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VARIANT CJD PRESENTS DIFFERENTLY IN OLDER PATIENTS
  1. Graham Mackay,
  2. Richard Knight,
  3. Robert Will
  1. Southern General Hospital, Glasgow; National CJD research and surveillance unit, Edinburgh

    Abstract

    Introduction Variant Creutzfeldt–Jakob (vCJD) has an established clinical phenotype and diagnostic criteria. However, this phenotype has largely been described in a cohort of young vCJD cases, with a median age of onset of 27.

    Methods Having reviewed the 176 definite and probable vCJD cases referred to UK CJD surveillance, six older cases, over 55 years were found. The clinical records of these cases were reviewed.

    Results The six older vCJD cases were all male. Two had received blood from donors confirmed to have had vCJD. Only two of these cases met the diagnostic criteria for vCJD in life. Three cases had atypical clinical histories without either early psychiatric symptoms or involuntary movements. Only one of five patients had the typical pulvinar sign on their MRI scan.

    Conclusion No cases of vCJD were diagnosed in the UK in 2012. However, clinicians should be aware of the occurrence of vCJD, with less typical phenotypes, in older patients. vCJD should be considered in older patients dying with dementia deteriorating in under three years, with ataxia and a rapid final deterioration. Post mortem should be considered in such cases, given the public health risks among a population more prone to undergoing medical procedures.

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