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

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.

• Authors: Gianluigi Zanusso, Giulia Camporese, Sergio Ferrari, Luca Santelli, Matilde Bongianni, Michele Fiorini, Salvatore Monaco, Renzo Manara, Annachiara Cagnin
• Journal: Annals of neurology (Ann Neurol) Vol. 80 Issue 4 Pg. 629-32 (Oct 2016) ISSN: 1531-8249 [Electronic] United States
• PMID: 27501375 (Publication Type: Case Reports, Journal Article)
• Copyright: © 2016 American Neurological Association.

Topics

• Aged
• Cerebral Cortex (diagnostic imaging)
• Creutzfeldt-Jakob Syndrome (diagnosis, diagnostic imaging, physiopathology)
• Disease Progression
• Fatal Outcome
• Female
• Humans
• Magnetic Resonance Imaging
• Prodromal Symptoms