Creutzfeldt-Jakob disease

Case contributed by Dr Edgar Lorente

Presentation

History of conversion disorder. Tonic-clonic seizures refractory to barbiturate coma.

Patient Data

Age: 60 years
Gender: Female
MRI

Bilateral diffusion restriction in both heads of the caudate nucleus and both putamina, without identification of enhancement after contrast administration.

With higher b-values this finding becomes more evident, being also identified as restriction of the diffusion in both pulvinar (see Pulvinar sign) and in right insular cortex.

Case Discussion

These findings are consistent with Creutzfeldt-Jakob disease. In this case, the patient had a history of rapidly progressive dementia in the last few months, with refractory tonic-clonic seizures in the last days. 

After MRI findings, specific study of the CSF was requested, where the presence of protein 14-3-3 was identified.

The challenge of this case is to detect bilateral symmetric pathology affecting the CNS. As a fact, this patient had a previous brain MRI 14 days before which was reported as normal. However (and in retrospect) slight diffusion restriction of both putamina and caudate heads were present.

Case courtesy of Dr. Revert and Hospital de Manises (Valencia).

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Case information

rID: 81898
Published: 6th Sep 2020
Last edited: 6th Sep 2020
Inclusion in quiz mode: Included
Institution: Hospital de Manises

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