Sporadic Creutzfeldt-Jakob Disease

This patient presented with a rapidly-progressive dementia, with initial hallucinations and behavioural change. The disease progressed to a mute, akinetic state with myoclonus. Electroencephalogram showed widespread periodic sharp waves.
This diffusion-weighted MR image shows right cortical and striatal high signal, consistent with a diagnosis of sporadic-type Creutzfeldt-Jakob disease (sCJD). Typical MR findings are present in 71% of sCJD cases, and specificity may be as high as 93%. Variant-type Creutzfeldt-Jakob disease gives different MRI findings, with putaminal signal more marked than striatal signal, and cortical changes are rarely seen.
Reference:
Kallenberg K, et al. Creutzfeldt-Jakob disease: comparative analysis of MR imaging sequences. AJNR Am J Neuroradiol 2006;27:1459-62
Credit: Dr Laughlin Dawes
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