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Creutzfeldt-Jakob disease

Case contributed by Ryan Thibodeau
Diagnosis certain


Patient with progressive dementia and discoordination.

Patient Data

Age: 50 years
Gender: Female

Diffusion-weighted imaging is notable for symmetric hyperintensity involving the corpus striatum including the caudate nucleus, lentiform nucleus, and globus pallidus.

FLAIR images demonstrate corresponding hyperintensity at the corpus striatum and the left cerebral cortex. Also noted on FLAIR imaging is hyperintensity involving the left greater than right posterior medial thalami demonstrating a "hockey-stick "appearance.

Evaluation of T2-weighted imaging demonstrates corresponding symmetric hyperintensity at the bilateral corpus striatum and thalami.

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%.

Alannah McCann

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