First hundred cases of variant Creutzfeldt-Jakob disease: retrospective case
note review of early psychiatric and neurological features
Michael D Spencer, research fellow, aRichard S G Knight, consultant neurologist, bRobert 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
Objective: To describe the early psychiatric and neurologicalfeatures of variant Creutzfeldt-Jakobdisease. Design: Cohortstudy. Setting: National surveillance system for Creutzfeldt-Jakob
disease in the UnitedKingdom. Participants: The first 100 cases of variant Creutzfeldt-Jakob
disease identified in the UnitedKingdom. Main outcome measures: The timing and nature of early psychiatricand neurological symptoms in variant Creutzfeldt-Jakobdisease.
Results: The early stages of variant Creutzfeldt-Jakobdisease
are dominated by psychiatric symptoms, but neurologicalsymptoms
precede psychiatric symptoms in 15% of cases and arepresent in
combination with psychiatric symptoms in 22% of casesfrom the onset
of disease. Common early psychiatric features includedysphoria,
withdrawal, anxiety, insomnia, and loss of interest.No common early
neurological features exist, but a significantproportion of patients
do exhibit neurological symptoms within4 months of clinical onset,
including poor memory, pain, sensorysymptoms, unsteadiness of gait,
anddysarthria. Conclusions: Although the diagnosis of variant Creutzfeldt-Jakobdisease may be impossible in the early stages of the illness,
particular combinations of psychiatric and neurological featuresmay
allow early diagnosis in an appreciable proportion ofpatients.
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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