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Neuroradiology

E3289. Imaging of Creutzfeldt-Jakob Disease: MR and PET/CT findings

Calderon Miranda W1,2,  Boll Woehrlen M1,  Balderrama Bañares J1,  Puac Polanco P.3 1. Instituto Nacional de Neurología y Neurocirugía Manuel Velasco Suárez, Mexico City, Mexico; 2. Hospital General Dr. Manuel Gea González, Mexico City, Mexico; 3. Sanatorio Nuestra Señora del Pilar, Guatemala, Guatemala

Address correspondence to W. Calderon Miranda (willem.calderon@hotmail.com)

Background Information: Creutzfeldt-Jakob disease (CJD) is a catastrophic neurodegenerative disorder caused by prion infection that leads to spongiform degeneration of the brain. It is estimated that the annual incidence is approximately 1 per 1 million population. CJD may be classified into 3 groups: sporadic, genetic or acquired. MRI is the mainstay in the neuroimaging assessment of CJD. DWI detects abnormalities much earlier than conventional sequences. Widespread brain hypometabolism is the main finding detected on PET/CT. In this exhibit, we illustrate the imaging findings of CJD on MRI and PET/CT.

Educational Goals/Teaching Points: Participants will be able to recognize MRI and PET/CT manifestations of CJD and understand the usefulness of DWI in the diagnosis and follow-up of this entity as well as describe other conditions that may mimic CJD.

Key Anatomic/Physiologic Issues and Imaging Findings/Techniques: We discuss the epidemiology, pathology and classification of prion diseases, molecular subtypes of CJD, imaging findings including patterns of involvement, diagnostic criteria and differential diagnosis.

Conclusion: CJD is a rare cause of rapidly progressive dementia with most patients dying within one year of onset. Knowledge of imaging patterns and diagnostic criteria helps in early diagnosis and appropriate management of these potentially infectious patients.