 6 bis 7…”