European Journal of Epidemiology

, Volume 21, Issue 6, pp 443–447 | Cite as

Increased incidence of sporadic Creutzfeldt-Jakob disease in the age groups between 70 and 90 years in Belgium

  • B. Van Everbroeck
  • A. Michotte
  • R. Sciot
  • C. Godfraind
  • M. Deprez
  • S. Quoilin
  • J. -J. Martin
  • P. Cras
Neuroepidemiology

Abstract

From 1998 a prospective surveillance study of Creutzfeldt-Jakob disease (CJD) has been initiated in Belgium. In addition to epidemiological data, information on cerebrospinal fluid biomarkers, prion protein gene and brain neuropathology was collected. From 1-1-1998 to 31-12-2004, 188 patients were referred to the surveillance system. In 85 patients a ‘definite’ diagnosis of sporadic CJD (sCJD) could be made, whereas 26 patients remained ‘probable’. We further identified two unrelated patients with an E200K mutation, and two patients with a seven octapeptide repeat insertion in one family. In one patient a familial history was noted but genetic analysis was not performed. In 72 patients different final diagnoses were made, Alzheimer’s disease being the most frequent (N = 20). The demographic parameters of the Belgian population were similar to those observed in the rest of Europe. We did notice a significantly increased age-specific incidence (‰>‰6/106/year) of sCJD patients between 70 and 90 years old in the period 2002–2004 compared to 1998–2001 and retrospectively obtained data (1990–1997, p < 0.01). We undertook a detailed clinical and biochemical analysis to investigate this increase but could not identify any reason other than an increased vigilance for the diagnosis.

In conclusion, our study identified that in the past sCJD may have been underestimated in patients over age 70 although these patients are both clinically and neurobiochemically similar to the general sCJD phenotype.

Keywords

Diagnosis Epidemiology Prion disease Transmissible spongiform encephalopathy 

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Copyright information

© Springer Science+Business Media, Inc. 2006

Authors and Affiliations

  • B. Van Everbroeck
    • 1
  • A. Michotte
    • 2
  • R. Sciot
    • 3
  • C. Godfraind
    • 4
  • M. Deprez
    • 5
  • S. Quoilin
    • 6
  • J. -J. Martin
    • 1
  • P. Cras
    • 1
    • 7
  1. 1.Born-Bunge Institute (BBI)University of Antwerp (UA), Campus Drie Eiken (CDE)AntwerpBelgium
  2. 2.Department of NeuropathologyAcademic hospital, Free University of BrusselsBrusselsBelgium
  3. 3.Department of PathologyCatholic University of LeuvenLeuvenBelgium
  4. 4.Pathology LaboratoryCatholic University of LouvainBrusselsBelgium
  5. 5.Laboratory of NeuropathologyUniversity of LiègeSart TilmanBelgium
  6. 6.Institute of Public Health-Louis PasteurBrusselsBelgium
  7. 7.Laboratory of NeurobiologyBBI, UA, CDEWilrijkBelgium

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