Creutzfeldt-Jakob disease is the prototypic rapidly progressive dementia. Esporadic CJD is the most common presentation. We report a 65 year-old patient with primary visual complaints, afterward psychosis with memory and higher cerebral functions complaints. After 4 months of evolution, the patient showed akinetic mutism and myoclonus. In the MRI, anormal hyperintensity was seen on T2 sequences in the subcortical and cortical regions of the occipital lobes. EEG revealed periodic generalized triphasic waves. We present this patient with the Heindenhain variant of sporadic CJD, in order to consider it when were are faced with a patient with rapidly progressive dementia (RPD). We emphasize the importance of early diagnosis for prognostic value and for minimize the risk of accidental iatrogenic transmission.

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