Abstract:
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Purpose: CJD is a prion-mediated neurodegenerative illness characterized by rapidly progressive dementia, ataxia and myoclonus leading to death. We characterize the diffusion-weighted imaging findings in CJD and their utility in the early diagnosis and follow-up of CJD patients.
Methods and Materials: We collected MR imaging studies of the brain performed at Thomas Jefferson University Hospital and Yale-New Haven Hospital on twelve patients with Creutzfeldt-Jakob disease (CJD). Ten of the patients were biopsy-proven, while the other two had clinical findings, laboratory results (including 14-3-3 protein analysis in the CSF) and MR imaging compatible with the diagnosis. MR studies consisted of pre- and post gadolinium T1, T2, FLAIR and DWI sequences
Results: Most of the patients had characteristic DWI findings, even on their earliest studies. These included hyperintense signal in the basal ganglia and/or focal areas of the cerebral cortex. Follow-up studies showed larger areas of signal abnormality in the cerebral cortex with associated cerebral atrophy that correlated with the clinical evolution.
Conclusion: DWI is the most sensitive and specific MR sequence in the diagnosis of CJD and provides a working diagnosis before laboratory results are available. DWI also may obviate the need for brain biopsy and can be used to follow the progression of disease in these patients.
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Cevasco MD, F,
Characteristic Diffusion-weighted Imaging Findings in Creutzfeldt-Jakob Disease. Radiological Society of North America 2003 Scientific Assembly and Annual Meeting, November 30 - December 5, 2003 ,Chicago IL.
http://archive.rsna.org/2003/3108297.html
