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Spongiform Encephalopathy Consistent with Creutzfeldt-Jakob Disease

Milos J Janicek, MD
Amir A Zamani, MD

March 5, 1996

Presentation

A 68-year-old woman presented with rapidly progressive dementia without any localizing neurological signs. She denied any history of trauma or systemic disease.

Imaging Findings

T2-weighted MRI of brain
T2-weighted MRI of brain, 2 months later

T2-weighted magnetic resonance (MR) images of the brain obtained two months apart demonstrate increasing conspicuousness of high intensity lesions (arrows) affecting the basal ganglia (caudate [white arrow heads] and putamen [black arrows]) symmetrically, with relative sparing of the palladium. The CT scan (obtained after stereotactic biopsy) was unrevealing.

Differential Diagnosis

Differential diagnosis is relatively short, including Creutzfeldt-Jakob disease and clinically unlikely conditions such as familial striatal degeneration, Leigh disease, and Wilson's disease (in which, however, T2 bright lesions are often accompanied by low intensity lesions due to iron pigment deposition).

Diagnosis

Based on MRI-demonstrated abnormalities, the patient underwent a stereotactic biopsy of the putamen and cerebral cortex, which confirmed the diagnosis of spongiform encephalopathy consistent with Creutzfeldt-Jakob disease.

Discussion

Finding of symmetrical high signal intensity abnormalities on T2-weighted images in the setting of rapidly progressing dementia has been reported in patients with Creutzfeldt-Jakob (CJ) disease. But given the rapid progression of the disease, it would not be unusual for MR and CT images, obtained at early stages of the disease, to appear normal.

Despite its rarity in the general population, Creutzfeldt-Jakob disease may become clinically manifest in approximately 3% of patients with AIDS or hematological malignancies, as a result of their cellular immunodeficiency.

References

1. Taveras JM, Ferrucci JT. Radiology Diagnosis-Imaging-Intervention, Vol. 3, Ch. 39; 7.

2. Drayer BP. Degenerative disorders of the CNS. RSNA Syllabus - Neuroradiology. 1994; 165.

3. DiRocco A, Molinari S, Stollman AL, et al. MR abnormalities in Creutzfeldt-Jakob disease. Neuroradiology 1993; 35:584-585.

4. Gertz HJ, Henkes H, Arvos-Navarro J. Creutzfeldt-Jakob disease: Correlation of MRI and neuropathologic findings. Neurology 1988; 38:1481-1482.

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