NR ATJU
AU Meissner,B.; Kallenberg,K.; Bartl,M.; Krasnianski,A.; Varges,D.; Kretzschmar,H.A.; Knauth,M.; Zerr,I.
TI Sporadic Creutzfeldt-Jakob disease: lesion profiles onT2, FLAIR- and diffusion-weighted MRI
QU International Conference - Prion 2005: Between fundamentals and society's needs - 19.10.-21.10.2005, Congress Center Düsseldorf - Poster Session: Diagnosis DIA-06
PT Konferenz-Poster
AB
Objective
Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare and fatal disease caused by the accumulation of prions. The diagnosis during lifetime is mainly based on the clinical findings, 14-3-3 protein in the cerebrospinal fluid (CSF) and the occurrence of periodic sharp-wave complexes (PSWC) in the EEG.
Hyperintense basal ganglia on T2-weighted MRI have been reported as the classical finding in CJD in the late 1980s. With the emergence of more sensitive MRI techniques (FLAIR, DWI), however, a wide range of MRI signal changes - mainly affecting cortical structures - became evident.
It was therefore our aim to evaluate the MRI signal changes seen on T2-, FLAIR- and diffusion-weighted images (DWI) and to define an MRI lesion pattern typical of sCJD.
Methods
The T2-, FLAIR- and diffusion-weighted MRIs (all from the same examination) of 56 CJD patients were assessed for signal increase of the cortex and subcortical structures (basal ganglia and thalamus).
Results
Signal increase of the basal ganglia was found in 61% (DWI) and 41% (FLAIR). Cortical signal increase was found in 89% (DWI) and 75% (FLAIR). Cortical signal increase was seen most often in the frontal and temporal lobes (82% and 75% on DWI). Basal ganglia and cortex were affected in 59% (DWI). The highest sensitivity was achieved for the affection of basal ganglia and/or cortex (91% on DWI).
Conclusions
To achieve the highest MRI sensitivity for diagnosing CJD, a typical lesion pattern including cortex and/or basal ganglia should be defined. To maintain a high specificity, the MRI lesion pattern should be interpreted with regard to CSF and EEG findings.
AD Bettina Meissner, Kai Kallenberg, Mario Bartl, Anna Krasnianski, Daniela Varges, Michael Knauth, Walter Schulz-Schaeffer, Inga Zerr, University of Göttingen, Germany; Hans A. Kretzschmar, University of Munich
SP englisch
PO Deutschland