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Andreas Schröter, MD; Inga Zerr, MD; Karsten Henkel, MD; Henriette J. Tschampa, MD; Michael Finkenstaedt, MD; Sigrid Poser, MD

Arch Neurol. 2000;57:1751-1757.

Objective  To evaluate the diagnostic usefulness of magnetic resonance imaging (MRI) in the clinical diagnosis of Creutzfeldt-Jakob disease (CJD).

Background  Creutzfeldt-Jakob disease is a rare neurodegenerative disease that belongs to the group of human spongiform encephalopathies and usually affects elderly people. It is clinically characterized by rapidly progressive dementia and development of neurological symptoms, such as myoclonus or ataxia. Until now, neuroradiologic investigations have only played a minor role in establishing the clinical diagnosis of CJD, and they are often performed to exclude differential diagnoses.

Setting  A university hospital, base of the German National Creutzfeldt-Jakob Disease Surveillance Study.

Methods and Patients  In this study, MRIs from suspected cases of CJD were examined by one investigator blinded to the diagnosis. Patients were classified according to the established clinical and neuropathological criteria.

Results  Bilateral symmetric, high signal intensities on T2-weighted MRIs were present in the basal ganglia of 109 (67%) of 162 patients with CJD. In the control group, which consisted of non-CJD dementia patients, these abnormalities on T2-weighted MRIs were found in 4 (7%) of 58 patients. This corresponds to a high specificity in the differential diagnosis of CJD.

Conclusion  These results indicate that MRI is a useful and valuable tool with reasonable sensitivity (67%) and high specificity (93%) and should be considered as an additional cornerstone in the clinical diagnosis of CJD.

From the Departments of Neurology (Drs Schröter, Zerr, Henkel, Tschampa, and Poser) and Neuroradiology (Dr Finkenstaedt), University of Göttingen, Göttingen, and the Magnetic Resonance/Computed Tomography Institute Hamburg, Hamburg (Dr Finkenstaedt), Germany.

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