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

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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.

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Author information

Authors and Affiliations

  1. Institute of Neuropathology, University Hospital of Zürich, Schmelzbergstrasse 12, 8091, Zurich, Switzerland

    Katharina Stoeck MD & Adriano Aguzzi MD, PhD

  2. Dept. of Neurology, University Hospital Zurich, Zurich, Switzerland

    Klaus Hess MD

  3. Swiss Federal Office of Public Health, Bern, Switzerland

    Lorenz Amsler MD & Tobias Eckert MD

  4. Institute of Surgical Pathology, University Hospital Zurich, Zurich, Switzerland

    Dieter Zimmermann PhD

  5. Institute of Neuropathology, University Hospital Hamburg-Eppendorf, Hamburg-Eppendorf, Germany

    Markus Glatzel MD

  6. Dept. of Neurology, University Hospital Hamburg-Eppendorf, Hamburg-Eppendorf, Germany

    Katharina Stoeck MD

  7. CSL Behring, Bern, Switzerland

    Lorenz Amsler MD

  8. Institute of Neuropathology, University Hospital Hamburg-Eppendorf, Martinistrasse 52, 20246, Hamburg, Germany

    Adriano Aguzzi MD, PhD & Markus Glatzel MD

Authors
  1. Katharina Stoeck MD
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  2. Klaus Hess MD
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  3. Lorenz Amsler MD
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  4. Tobias Eckert MD
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  5. Dieter Zimmermann PhD
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  6. Adriano Aguzzi MD, PhD
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  7. Markus Glatzel MD
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Corresponding authors

Correspondence to Adriano Aguzzi MD, PhD or Markus Glatzel MD.

Additional information

M. Glatzel and A. Aguzzi coordinated the design and operation of the study. Katharina Stoeck and Klaus Hess were involved in clinical assessment of patients. M. Glatzel and Dieter Zimmermann were involved in assessment of specimen. All authors contributed to the manuscript and approved the final version. M. Glatzel and A. Aguzzi had full access to all data in the study and had final responsibility for the decision to submit for publication.

The study was performed according to established ethical guidelines

This study was supported by grants of the Swiss Federal Office of Public Health and the Swiss National Science Foundation.

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Stoeck, K., Hess, K., Amsler, L. et al. Heightened incidence of sporadic Creutzfeldt-Jakob disease is associated with a shift in clinicopathological profiles. J Neurol 255, 1464–1472 (2008). https://doi.org/10.1007/s00415-008-0900-0

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  • DOI: https://doi.org/10.1007/s00415-008-0900-0

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