Creutzfeldt-Jakob disease (CJD)

Case contributed by Dr Gabrielle Matta

Presentation

4 months history of rapid functional and cognitive decline. Initial symptoms were depression and sleep disturbance. She then developed myoclonic jerks and ataxia.

Patient Data

Age: 55 years
Gender: Female

MRI brain with contrast

MRI

There is T2/FLAIR hyperintensity and restricted diffusion involving the caudate head and anterior putamen bilaterally as well as the medial temporal lobes and limbic system. Normal ventricles, sulci and cisterns. Normal major venous intracranial flow voids. No abnormal enhancement. No other foci of restricted diffusion. This may favor a cause such as CJD.

Case Discussion

Cerebrospinal fluid was positive for 14-3-3 protein consistent with a diagnosis of Creutzfeld-Jakob disease (CJD). 

Note the high signal in the caudate heads and putamen on FLAIR. There is also some cortical ribboning seen on DWI. 

From a psychiatric point of view, it is important to be aware that the earliest clinical manifestations of CJD can be mental state changes and patients may initially present with a complaint such as depression. 

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