Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
Sign up to receive an email alert when a new Case of the Week is posted.
February 21, 2011
Creutzfeldt-Jakob Disease
- Creutzfeldt-Jakob disease (CJD) is a rare, fatal, neurodegenerative disorder characterized by rapidly progressive dementia.
- Four varieties of CJD are known: sporadic (s), familial, iatrogenic (i) and variant (v). Of these, sporadic is the most common.
- sCJD presents with a rapidly progressive dementia and neurological symptoms including myoclonic jerks and ataxia. vCJD presents with behavioral/psychiatric symptoms, and painful dysesthesias.
- Key Diagnostic Features: MR is the imaging modality of choice. sCJD is characterized by foci of signal abnormality/restricted diffusion seen to involve the caudate nuclei , the putamina, and/or the cortex. vCJD is characterized by bilateral symmetric foci of signal abnormality seen to involve the pulvinar of the thalami.
- DDx: Hypoxic-ischemic encephalopathy, venous sinus thrombosis, Leigh disease, osmotic demyelination syndrome.
- Rx: Local RT, Chemotherapy
