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Ann Neurol. 2016 Oct;80(4):629-32. doi: 10.1002/ana.24757. Epub 2016 Aug 22.

Long-term preclinical magnetic resonance imaging alterations in sporadic Creutzfeldt-Jakob disease.

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  • 1Department of Neurosciences, Biomedicine and Movement Sciences, University of Verona, Verona.
  • 2Department of Neurosciences, University of Padua, Padua.
  • 3Neurology Unit, Sant'Antonio Hospital, Padua.
  • 4Division of Neuroradiology, Department of Medicine and Surgery, University of Salerno, Salerno.
  • 5Department of Neurosciences, University of Padua, Padua.
  • 6Institute of Research and Scientific Care San Camillo Hospital Foundation, Venice, Italy.


An asymptomatic 74-year-old woman, on follow-up for a carotid body tumor, showed magnetic resonance imaging (MRI) focal restricted diffusion confined to the left temporal and occipital cortices. Thirteen months later, diffusion-weighted images revealed a bilateral cortical ribbon sign involving all lobes. After 1 month, the patient developed gait instability and cognitive decline rapidly evolving to severe dementia and death within 3 months. Prion protein gene sequence, molecular, and neuropathological studies confirmed the diagnosis of sporadic Creutzfeldt-Jakob disease (sCJD) MM1 subtype. Here we show the kinetics of MRI changes and prion spreading in preclinical sCJD MM1. Ann Neurol 2016;80:629-632.

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