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Creutzfeldt-Jacob Disease: a case report
Eren Gozke1, Nursel Erdal, Muge Unal
1Department of Neurology, FSM Teaching and Research Hospital, Istanbul, Turkey. erengozke@superonline.com.
Cases Journal
|September 11, 2008
Summary
Rapidly progressive dementia, like Creutzfeldt-Jacob Disease (CJD), can be diagnosed using diffusion-weighted MRI (DW MRI). Cortical ribboning on DW MRI is a key indicator for CJD diagnosis.
Area of Science:
- Neurology
- Radiology
- Pathology
Background:
- Creutzfeldt-Jacob Disease (CJD) is the most common prion disease.
- CJD presents with rapid dementia, myoclonus, and neurological signs.
- Definitive diagnosis requires histological examination, but supportive findings include EEG, DW MRI, and CSF analysis.
Purpose of the Study:
- To highlight the diagnostic utility of diffusion-weighted MRI (DW MRI) in Creutzfeldt-Jacob Disease.
- To emphasize the significance of cortical ribboning as a diagnostic marker for CJD.
Main Methods:
- A case presentation of a 70-year-old male with CJD symptoms.
- Electroencephalogram (EEG) showed periodic triphasic waves.
- Diffusion-weighted MRI (DW MRI) revealed characteristic cortical ribboning, despite normal T2 and FLAIR sequences.
Main Results:
- The patient exhibited progressive dementia, involuntary movements, and akinetic mutism.
- EEG confirmed periodic triphasic waves.
- DW MRI demonstrated hyperintense signal changes in cortical regions (cortical ribboning), a key diagnostic finding.
Conclusions:
- Patients with rapidly progressive dementia and atypical features warrant DW MRI investigation.
- Cortical ribboning on DW MRI is a highly valuable diagnostic sign for Creutzfeldt-Jacob Disease.
- DW MRI aids in the early and accurate diagnosis of CJD.
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