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

BMJ 2002; 324 doi: (Published 22 June 2002) Cite this as: BMJ 2002;324:1479
  1. Michael D Spencer, research fellowa,
  2. Richard S G Knight, consultant neurologistb,
  3. Robert G Will (r.g.will{at}, professor of clinical neurologyb
  1. a Department of Psychiatry, Box 189, Addenbrooke's Hospital, Cambridge CB2 2QQ
  2. b National CJD Surveillance Unit, Western General Hospital, Edinburgh EH4 2XU
  1. 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
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