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MRI and clinical syndrome in dura materrelated Creutzfeldt-Jakob disease

Meissner, B. ; Kallenberg, K. ; Sanchez-Juan, P. ; Ramljak, S. ; Krasnianski, A. ; Heinemann, U. ; Eigenbrod, S. ; Gelpi, E. ; Barsic, B. ; Kretzschmar, H. ; Schulz-Schaeffer, W. ; Knauth, M. ; Zerr, I.

In: Journal of Neurology, 2009, vol. 256, no. 3, p. 355-363

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    Titre
    • MRI and clinical syndrome in dura materrelated Creutzfeldt-Jakob disease
    Auteur
    • Meissner, B.. National TSE Reference Center, Dept. of Neurology, University of Göttingen, Robert-Koch-Str. 40, 37075, Göttingen, Germany
    • Kallenberg, K.. Dept. of Neuroradiology and MR research in Neurology and Psychiatry, Göttingen, Germany
    • Sanchez-Juan, P.. Foundation Marqués de Valdecilla, IFIMAV, Santander, Spain
    • Ramljak, S.. Dept. of Neuropathology, Georg-August University, Göttingen, Germany
    • Krasnianski, A.. National TSE Reference Center, Dept. of Neurology, University of Göttingen, Robert-Koch-Str. 40, 37075, Göttingen, Germany
    • Heinemann, U.. National TSE Reference Center, Dept. of Neurology, University of Göttingen, Robert-Koch-Str. 40, 37075, Göttingen, Germany
    • Eigenbrod, S.. Center for Neuropathology and Prion Research, Ludwig-Maximilians-Universität, München, Germany
    • Gelpi, E.. Institute of Neurology, Medical University of Vienna, Vienna, Austria
    • Barsic, B.. University Hospital of Infectious Diseases, Zagreb, Croatia
    • Kretzschmar, H.. Center for Neuropathology and Prion Research, Ludwig-Maximilians-Universität, München, Germany
    • Schulz-Schaeffer, W.. Dept. of Neuropathology, Georg-August University, Göttingen, Germany
    • Knauth, M.. Dept. of Neuroradiology and MR research in Neurology and Psychiatry, Göttingen, Germany
    • Zerr, I.. National TSE Reference Center, Dept. of Neurology, University of Göttingen, Robert-Koch-Str. 40, 37075, Göttingen, Germany
    Type de document
    • Postprint
    Identifiant OAI-PMH
    • oai:doc.rero.ch:313954
    Summary
    Objective : Iatrogenic Creutzfeldt-Jakob disease (iCJD) is mainly associated with dura mater (DM) grafts and administration of human growth hormones (hGH). Data on disease course in DM-CJD are limited. We describe the clinical and diagnostic findings in this patient group with special emphasis on MRI signal alterations. Methods : Ten DM-CJD patients were studied for their clinical symptoms and diagnostic findings. The MRIs were evaluated for signal increase of the cortical and subcortical structures. Results : DM-CJD patients had a median incubation time of 18 years and median disease duration of 7 months. The majority of patients were MM homozygous at codon 129 of the prion protein gene (PRNP) and presented with gait ataxia and psychiatric symptoms. No correlation between the graft site and the initial disease course was found. The MRI showed cortical and basal ganglia signal increase each in eight out of ten patients and thalamic hyperintensity in five out of ten cases. Of interest, patients with thalamic signal increase were homozygous for methionine. Conclusion : The MRI findings in DM-CJD largely resemble those seen in sporadic CJD, as the cortex and basal ganglia are mainly affected