Clinical features of new variant CJD

The new variant of CJD is distinct from the sporadic or classical form. It is characterised primarily by young onset, often with early psychiatric or behavioural disturbances and early cerebellar ataxia. The illness is more often prolonged and the classical EEG findings are often absent.

The following features have been described:

• early age of onset or death (18-41 yrs average 27.6)
  
  
• prolonged illness (7.5-24 months average 13.1)
  
  
• psychiatric presentation:
  • anxiety
  • depression
  • withdrawal
  • progressive behavioural change
  • presentation to a psychiatrist
    
    
• limb and/or facial dysaethesiae
  
  
• cerebellar syndrome with gait and limb ataxia
  
  
• late development of memory disturbance with progression to severe cognitive impairment and a state of akinetic mutism
  
  
• myoclonus develops late, often preceded by choreiform movements