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Journal of Neurology

, Volume 244, Issue 9, pp 566–570 | Cite as

S-100 protein concentration in the cerebrospinal fluid of patients with Creutzfeldt-Jakob disease

  • Markus Otto
  • Holger Stein
  • Annemarie Szudra
  • Inga Zerr
  • Monika Bodemer
  • Olaf Gefeller
  • Sigrid Poser
  • Hans A. Kretzschmar
  • Michael Mäder
  • Thomas Weber
Original communication

Abstract

We evaluated S-100 levels in paired cerebrospinal fluid (CSF) and serum samples in a group of 135 patients referred to the German Creutzfeldt-Jakob disease (CJD) surveillance unit from June 1993 to May 1995. The patients were seen in a prospective case control study. The diagnosis of probable CJD during life was made in any patient presenting with rapidly progressive dementia of less than 2 years’ duration, typical periodic sharp wave complexes (PSWCs) in the EEG and at least two of the following findings: myoclonus, visual/or cerebellar symptoms, pyramidal and/or extrapyramidal signs and/or akinetic mutism. Patients presenting with the above clinical signs and symptoms but without PSWCs were classified as possible, while those with a dementia of a duration exceeding 2 years and without PSWCs were classified as other. S-100 was determined in paired CSF and serum samples by a commercially available enzyme-linked immunosorbent assay. In a group of 76 patients with definite and probable CJD, S-100 concentration (median 25 ng/ml, range 2–117) in CSF was significantly higher (P < 0.0001) than in 32 patients diagnosed as other (median 4 ng/ml, range 1–19). Serum levels of S-100 were below 0.5 ng/ml in all groups. At a cut-off of 8 ng/ml an optimum sensitivity of 84.2% with a specificity of 90.6% for the diagnosis of CJD by the determination of S-100 in CSF is obtained. S-100 levels exceeding 8 ng/ml in CSF support the diagnosis of CJD in any patient presenting with rapidly progressive dementia.

Key words Protein S-100 CSF Creutzfeldt-Jakob disease 

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Copyright information

© Springer-Verlag Berlin Heidelberg 1997

Authors and Affiliations

  • Markus Otto
    • 1
  • Holger Stein
    • 1
  • Annemarie Szudra
    • 1
  • Inga Zerr
    • 1
  • Monika Bodemer
    • 1
  • Olaf Gefeller
    • 2
  • Sigrid Poser
    • 1
  • Hans A. Kretzschmar
    • 3
  • Michael Mäder
    • 1
  • Thomas Weber
    • 1
  1. 1.Neurologische Klinik und Poliklinik Georg-August-Universität Göttingen, Göttingen, GermanyDE
  2. 2.Abteilung Medizinische Statistik, Georg-August-Universität Göttingen, Göttingen, GermanyDE
  3. 3.Institut für Neuropathologie, Georg-August-Universität Göttingen, Göttingen, GermanyDE

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