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Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease


Zerr, I., Kallenberg, K., Summers, D. M., Romero, C., Taratuto, A., Heinemann, U., Breithaupt, M., Varges, D., Meissner, B., Ladogana, A., Schuur, M., Haik, S., Collins, S. J., Jansen, G. H., Stokin, G. B., Pimentel, J., Hewer, E., Collie, D., Smith, P., Roberts, H., Brandel, J. P., van Duijn, C., Pocchiari, M., Begue, C., Cras, P., Will, R. G., Sanchez-Juan, P.


Brain: A Journal Of Neurology, Volume: 132, No.: Pt 10, Pages.: 2659-2668

Year of Publication



Several molecular subtypes of sporadic Creutzfeldt-Jakob disease have been identified and electroencephalogram and cerebrospinal fluid biomarkers have been reported to support clinical diagnosis but with variable utility according to subtype. In recent years, a series of publications have demonstrated a potentially important role for magnetic resonance imaging in the pre-mortem diagnosis of sporadic Creutzfeldt-Jakob disease. Magnetic resonance imaging signal alterations correlate with distinct sporadic Creutzfeldt-Jakob disease molecular subtypes and thus might contribute to the earlier identification of the whole spectrum of sporadic Creutzfeldt-Jakob disease cases. This multi-centre international study aimed to provide a rationale for the amendment of the clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease. Patients with sporadic Creutzfeldt-Jakob disease and fluid attenuated inversion recovery or diffusion-weight imaging were recruited from 12 countries. Patients referred as ‘suspected sporadic Creutzfeldt-Jakob disease’ but with an alternative diagnosis after thorough follow up, were analysed as controls. All magnetic resonance imaging scans were assessed for signal changes according to a standard protocol encompassing seven cortical regions, basal ganglia, thalamus and cerebellum. Magnetic resonance imaging scans were evaluated in 436 sporadic Creutzfeldt-Jakob disease patients and 141 controls. The pattern of high signal intensity with the best sensitivity and specificity in the differential diagnosis of sporadic Creutzfeldt-Jakob disease was identified. The optimum diagnostic accuracy in the differential diagnosis of rapid progressive dementia was obtained when either at least two cortical regions (temporal, parietal or occipital) or both caudate nucleus and putamen displayed a high signal in fluid attenuated inversion recovery or diffusion-weight imaging magnetic resonance imaging. Based on our analyses, magnetic resonance imaging was positive in 83% of cases. In all definite cases, the amended criteria would cover the vast majority of suspected cases, being positive in 98%. Cerebral cortical signal increase and high signal in caudate nucleus and putamen on fluid attenuated inversion recovery or diffusion-weight imaging magnetic resonance imaging are useful in the diagnosis of sporadic Creutzfeldt-Jakob disease. We propose an amendment to the clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease to include findings from magnetic resonance imaging scans.;

Bibtex Citation

@article{Zerr_2009, doi = {10.1093/brain/awp191}, url = {}, year = 2009, month = {sep}, publisher = {Oxford University Press ({OUP})}, volume = {132}, number = {10}, pages = {2659--2668}, author = {I. Zerr and K. Kallenberg and D. M. Summers and C. Romero and A. Taratuto and U. Heinemann and M. Breithaupt and D. Varges and B. Meissner and A. Ladogana and M. Schuur and S. Haik and S. J. Collins and G. H. Jansen and G. B. Stokin and J. Pimentel and E. Hewer and D. Collie and P. Smith and H. Roberts and J. P. Brandel and C. van Duijn and M. Pocchiari and C. Begue and P. Cras and R. G. Will and P. Sanchez-Juan}, title = {Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease}, journal = {Brain} }


adult, aged, aged, 80 and over, analysis, antiinflammatory agents, biological markers, cerebral cortex, cerebrospinal fluid, codon, diagnosis, disease, electroencephalography, false positive reactions, female, genetics, genotype, humans, international cooperation, magnetic resonance imaging, male, middle aged, nv, pathology, reference standards, therapeutic use

Countries of Study


Types of Study

Case Control Study

Type of Interventions

Diagnostic Target Identification

Diagnostic Targets

Neuroimaging (e.g. MRI, PET, CAT etc.)