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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
View Author and Article Information

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.

Abstract

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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References

Masters  CL;  Harris  JO;  Gajdusek  DC  et al:  Creutzfeldt-Jakob disease: patterns of worldwide occurrence and the significance of familial and sporadic clustering.  Ann Neurol 1979; 5:177–188
[CrossRef] | [PubMed]
 
Brandel  JP;  Delasnerie-Lauprêtre  N;  Laplanche  JL  et al:  Diagnosis of Creutzfeldt-Jakob disease: effect of clinical criteria on incidence estimates.  Neurology 2000; 54:1095–1099
[CrossRef] | [PubMed]
 
Zerr  I;  Kallenberg  K;  Summers  DM  et al:  Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease.  Brain 2009; 132:2659–2668
[CrossRef] | [PubMed]
 
Meissner  B;  Kallenberg  K;  Sanchez-Juan  P  et al:  MRI lesion profiles in sporadic Creutzfeldt-Jakob disease.  Neurology 2009; 72:1994–2001
[CrossRef] | [PubMed]
 
Wall  CA;  Rummans  TA;  Aksamit  AJ  et al:  Psychiatric manifestations of Creutzfeldt-Jakob disease: a 25-year analysis.  J Neuropsychiatry Clin Neurosci 2005; 17:489–495
[CrossRef] | [PubMed]
 
Hancock  P;  Larner  AJ:  Test Your Memory test: diagnostic utility in a memory clinic population.  Int J Geriatr Psychiatry 2011; 26:976–980
[CrossRef] | [PubMed]
 
Larner  AJ;  Doran  M:  Prion disease at a regional neuroscience centre: retrospective audit.  J Neurol Neurosurg Psychiatry 2004; 75:1789–1790
[CrossRef] | [PubMed]
 
Rabinovici  GD;  Wang  PN;  Levin  J  et al:  First symptom in sporadic Creutzfeldt-Jakob disease.  Neurology 2006; 66:286–287
[CrossRef] | [PubMed]
 
Poser  S;  Mollenhauer  B;  Kraubeta  A  et al:  How to improve the clinical diagnosis of Creutzfeldt-Jakob disease.  Brain 1999; 122:2345–2351
[CrossRef] | [PubMed]
 
Krasnianski  A;  Schulz-Schaeffer  WJ;  Kallenberg  K  et al:  Clinical findings and diagnostic tests in the MV2 subtype of sporadic CJD.  Brain 2006; 129:2288–2296
[CrossRef] | [PubMed]
 
Josephs  KA;  Ahlskog  JE;  Parisi  JE  et al:  Rapidly progressive neurodegenerative dementias.  Arch Neurol 2009; 66:201–207
[CrossRef] | [PubMed]
 
CJD Support Network Information sheet for sporadic CJD, Jan. 2008
 
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