Clinical features of variant Creutzfeldt-Jakob disease.
Since 1996, over one hundred cases of variant Creutzfeldt-Jakob disease have appeared, mostly in the United Kingdom. In this review, we summarise the major clinical features of this progressive neurodegenerative condition and compare them with those of sporadic Creutzfeldt-Jakob disease. We emphasise the young age (median 26 years) at presentation and the dominant psychiatric/behavioural features, particularly depression. Sensory symptoms are present initially in half the cases and florid psychiatric symptoms, such as delusions or hallucinations, are also common. Given these symptoms, many patients present in clinical practice initially to a psychiatrist but are referred to neurologists when neurological signs become apparent. Although the definitive diagnosis remains neuropathological, a confident pre-mortem diagnosis is now possible when the 'pulvinar sign' is seen on magnetic resonance imaging studies.
Henry
Colm
C
National Creutzfeldt-Jakob Disease Surveillance Unit, Western General Hospital, Edinburgh EH4 2XU, UK.
Knight
Richard
R
D000328:Adult; D000367:Age Factors; D007562:Creutzfeldt-Jakob Syndrome / Q000175:diagnosis* / Q000453:epidemiology / Q000523:psychology; D003863:Depression / Q000175:diagnosis; D018450:Disease Progression; D006801:Humans; D008279:Magnetic Resonance Imaging; D013788:Thalamus / Q000473:pathology; D006113:United Kingdom / Q000453:epidemiology