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The usual clinical profile of sporadic Creutzfeldt-Jakob disease (sCJD) is subacute dementia, motor dysfunction and myoclonus. Occasionally, some patients present atypical clinical features. We report a case of probable sCJD in a 73-year-old man with a rapidly progressive lateralized neurologic dysfunction of the left hemisphere. In a few weeks the clinical picture deteriorated dramatically to akinetic mutism and myoclonus. The 14-3-3 protein was positive in the cerebrospinal fluid. Diffusion-weighted (DWI) magnetic resonance imaging (MRI) revealed increased signal in the left cortical ribbon and deep gray matter corresponding to the clinical lateralization. He died 9 weeks after onset, autopsy was not performed. This case illustrates the correlation between the lateralized clinical and DWI MRI abnormalities in sCJD.
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