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Clinical and Research Reports   |    
Psychiatric Presentation of Sporadic Creutzfeldt-Jakob Disease: A Challenge to Current Diagnostic Criteria
Rehiana Ali, B.A., MB BChir (Cam), M.A., MRCP; Atik Baborie, M.D., FRCPath; Andrew J. Larner, M.D., MRCP; Richard White, M.D., MRCP
The Journal of Neuropsychiatry and Clinical Neurosciences 2013;25:335-338. doi:10.1176/appi.neuropsych.13020025
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The authors declare no competing interests.

From the Dept. of Medicine, Division of Experimental Medicine, Centre for Neuroscience, Imperial College, London, U.K., and the Walton Centre for Neurology and Neurosurgery, Liverpool, U.K.

Send correspondence to Dr. R. Ali, Dept. of Medicine, Division of Experimental Medicine, Imperial College, London; e-mail: r.ali12@imperial.ac.uk

Copyright © 2013 by the American Psychiatric Association

Received February 07, 2013; Revised March 30, 2013; Accepted April 17, 2013.


Pathological diagnosis remains the gold standard for the diagnosis of sporadic Creutzfeldt-Jakob disease (sCJD), but being able to differentiate between CJD and non-prion diseases clinically is important because many of the non-prion, rapidly progressive dementias are treatable. Diagnostic criteria need both high sensitivity and specificity while remaining applicable to clinical practice. Despite extensive updates to the clinical criteria for sCJD, there remains a heavy emphasis on neurological signs. We describe a psychiatric presentation of sCJD that did not fulfill the diagnostic criteria until very late in a prolonged disease course and required biopsy for diagnosis.

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FIGURE 1. Left Frontal Brain Biopsy Highlighting the Characteristic Appearance of sCJD

The figure shows [A]: spongiform degeneration with marked neuronal loss; [B]: diffuse GFAP-positive astrocytosis; and [C]: KG9-positive synaptic or granular prion protein deposits within the same area of the left frontal lobe biopsy (bar=100 microns).



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CJD Support Network Information sheet for sporadic CJD, Jan. 2008
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