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Spongiforme Encephalopathies

  • Chapter
Inflammatory Diseases of the Brain

Part of the book series: Medical Radiology ((Med Radiol Diagn Imaging))

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Abstract

Sporadic and variant Creutzfeldt-Jakob disease (CJD), as the most important spongiform encephalopathies, are fatal neurodegenerative disorders caused by infectious proteins called prions which aggregate, accumulate and induce neuronal death and spongiform changes of the brain. Definite diagnosis of sporadic CJD (sCJD) requires biopsy, and clinical diagnosis is based on typical clinical symptoms associated with a characteristic EEG and/or detection of 14-3-3 protein in CSF. MRI shows bilateral striatal and/or cortical signal hyperintensity on DWI and FLAIR sequences in about 80% of cases. With a typical clinical picture, this MRI pattern is highly specific. Variant CJD (vCJD) has a different clinical course and a highly specific MRI pattern. It consists of symmetric bilateral increased signal intensity of the pulvinar thalami (“pulvinar sign”) in about 80% of cases, and the mediodorsal thalamic nuclei can be additionally affected (“hockey-stick” sign). In contrast to sCJD signal intensity in the thalamus is always higher than in the striate.

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Authors and Affiliations

  1. Department Radiology/Neuroradiology, University of Bonn Medical Center, Sigmund-Freud-Straße 25, 53105, Bonn, Germany

    Horst Urbach & Henriette Tschampa

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  1. Horst Urbach
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  2. Henriette Tschampa
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Editors and Affiliations

  1. Division of Neuroradiology, University of Heidelberg Medical Center, Im Neuenheimer Feld 400, 69120, Heidelberg, Germany

    Stefan Hähnel

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© 2009 Springer-Verlag Berlin Heidelberg

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Urbach, H., Tschampa, H. (2009). Spongiforme Encephalopathies. In: Hähnel, S. (eds) Inflammatory Diseases of the Brain. Medical Radiology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-76660-5_8

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  • DOI: https://doi.org/10.1007/978-3-540-76660-5_8

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-540-76659-9

  • Online ISBN: 978-3-540-76660-5

  • eBook Packages: MedicineMedicine (R0)

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