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MR imaging of Creutzfeldt-Jakob disease
M Finkenstaedt1, A Szudra, I Zerr
1Department of Radiology, Scott and White Clinic and Memorial Hospital, Sherwood, Temple, TX 76508, USA.
Purpose:
To describe the magnetic resonance (MR) imaging appearance of Creutzfeldt-Jakob disease (CJD).
Materials And Methods:
MR images obtained in 29 patients who died of CJD (aged 53-77 years at death) were retrospectively reviewed by three neuroradiologists blinded to the diagnosis.
Results:
Moderate to marked bilateral, symmetrically increased signal intensity was demonstrated in the putamen and caudate nucleus on T2- and proton-density-weighted MR images in 23 patients (79%). In six patients (21%), images showed no major signal intensity abnormalities. T1-weighted images revealed no signal intensity abnormalities and no contrast material enhancement. The degree of atrophy in the cortex and basal ganglia corresponded to the time between onset of symptoms and MR imaging. All patients with a disease duration of longer than 4 months had substantial volume loss.
Conclusion:
Although approximately 20% of the patients did not have MR imaging abnormalities, MR imaging did show signal intensity alterations due to gliosis and spongiform changes early in the course of CJD in the remaining 80%. The demonstration of bilateral areas of increased signal intensity that predominantly affected the caudate nuclei and the putamina on long-repetition-time MR images in an elderly patient with rapidly progressive dementia represents a specific finding and clearly should suggest the diagnosis of CJD.
Insights
Magnetic resonance imaging (MRI) can detect Creutzfeldt-Jakob disease (CJD) in 80% of cases. Characteristic bilateral signal changes in the caudate nucleus and putamen suggest CJD in patients with rapidly progressive dementia.
Area of Science:
- Neurology
- Radiology
- Neuroscience
Background:
- Creutzfeldt-Jakob disease (CJD) is a rare, fatal neurodegenerative prion disease.
- Early diagnosis of CJD is crucial for patient management and research.
- Magnetic resonance (MR) imaging is a key diagnostic tool in neurology.
Purpose of the Study:
- To characterize the specific magnetic resonance (MR) imaging findings associated with Creutzfeldt-Jakob disease (CJD).
- To evaluate the sensitivity of MR imaging in detecting CJD.
- To establish MR imaging as a diagnostic marker for CJD.
Main Methods:
- Retrospective review of MR images from 29 deceased CJD patients.
- Neuroradiologists assessed images blinded to the diagnosis.
- Analysis focused on signal intensity and atrophy patterns on various MR sequences.
Main Results:
- 80% of patients showed bilateral, symmetrical hyperintensities in the putamen and caudate nucleus on T2- and proton-density-weighted images.
- No signal abnormalities or enhancement were observed on T1-weighted images.
- Disease duration correlated with cortical and basal ganglia atrophy; significant volume loss occurred after 4 months.
Conclusions:
- MR imaging reveals characteristic signal alterations in 80% of CJD cases, indicative of gliosis and spongiform changes.
- The presence of bilateral hyperintensities in the caudate nuclei and putamina on long-repetition-time MR images is highly suggestive of CJD.
- MR imaging findings, particularly in elderly patients with rapidly progressive dementia, strongly support a CJD diagnosis.