First hundred cases of variant Creutzfeldt-Jakob disease: retrospective case note review of early psychiatric and neurological featuresBMJ 2002; 324 doi: https://doi.org/10.1136/bmj.324.7352.1479 (Published 22 June 2002) Cite this as: BMJ 2002;324:1479
- 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
- Accepted 29 May 2002
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.
Funding MDS carried out this work under the tenure of a research SHO post on the Cambridge psychiatry training rotation. The project was funded by the Department of Health. The views expressed are those of the authors.
Competing interests None declared.
- Accepted 29 May 2002