Laura Burns Eisenmenger BA, Presenter: Nothing to Disclose

Marie-Claire Porter, Abstract Co-Author: Nothing to Disclose

Andrew Thompson MD, Abstract Co-Author: Nothing to Disclose

Ana Lukic MD, Abstract Co-Author: Nothing to Disclose

Rolf Jager MD, Abstract Co-Author: Nothing to Disclose

Peter Rudge MD, Abstract Co-Author: Nothing to Disclose

Simon Mead MD, PhD, Abstract Co-Author: Nothing to Disclose

John Collinge MD, Abstract Co-Author: Nothing to Disclose

Harpreet K. Hyare MBBS, Abstract Co-Author: Nothing to Disclose

Hyperintensity in the cortex, basal ganglia and thalamus are the classical findings on Diffusion Weighted Imaging (DWI), fluid-attenuated inversion recovery (FLAIR) and T2-weighted sequences in Creutzfeldt-Jakob Diseases (1). The pattern of anatomical involvement can be variable and is thought to be distinct for each molecular subtype. Here we report the serial MRI findings in a large cohort of patients with different types of CJD to determine the evolution of signal abnormalities with clinical course.

Inclusion criteria were: signal abnormality on MRI sequences; at least 2 MRI studies performed and DWI sequences performed at each time point. DWI and FLAIR sequences on the baseline study were reviewed by 2 neuroradiologists and increased signal intensity (SI) noted in 7 cortical regions, basal ganglia, thalamus and cerebellum. Subsequent studies in the same patient were reviewed for change in extent and SI in each region. Atrophy was also noted.  

34 patients, 26 with sporadic CJD (sCJD), 6 with variant CJD (vCJD) and 2 with Inherited Prion Disease (IPD) with a mean age 56.6 years (range 18-85) were included in the study. Mean number of scans per patient was 2.6 (range 2-8), with mean time between first and last scans, 3.7 months (range 0.5-30). All the sCJD cases and 2 IPD cases, there was an increase in SI in the basal ganglia on serial imaging (Figure 1A). 24 of the sCJD cases showed an increase in SI and extent of signal in contiguous cortical areas compared to baseline MRI. In 2 of the sCJD cortical SI decreased on final imaging (>18months), before death and corresponded with marked supratentorial atrophy (Figure 1B).

This is the first longitudinal study describing the MRI findings in a large cohort of patients with CJD. Follow-up imaging showed increased SI in the basal ganglia. It is not yet clear whether the increased signal is due to restricted diffusion or a prolonged T2 effect and subsequent quantitative studies will address this question. Resolution of cortical signal intensity at end-stage MRI is a potential pitfall that should be made aware to Neuroradiologists.

Follow-up imaging in patients with CJD showed increased SI in the basal ganglia. Resolution of cortical signal intensity at end-stage MRI is also seen.

Eisenmenger, L, Porter, M, Thompson, A, Lukic, A, Jager, R, Rudge, P, Mead, S, Collinge, J, Hyare, H, Evolution of MRI Signal Abnormalities in Creutzfeldt-Jakob Disease.  Radiological Society of North America 2012 Scientific Assembly and Annual Meeting, November 25 - November 30, 2012 ,Chicago IL. http://archive.rsna.org/2012/12034783.html