Journal of Neurology

, Volume 255, Issue 10, pp 1464–1472 | Cite as

Heightened incidence of sporadic Creutzfeldt-Jakob disease is associated with a shift in clinicopathological profiles

  • Katharina Stoeck
  • Klaus Hess
  • Lorenz Amsler
  • Tobias Eckert
  • Dieter Zimmermann
  • Adriano Aguzzi
  • Markus Glatzel
ORIGINAL COMMUNICATION

Abstract

Incidences of human transmissible spongiform encephalopathies are monitored by national registries in the majority of countries in Western Europe. During the past 13 years incidences for Creutzfeldt-Jakob disease (CJD) in Switzerland fluctuated between 0.4 and 2.63 cases/106 inhabitants. We have compared clinicpathological patient profiles including geographic and gender distribution, age at disease onset, duration of disease, clinical symptoms, and recognized or hypothetical risk factors for CJD, genetic risk factors, biochemical and histopathological data for two cohorts of Swiss sporadic CJD patients from years of regular sporadic CJD incidence (1996–2000, mean incidence 1.3 cases/106 inhabitants, n = 47) to Swiss sporadic CJD patients from years of elevated sporadic CJD incidence (2001–2004, mean incidence 2.3 cases/106 inhabitants, n = 73). Sporadic CJD patients from the cohort with elevated sporadic CJD incidence presented with a higher frequency of rare sporadic CJD subtypes. Patients of these subtypes were significantly older and showed a skewed male/female ratio when compared to published patients of identical sporadic CJD-types or to patients from the 1996–2000 cohort and indicates that improved detection of rare sporadic CJD subtypes may have contributed to increased incidence.

Key words

Creutzfeldt-Jakob disease prions dementia epidemiology 

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Copyright information

© Springer 2008

Authors and Affiliations

  • Katharina Stoeck
    • 1
    • 6
  • Klaus Hess
    • 2
  • Lorenz Amsler
    • 3
    • 7
  • Tobias Eckert
    • 3
  • Dieter Zimmermann
    • 4
  • Adriano Aguzzi
    • 1
    • 8
  • Markus Glatzel
    • 5
    • 8
  1. 1.Institute of NeuropathologyUniversity Hospital of ZürichZurichSwitzerland
  2. 2.Dept. of NeurologyUniversity Hospital ZurichZurichSwitzerland
  3. 3.Swiss Federal Office of Public HealthBernSwitzerland
  4. 4.Institute of Surgical PathologyUniversity Hospital ZurichZurichSwitzerland
  5. 5.Institute of NeuropathologyUniversity Hospital Hamburg-EppendorfHamburg-EppendorfGermany
  6. 6.Dept. of NeurologyUniversity Hospital Hamburg-EppendorfHamburg-EppendorfGermany
  7. 7.CSL BehringBernSwitzerland
  8. 8.Institute of NeuropathologyUniversity Hospital Hamburg-EppendorfHamburgGermany

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