Sporadic and variant Creutzfeldt–Jakob disease 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. Clinical probable diagnosis is based on typical clinical symptoms associated with a characteristic EEG and/or detection of 14-3-3 protein in CSF and/or bilateral striatal and/or cortical signal hyperintensity on Diffusion-weighted and FLAIR MRI sequences. With a typical clinical picture, this MRI pattern is highly specific. Variant CJD (vCJD) has a different clinical course and MRI pattern. It consists of symmetric bilateral increased signal intensity of the pulvinar thalami (pulvinar sign) in about 80 % of cases, 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.
KeywordsMultiple System Atrophy Bovine Spongiform Encephalopathy Spongiform Change Bovine Spongiform Encephalopathy Case Akinetic Mutism
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