Chronological Changes in MRI and CSF Biochemical Markers in Creutzfeldt-Jakob Disease Patients

Abstract

Background: There are currently no markers for evaluating chronological changes in Creutzfeldt-Jakob disease (CJD). We examined if chronological changes in biochemical markers in cerebrospinal fluid (CSF) and diffusion-weighted magnetic resonance imaging (DWI) were utilizable for this purpose. Methods: Ten independent patients were divided into two groups of 5 patients each. We analyzed CSF biochemical markers, DWI and the clinical course in one group. In the remaining group, only the CSF biochemical markers were analyzed before and after the onset of akinetic mutism. Results: The level of total tau (t-tau) protein in CSF in the early phase after disease onset was 2,655 ± 423.9 pg/ml, reaching a mean peak of 14,675 ± 1,240 pg/ml in the middle phase and gradually declining after that. Just before patients deteriorated into akinetic mutism, t-tau protein titers reached a maximum (8,786 ± 2,975 pg/ml). There were dramatic changes in t-tau protein levels throughout the clinical course, unlike the other markers. DWI was not always utilizable, because of discordance with clinical symptoms seen in this study. Four cases exhibited peaks in t-tau protein levels while the patients fell into akinetic mutism except 1 case. Conclusion: Our results suggest that t-tau protein is the most sensitive marker of disease progression in CJD patients.