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
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May 17, 2010
Creutzfeldt-Jakob Disease
- Creutzfeldt-Jakob Disease (CJD) is a rapidly progressing, fatal, potentially transmissible dementing disorder caused by a prion (proteinacious infectious particle devoid of DNA and RNA).
- Typical clinical findings are rapid progression of dementia, myoclonus, and akinetic mutism; EEG typically shows periodic high-voltage sharp waves on background of low-voltage activity.
- Two varieties commonly described include the sCJD (sporadic CJD) affects older patients, and variant (vCJD) affects younger individuals.
- MRI is the imaging modality of choice. Diffusion-weighted imaging (DWI) best demonstrates the abnormality. FLAIR and T2WI can also be utilized to evaluate CJD.
- sCJD is characterized by bilateral symmetric foci of signal abnormality/restricted diffusion which are seen to involve the caudate nuclei and the putamina. Restricted diffusion can involve the cortical gray matter. vCJD is characterized by bilateral symmetric foci of signal abnormality seen to involve the pulvinar of the thalami.
- Signs described on imaging include: "Pulvinar" sign: Bilateral symmetrical hyperintensity of pulvinar (posterior) nuclei of thalamus. "Hockey-stick" sign: Symmetrical pulvinar and dorsomedial thalamic nuclear hyperintensity.
- PET: Regional hypometabolism of glucose correlates with sites of neuropathologic lesions.
- Prognosis: Mean duration of sCJD is 8 months. vCJD is 16 months. 10% one-year survival. No effective treatment.
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