期刊
ANNALS OF NEUROLOGY
卷 80, 期 4, 页码 629-632出版社
WILEY-BLACKWELL
DOI: 10.1002/ana.24757
关键词
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资金
- Veneto Region Finalized Research (Experimental Study of a Clinical Network for Diagnosing Rapidly Progressive Dementias) [RP-2014-00000400]
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.
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