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NEUROLOGY 2006;67:637-643
© 2006 American Academy of Neurology

CSF tests in the differential diagnosis of Creutzfeldt-Jakob disease

P. Sanchez-Juan, MD, A. Green, PhD, A. Ladogana, MD, N. Cuadrado-Corrales, PhD, R. Sáanchez-Valle, MD, E. Mitrováa, MD, K. Stoeck, MD, T. Sklaviadis, PhD, J. Kulczycki, PhD, K. Hess, MD, M. Bodemer, D. Slivarichová, MSc, A. Saiz, MD, M. Calero, PhD, L. Ingrosso, PhD, R. Knight, FRCP(E), A. C.J.W. Janssens, PhD, C. M. van Duijn, PhD and I. Zerr, MD

From the Departments of Epidemiology &s Biostatistics (P.S.-J., C.M.v.D.) and Public Health (A.C.J.W.J.), Erasmus University Medical Center Rotterdam, The Netherlands; National CJD Surveillance Unit (A.G., R.K.), The University of Edinburgh, UK; Istituto Superiore di Sanita (A.L., L.I.), Laboratory of Virology, Rome, Italy; Instituto de Salud Carlos III (N.C.-C., M.C.), Madrid, Spain; Hospital Clinic Provincial de Barcelona (R.S.-V., A.S.), Spain; Institute of Preventive and Clinical Medicine (E.M., D.S.), Bratislava, Slovakia; National Reference Center for Human Prion Diseases (NRPE) (K.S.), Institute of Neuropathology, Zurich, Switzerland; Laboratory of Pharmacology (T.S.), Department of Pharmaceutical Sciences, School of Health Sciences, Aristotle University of Thessaloniki, Greece; Institute of Psychiatry and Neurology (J.K.), I-st Neurological Department, Warsaw, Poland; Department of Neurology (K.H.), University Hospital Zurich; and National Reference Center for Transmissible Spongiform Encephalopathies (M.B., I.Z.), Department of Neurology, Georg-August-University Göttingen, Germany.

Address correspondence and reprint requests to Dr. Inga Zerr, National Reference Center for Transmissible Spongiform Encephalopathies, Department of Neurology, Georg-August-University Göttingen, Robert-Koch-Str. 40, 37075 Göttingen, Germany; e-mail: IngaZerr{at}med.uni-goettingen.de

Objectives: To analyze the diagnostic sensitivity and specificity of various brain-derived proteins (14-3-3, Tau, neuron specific enolase [NSE], and S100b) in the CSF of patients with Creutzfeldt-Jakob disease (CJD) and to analyze biologic factors that modify these parameters.

Methods: CSF was tested for 14-3-3, Tau, NSE, and S100b in 1,859 patients with sporadic, genetic, iatrogenic, and variant CJD, and in 1,117 controls.

Results: The highest sensitivity was achieved for 14-3-3 and Tau in sporadic CJD (85% and 86%), and a combined determination of 14-3-3 and Tau, S100b, or NSE increased the sensitivity to over 93%. A multivariate analysis showed that the sensitivity of all tests was highest in patients with the shortest disease duration, age at onset >40 years, and homozygosity at codon 129 of the prion protein gene. In a group of patients with repeated lumbar punctures, a second test also increased the diagnostic sensitivity.

Conclusions: The detection of elevated levels of brain-derived proteins in the CSF in patients with suspected Creutzfeldt-Jakob disease is a valuable diagnostic test. A second lumbar puncture may be of value in patients with atypical clinical course in whom the first test was negative.

Additional material related to this article can be found on the Neurology Web site. Go to www.neurology.org and scroll down the Table of Contents for the August 22 issue to find the title link for this article.

The national studies were supported in Greece by the Greek Ministry of Health, through KEEL (Center for Control of Infectious Diseases), in Italy by the Ministry of Health and the Instituto Superiore di Sanita, in the Netherlands by the Dutch Ministry of Health, Welfare and Sports, in Slovakia by Ministry of Health and European Commission (SEEC-CJD project), in Spain by a grant from the Ministerio de Ciencia y Tecnologáia (MCyT EET 2001/2216) and Spanish Ministerio de Sanidad y Consumo, grant number DGVI 1312/04-1, in Switzerland by Bundesamt für Gesundheit, Bern (NRPE-BAG contracts n° 03.001297 and n° 04.002363), in United Kingdom by the Department of Health and the Scottish Home Office Department of Health, and in Germany by the Bundesministerium für Gesundheit und Soziale Sicherung (BMGS) (GZ: 325-4471-02/15) and by the Bundesministerium für Bildung und Forschung (BMBF) (KZ: 0312720 and 01GI0301 to I.Z). Raquel Sanchez-Valle is a recipient of a post-residency grant from Instituto de Salud Carlos III (ISCIII, Madrid, Spain). Pascual Sanchez-Juan was supported by the postMIR grant, Wenceslao Lopez Albo from the IFIMAV Institute of the Fundaciáon Páublica Marquáes de Valdecilla. The collaborative study was funded by grants from the European Commission (EC) (QLG3-CT-2002-81606, Co-ordinator: I. Zerr).

Disclosure: The authors report no conflicts of interest.

Received July 26, 2005. Accepted in final form April 27, 2006.




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