Creutzfeldt-Jakob disease

Case contributed by A.Prof Frank Gaillard

Presentation

4 month history of rapid decline in cognitive function.

Patient Data

Age: 55 years
Gender: Female
MRI

Restricted diffusion in the basal ganglia (caudate and lentiform nuclei) is prominent associated with less marked, but nonetheless definite, increase in T2 signal. 

Case Discussion

The diagnosis of Creutzfeldt-Jakob disease (CJD) has been established, with further corroborating clinical features and CSF. Initially, the cognitive decline started with depressive features, sleep disturbance then myoclonic jerks and ataxia. CSF was positive for 14-3-3 protein. Imaging features are also characteristic. 

A differential to always consider is autoimmune encephalitis, particularly anti-D2 dopamine antibodies, which can have a similar distribution of imaging changes and similar initial presentation. Helpful in distinguishing between the two is that T2 signal change is dominant in autoimmune encephalitis, whereas diffusion change is most striking in CJD. 

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Case information

rID: 44855
Case created: 5th May 2016
Last edited: 28th Feb 2017
Inclusion in quiz mode: Included

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