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Clinical features of new variant CJD

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

Authoring team

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

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The content herein is provided for informational purposes and does not replace the need to apply professional clinical judgement when diagnosing or treating any medical condition. A licensed medical practitioner should be consulted for diagnosis and treatment of any and all medical conditions.

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