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Diagnosis of Creutzfeldt-Jakob disease by measurement of S100 protein in serum: prospective case-control study

BMJ 1998; 316 doi: https://doi.org/10.1136/bmj.316.7131.577 (Published 14 February 1998) Cite this as: BMJ 1998;316:577

This article has a correction. Please see:

  1. Markus Otto (100634.133{at}compuserve.com), group leader, laboratory diagnosis of CJDa,
  2. Jens Wiltfang, senior research fellowb,
  3. Ekkehard Schütz, research fellowc,
  4. Inga Zerr, research fellowa,
  5. Anke Otto, research fellowa,
  6. Annette Pfahlberg, statisticiand,
  7. Olaf Gefeller, associate professor of medical statisticsd,
  8. Manfred Uhr, research fellowe,
  9. Armin Giese, research fellowf,
  10. Thomas Weber, head, department of neurologyg,
  11. Hans A Kretzschmar, head, department of neuropathologyf,
  12. Sigrid Poser, head, epidemiology and early diagnosis of CJDa
  1. a Neurologische Klinik und Poliklinik, Georg-August-Universität Göttingen, Robert-Koch Strasse 40, D-37075 Göttingen, Germany
  2. b Psychiatrische Klinik und Poliklinik, Georg-August-Universität Göttingen
  3. c Abteilung Klinische Chemie, Georg-August-Universität Göttingen
  4. d Abteilung Medizinische Statistik, Georg-August-Universität Göttingen
  5. e Max-Planck Institut für Psychiatrie, Kraepelinstrasse 10, D-80804 Munich, Germany
  6. f Institut für Neuropathologie, Georg-August-Universität Göttingen
  7. g Neurologische Abteilung Marienkrankenhaus Hamburg, Alfredstrasse 9, D-22087 Hamburg, Germany
  1. Correspondence to: Dr Otto
  • Accepted 28 October 1997

Abstract

Abstract

Objective: To analyse serum concentrations of brain specific S100 protein in patients with Creutzfeldt-Jakob disease and in controls.

Design: Prospective case-control study.

Setting: National Creutzfeldt-Jakob disease surveillance unit.

Subjects: 224 patients referred to the surveillance unit with suspected Creutzfeldt-Jakob disease and 35 control patients without dementia.

Main outcome measure: Serum concentration of S100 protein in patients with Creutzfeldt-Jakob disease, in patients with other diseases causing dementia, and in the control group.

Results: Of the 224 patients with suspected Creutzfeldt-Jakob disease, 65 were classed as definitely having the disease after neuropathological verification, an additional 6 were classed as definitely having the disease as a result of a genetic mutation, 43 as probably having the disease, 36 as possibly having the disease, and 74 patients were classed as having other disease. In the 108 patients classed as definitely or probably having Creutzfeldt-Jakob disease the median serum concentration of S100 was 395 pg/ml (SD 387 pg/ml). This was significantly higher than concentrations found in the 74 patients classed as having other diseases (median 109 pg/ml; SD 177 pg/ml; P=0.0001). At a cut off point of 213 pg/ml sensitivity for the diagnosis of the disease was 77.8% (95% confidence interval 68.8% to 85.2%) and specificity was 81.1% (70.3% to 89.3%). There was a significant difference in survival at different concentrations of S100 in Kaplan-Meier curves (P=0.023).

Conclusion: Measurement of serum concentrations of S100 is a valuable tool which can be used more easily than tests on cerebrospinal fluid in the differential diagnosis of Creutzfeldt-Jakob disease. More studies are needed to determine whether serial testing of serum S100 improves diagnostic accuracy.

Key messages

  • Creutzfeldt-Jakob disease is a rare, fatal neurodegenerative disease. Diagnosis is made clinically and neuropathologically

  • There is no serum test which allows the diagnosis to be made while the patient is alive

  • In this study raised serum concentrations of S100 protein were found in patients with Creutzfeldt-Jakob disease

  • Serum concentrations of S100 could be used with a sensitivity of 77.8% and a specificity of 81.1% to confirm Creutzfeldt-Jakob disease in the differential diagnosis of diseases that cause dementia

  • Serial measurement of S100 concentrations will enhance diagnostic accuracy

Footnotes

  • Accepted 28 October 1997
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