BMJ 2002;324:1479-1482 ( 22 June )

Papers

First hundred cases of variant Creutzfeldt-Jakob disease: retrospective case note review of early psychiatric and neurological features

Michael D Spencer, research fellow, ^a Richard S G Knight, consultant neurologist, ^b Robert G Will, professor of clinical neurology. ^b

^a Department of Psychiatry, Box 189, Addenbrooke's Hospital, Cambridge CB2 2QQ, ^b National CJD Surveillance Unit, Western General Hospital, Edinburgh EH4 2XU

Correspondence to: Robert G Will r.g.will{at}ed.ac.uk

Objective: To describe the early psychiatric and neurological features of variant Creutzfeldt-Jakob disease.
Design: Cohort study.
Setting: National surveillance system for Creutzfeldt-Jakob disease in the United Kingdom.
Participants: The first 100 cases of variant Creutzfeldt-Jakob disease identified in the United Kingdom.
Main outcome measures: The timing and nature of early psychiatric and neurological symptoms in variant Creutzfeldt-Jakob disease.
Results: The early stages of variant Creutzfeldt-Jakob disease are dominated by psychiatric symptoms, but neurological symptoms precede psychiatric symptoms in 15% of cases and are present in combination with psychiatric symptoms in 22% of cases from the onset of disease. Common early psychiatric features include dysphoria, withdrawal, anxiety, insomnia, and loss of interest. No common early neurological features exist, but a significant proportion of patients do exhibit neurological symptoms within 4 months of clinical onset, including poor memory, pain, sensory symptoms, unsteadiness of gait, and dysarthria.
Conclusions: Although the diagnosis of variant Creutzfeldt-Jakob disease may be impossible in the early stages of the illness, particular combinations of psychiatric and neurological features may allow early diagnosis in an appreciable proportion of patients.

What is already known on this topic The early stages of variant Creutzfeldt-Jakob disease are dominated by psychiatric symptomatology Some patients have early neurological features that might suggest the presence of an underlying neurological disorder What this study adds This study provides a comprehensive description of the evolution of psychiatric and neurological features in variant Creutzfeldt-Jakob disease An appreciable proportion of patients have early neurological symptoms A high proportion of patients have a combination of psychiatric and neurological features within four months of clinical onset that suggest the diagnosis of variant Creutzfeldt-Jakob disease

---

© BMJ 2002
CiteULike    Complore    Connotea    Del.icio.us    Digg    Reddit    StumbleUpon    Technorati    What's this?

Relevant Article

Clinical features may allow early diagnosis of variant CJD
BMJ 2002 324: 0. [Full Text] [PDF]

This article has been cited by other articles:

• Beck, J A, Campbell, T A, Adamson, G, Poulter, M, Uphill, J B, Molou, E, Collinge, J, Mead, S (2008). Association of a null allele of SPRN with variant Creutzfeldt-Jakob disease. J. Med. Genet. 45: 813-817 [Abstract] [Full text]  
• Stewart, L. A., Rydzewska, L. H.M., Keogh, G. F., Knight, R. S.G. (2008). Systematic review of therapeutic interventions in human prion disease. Neurology 70: 1272-1281 [Abstract] [Full text]  
• Macfarlane, R G, Wroe, S J, Collinge, J, Yousry, T A, Jager, H R (2007). Neuroimaging findings in human prion disease. J. Neurol. Neurosurg. Psychiatry 78: 664-670 [Abstract] [Full text]  
• BUTLER, R. (2006). Prion diseases in humans: an update. Br. J. Psychiatry 189: 295-296 [Abstract] [Full text]  
• Balen, A. H., Lumholtz, I. B. (2005). Consensus statement on the bio-safety of urinary-derived gonadotrophins with respect to Creutzfeldt-Jakob disease. Hum Reprod 20: 2994-2999 [Abstract] [Full text]  
• Wadsworth, J. D., Hill, A. F, Beck, J. A, Collinge, J. (2003). Molecular and clinical classification of human prion disease. Br Med Bull 66: 241-254 [Abstract] [Full text]  
• Kubler, E., Oesch, B., Raeber, A. J (2003). Diagnosis of prion diseases. Br Med Bull 66: 267-279 [Abstract] [Full text]  
• Belay, E. D., Maddox, R. A., Gambetti, P., Schonberger, L. B. (2003). Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States. Neurology 60: 176-181 [Abstract] [Full text]  
• (2002). New Variant Creutzfeldt-Jakob Disease: Early Clinical Features. JWatch Psychiatry 2002: 13-13 [Full text]  
• (2002). Probable Variant Creutzfeldt-Jakob Disease in a US Resident--Florida, 2002. JAMA 288: 2965-2967 [Full text]  
• (2002). New Variant Creutzfeldt-Jakob Disease: Early Clinical Features. JWatch Neurology 2002: 5-5 [Full text]  

Rapid Responses:

Read all Rapid Responses

Initial cases of variant CJD may have different symptoms from later cases

Thomas H Hughes-Davies
bmj.com, 22 Jun 2002 [Full text]

vCJD - some thoughts

John Fryer
bmj.com, 26 Jun 2002 [Full text]

Re: vCJD - some thoughts

Peter Morrell
bmj.com, 27 Jun 2002 [Full text]

First hundred cases of variant Creutzfeldt-Jakob disease

George Venters
bmj.com, 4 Jul 2002 [Full text]

Online poll

Find out more on doc2doc >>