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.

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.