Henriette J. Tschampa MD, Presenter: Nothing to Disclose

Kai Kallenberg MD, Abstract Co-Author: Nothing to Disclose

Inga Zerr MD, Abstract Co-Author: Nothing to Disclose

Hans A. Kretzschmar MD, PhD, Abstract Co-Author: Nothing to Disclose

Michael Knauth MD, PhD, Abstract Co-Author: Nothing to Disclose

Horst Urbach MD, Abstract Co-Author: Nothing to Disclose

Hyperintense signal changes in cortical and subcortical grey matter in T2-, FLAIR- and diffusion weighted (DW) magnetic resonance images (MRI) are known in sporadic Creutzfeldt-Jakob Disease (sCJD). Recently the distribution of these changes was classified as striatal, cerebral cortical and a combination of both using DW MRI. The aim of our study was to test this classification in a group of sCJD patients.

133 sCJD patients from the German reference center for CJD (60 neuropathologically confirmed and 73 clinically probable sCJD cases, examined 2001-2003) were included. We studied the MR scans of these patients as hardcopies: 125 T2w-, 88 FLAIR- and 49 DW scans. Two observers analyzed the images in consensus for high signal in the striatum, the thalamus and the cortical ribbon. Symmetry of the lesions was possible but not necessary.

Hyperintense signal changes in the cortical or subcortical grey matter were present in 98/133 cases (74%). High signal in the striatum was observed in 94/133 cases (71%), hyperintense changes in the thalamus and the cortex were present in 51/133 (38%) cases each. In detail: 23/133 (17.5%) had a “striatal only” and 23/133 (17.5%) a “striato-thalamic” involvement. A “whole cerebral grey matter” type (striatal, thalamic and cortical lesions) was present in 27/133 patients (20%). 21/133 patients (16%) showed “striato-cortical” hyperintensities without thalamic signal changes. 3/133 patients (2%) had a “cortex only” form without involvement of the basal ganglia. A “thalamus only” form was observed in one patient. A combination of high signal in the thalamus and the cortex sparing the striatum was not seen. No grey matter signal changes (“negative MRI”) were observed in 35/133 cases (26%).

In addition to striatal and cortical high signal, the thalamus is frequently involved in sCJD. Six MR-subtypes were identified combining the different lesions: striatal only, striato-thalamic, whole cerebral grey matter, striato-cortical, cortex only, thalamus only. A correlation with clinical and pathologic data will further define these subtypes.

Tschampa, H, Kallenberg, K, Zerr, I, Kretzschmar, H, Knauth, M, Urbach, H, Subtypes of Sporadic Creutzfeldt-Jakob Disease Defined by Magnetic Resonance Imaging.  Radiological Society of North America 2005 Scientific Assembly and Annual Meeting, November 27 - December 2, 2005 ,Chicago IL. http://archive.rsna.org/2005/4409321.html