First hundred cases of variant Creutzfeldt-Jakob disease: retrospective case note review of early psychiatric and neurological features.
OBJECTIVE
To describe the early psychiatric and neurological features of variant Creutzfeldt-Jakob disease.


METHODS
Cohort study.


METHODS
National surveillance system for Creutzfeldt-Jakob disease in the United Kingdom.


METHODS
The first 100 cases of variant Creutzfeldt-Jakob disease identified in the United Kingdom.


METHODS
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.
Spencer
Michael D
MD
Department of Psychiatry, Addenbrooke's Hospital, Cambridge CB2 2QQ, UK. r.g.will@ed.ac.uk
Knight
Richard S G
RS
Will
Robert G
RG
D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000368:Aged; D002648:Child; D015331:Cohort Studies; D007562:Creutzfeldt-Jakob Syndrome / Q000175:diagnosis / Q000453:epidemiology / Q000523:psychology*; D006801:Humans; D001523:Mental Disorders / Q000209:etiology*; D008875:Middle Aged; D012189:Retrospective Studies; D013997:Time Factors