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

Cortical high signal on FLAIR or diffusion weighted (DW) MRI is a frequent finding in sporadic Creutzfeldt-Jakob disease (sCJD). We analyzed, whether the cortical changes in sCJD patients have a typical pattern.

40 patients were studied: 18 neuropathologically confirmed (definite sCJD) and 22 probable sCJD patients. The patients had been referred to the German CJD surveillance unit in the years 2001-2003. As a prerequisite, only those sCJD patients were included in the study whose MRIs 1) showed hyperintense cortical signal (defined as focal linear high signal in the cerebral cortex) and 2) did not reveal another cause for cortical high signal. For analysis, the hardcopy documentations of axial scans were used. 13 patients were examined with FLAIR sequences, 8 patients with diffusion-weighted sequences, 19 patients were examined with both. Two observers analyzed the MRIs in consensus, assigning cortical signal changes to the corresponding anatomical structures in slices from a brain atlas.

38/40 patients (95%) showed high signal in the insulate gyrus, 36/40 patients (91%) in the cingulate and in the superior frontal gyri each. The cortical areas near the midline were frequently involved: the paracentral lobe in 30/40 patients (75%), the precuneus in 28/40 patients (70%). Less frequently, abnormal signal was detected in the primary motor cortex (precentral gyrus, 8/20, 21%) and the primary sensory cortex (postcentral gyrus, 6/40, 15%). Asymmetric lesions were present in 15/40 patients (37.5%). There was no involvement of white matter adjacent to the areas of cortical high signal, and the distribution of the cortical hyperintensities did not match any vascular territory.

Cortical MR-changes in sCJD have a typical pattern: it consists of hyperintense signal in the insulate, cingulate and superior frontal gyri as well as the cortical areas near the midline. The primary motor and sensory cortex are mostly spared. Asymmetric distribution of the lesions does not exclude sCJD. Demented patients with clinical symptoms of sCJD and the described cortical changes should be diagnosed with sCJD.

Tschampa, H, Kallenberg, K, Zerr, I, Kretzschmar, H, Knauth, M, Urbach, H, Pattern of Cortical Changes in Sporadic Creutzfeldt-Jakob Disease.  Radiological Society of North America 2005 Scientific Assembly and Annual Meeting, November 27 - December 2, 2005 ,Chicago IL. http://archive.rsna.org/2005/4409153.html