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Collections under which this article appears:
variant Creutzfeld-Jakob Disease
Other Neurology
Other Psychiatry

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 fellowa Richard S G Knight, consultant neurologistb Robert G Will, professor of clinical neurologyb

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@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



 


 


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Initial cases of variant CJD may have different symptoms from later cases
Thomas H Hughes-Davies
bmj.com, 21 Jun 2002 [Full text]


 

 


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