NR AWII
AU Krasnianski,A.; Kallenberg,K.; Collie,D.A.; Meissner,B.; Schulz-Schaeffer,W.J.; Heinemann,U.; Summers,D.M.; Kretzschmar,H.A.; Will,R.G.; Zerr,I.
TI MRI in the classical MM1 and the atypical MV2 subtype of sCJD: an interobserver agreement study
QU International Conference - Prion 2006: Strategies, advances and trends towards protection of society - 3.10.-6.10.2006, Torino, Italy, Lingotto Conference Centre - Poster sessions DIA-32
PT Konferenz-Poster
AB The objective of the present study was to establish clinical and radiological features in the atypical MV2 subtype of sCJD compared with the classical MM1 subtype as well as region- and weighteningdependent inter-observer correlation. Clinical features, EEG, 14-3-3 investigation, and MRI hyperintensities of basal ganglia, various cortical regions and thalamus were evaluated in 31 MM1 and 32 MV2 patients. Each MR scan was separately analyzed by two neuroradiologists blinded for PRNP genotype/prion protein type. Myoclonus, akinetic mutism and PSWCs in EEG were significantly more often in MM1 than in MV2 patients. Protein 14-3-3 sensitivity was lower in MV2 (74%) than in MM1 patients (96%). T2-sensitivity for basal ganglia hyperintensities was higher in the MV2 subtype (84% in both observers versus 61% in observer 1 and 42% in observer 2 in MM1 patients). Significant inter-observer agreement was found for basal ganglia and thalamus on T2, FLAIR, PD, and DWI, but for cortical hyperintensities only on DWI. Thalamic changes were significantly more frequent in MV2 patients (86% vs. 12.5% in MM1 patients on DWI). MRI was the most sensitive investigation in MV2, and 14-3-3 test in MM1 patients. High frequency of thalamic hyperintensities in the MV2 subtype allowed differentiation from MM1 patients. Good interobserver agreement was found for basal ganglia and thalamus in all weightenings. DWI was the only imaging method allowing reliably reproducible detection of cortical hyperintensities. Since interobserver correlation for cortical hyperintensities on T2, FLAIR and PD is relatively low, the cortical changes should not be over-interpreted in these sequences.
AD A. Krasnianski (anna.krasnianski@med.uni-goettingen.de), B. Meissner, U. Heinemann, I. Zerr: Department of Neurology, Georg-August University Göttingen, Germany; K. Kallenberg: Department of Neuroradiology, Georg-August University Göttingen, Germany; D.A. Collie, D.M. Summers: Department of Neuroradiology, Western General Hospital Edinburgh, UK; W.J. Schulz-Schaeffer: Department of Neuropathology, Georg-August University Göttingen, Germany; H.A. Kretzschmar: Department of Neuropathology, Ludwig-Maximilian University Munich, Germany; R.G. Will: Department of Neurology, Western General Hospital Edinburgh, UK
SP englisch
PO Italien