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Case 20

Creutzfeld-Jakob Disease

A 58 year old with rapid onset dementia

Terry Featherstone

A 58 year old man presented with a short history of profound lethargy, diminished concentration, memory impairment and poor balance. On admission he was disorientated in relation to time, place and person.

On examination he had intention tremor, truncal ataxia and bilateral grasp reflex. Blood tests revealed no abnormality. A CT scan was normal.

Thereafter an MRI scan was performed; the T1 weighted scans were normal. Sample axial proton density and T2 weighted images are shown.

Findings

There are bilateral symmetrical paired areas of increased signal intensity in the lentiform nuclei (basal ganglia).

Diagnosis

Creutzfeld-Jakob Disease (CJD)

Differential

1. Asphyxia - carbon monoxide poisoning, strangulation or drowning (NB. however on CT these conditions usually show hypodense areas and low signal on MRI T1 weighted images).
2. Wilsons disease (NB. patients will have abnormal serum levels of copper and ceruloplasmin).
3. Leigh disease (a rare disease of childhood - lesions are hypodense on CT).
4. HIV infection.

Discussion

CJD was confirmed by post mortem analysis of the brain a few weeks after the MRI scan.

Bilateral hyperintense abnormalities in the basal ganglia on proton density and T2 weighted images have now been described in a number of cases of CJD.

The prevalence of these lesions in patients with proven CJD is not known. In time when more cases have been reviewed this abnormality in patients with rapidly progressive dementia may prove to be a specific sign of this particular subacute spongiform encephalopathy.

Addendum - Sep 2001, A Downie

Knowledge of CJD has moved on since this case was submitted. High intensity signal in the putamen and head of caudate nucleus on T2 weighted images, as seen here, are thought to be fairly sensitive but more than 90% specific for spontaneous CJD (sCJD).

Following the Bovine Spongiform Encephalitis (BSE) epidemic in cows in the UK, a new variant of CJD (vCJD) emerged in the 1990s, thought to be related to BSE. This has a rapid clinical course in younger patients, and the imaging findings are also different. The characteristic finding is of high signal intensity in the pulvinar, the most posterior nucleus of the thalamus. These features are well described in the third reference.

References

1. Barboriak DP, Provenzale JM, Boyko OB. MRI diagnosis of Creutzfeld-Jakob disease: significance of high signal intensity of the basal ganglia. AJR 1994;162:137-140.
2. Weerasinghe S, Featherstone T. Creutzfeld-Jakob disease - can MRI help in diagnosis? International Journal of Geriatric Psychiatry 1996;11:271-272.
3. Collie DA et al. MRI of Creutzfeld-Jakob Disease: Imaging features and recommended MRI protocol. Clinical Radiology 2001;56:726-739.

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