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AJNR - American Journal of Neuroradiology

Published ahead of print on July 3, 2008
doi: 10.3174/ajnr.A1122

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American Journal of Neuroradiology 29:1519-1524, September 2008
© 2008 American Society of Neuroradiology

BRAIN

Isolated Cortical Signal Increase on MR Imaging as a Frequent Lesion Pattern in Sporadic Creutzfeldt-Jakob Disease

B. Meissner^a,e, K. Kallenberg^b, P. Sanchez-Juan^c,d, A. Krasnianski^a, U. Heinemann^a, D. Varges^a, M. Knauth^b and I. Zerr^a

^a National TSE Reference Center at the Department of Neurology, Georg-August University of Göttingen, Göttingen, Germany
^b Department of Neuroradiology and MR Research in Neurology and Psychiatry, Georg-August University of Göttingen, Göttingen, Germany
^c Foundation Marqués de Valdecilla, El Instituto de Formación e Investigación Marqués de Valdecilla (IFIMAV), Santander, Spain
^d Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), University of Zürich, Zürich, Switzerland
^e Department of Gerontopsychiatry, University of Zürich, Zürich, Switzerland

Please address correspondence to Bettina Meissner, MD, Department of Gerontopsychiatry, University of Zürich, Minervastr. 145, CH-8008 Zürich, Switzerland; e-mail: bettina.meissner{at}puk.zh.ch

BACKGROUND AND PURPOSE: Hyperintense basal ganglia on MR imaging support the diagnosis of sporadic Creutzfeldt-Jakob disease (CJD). Our aim was to study the frequency of patients with sporadic CJD presenting with and without characteristic basal ganglia lesions on MR imaging and to examine the corresponding patient characteristics.

MATERIALS AND METHODS: Fluid-attenuated inversion recovery (FLAIR) and diffusion-weighted images (DWI) of 55 patients with CJD were assessed for signal-intensity increase (FLAIR) or restricted diffusion (DWI) in 7 cortex regions and the basal ganglia, thalamus, and cerebellum. Patient characteristics as well as electroencephalography, CSF, and codon 129 genotype of the prion protein gene (PRNP) were correlated with the most frequent MR imaging lesion patterns.

RESULTS: Two major lesion patterns were identified by DWI: cortex and basal ganglia involvement (two thirds) and isolated cortex involvement (one third). In the latter patient group, the cortex involvement was widespread (at least 3 regions affected in 89% on DWI) and usually included the frontal and parietal lobes (78%). The length of the disease course was significantly prolonged (median, 12 versus 5 months). No significant differences were observed concerning electroencephalography and CSF findings and codon 129 genotype distributions. Of 4 patients with normal MR imaging findings, the CSF was positive for the 14-3-3 protein in 3.

CONCLUSION: A high number of patients with CJD present without basal ganglia lesions on MR imaging. Isolated cortex involvement on DWI and FLAIR should lead to suggestion of CJD, even if the disease course is only slowly progressive. Additional 14-3-3 protein analysis in the CSF may support the CJD diagnosis.