Presentation
Patient with progressive dementia and discoordination.
Patient Data
Age: 50 years
Gender: Female
From the case:
Creutzfeldt-Jakob disease
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Diffusion-weighted imaging is notable for symmetric hyperintensity involving the corpus striatum including the caudate nucleus, lentiform nucleus, and globus pallidus.
Case Discussion
This is a case of Creutzfeldt-Jakob disease (CJD). There is restricted diffusion primarily of the corpus striatum. Clinically, the symptoms were suggestive of CJD, including rapidly progressive dementia. A lumbar puncture was performed and CSF was sent for analysis. Serology was positive for T tau protein (>4000 pg/mL; reference range 0-1149 pg/mL) and 14-3-3. Given these results, the estimated probability of prion disease was >98%.
Co-author:
Alannah McCann