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Long‐term preclinical magnetic resonance imaging alterations in sporadic Creutzfeldt–Jakob disease
Authors:Gianluigi Zanusso MD  Giulia Camporese MD  Sergio Ferrari MD  Luca Santelli MD  Matilde Bongianni PhD  Michele Fiorini PhD  Salvatore Monaco MD  Renzo Manara MD  Annachiara Cagnin MD
Affiliation:1. Department of Neurosciences, Biomedicine and Movement Sciences, University of Verona, Verona;2. Department of Neurosciences, University of Padua, Padua;3. Neurology Unit, Sant'Antonio Hospital, Padua;4. Division of Neuroradiology, Department of Medicine and Surgery, University of Salerno, Salerno;5. Institute of Research and Scientific Care San Camillo Hospital Foundation, Venice, Italy
Abstract:
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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