BPSU study - PIND (Progressive Intellectual and Neurological Deterioration)

Surveillance of Progressive Intellectual and Neurological Deterioration (PIND) in Children (including Creutzfeldt-Jakob Disease) through the British Paediatric Surveillance Unit began in May 1997 with the main aim of determining whether or not a child has developed variant Creutzfeldt Jakob disease (vCJD). As the clinical presentation of vCJD is not typical of classical CJD, the aim is to detect suspected cases by looking at a broader group of conditions.

Lead investigator

Dr Chris Verity

Contact details

AnneMarie Winstone / Alison Powell, Research Coordinator
Box 267
Addenbrooke’s Hospital
Hills Road
Cambridge CB2 0QQ
Email: annemarie.winstone@addenbrookes.nhs.uk/alison.powell@addenbrookes.nhs.uk

About study

Overview

Active prospective surveillance of UK children with progressive intellectual and neurological deterioration (PIND) commenced in May 1997. The main aim is to determine whether or not any child has developed variant Creutzfeldt Jakob disease (vCJD). vCJD has appeared in patients as young as 12 years of age and could occur in younger children. Although no paediatric cases have appeared since 2001, vCJD has not gone away. As the clinical presentation of vCJD is not typical of classical CJD and could be different in children, the aim is to detect suspected cases by looking at a broader group of conditions. This group needs to be large enough to include all possible cases of CJD hence the need to perform surveillance for all children with PIND.

You can download the protocol card, including references, below.

Case definition

Any child under 16 years of age at onset of symptoms who fulfils all of the following three criteria:

  • progressive deterioration for more than three months, with
  • loss of already attained intellectual/developmental abilities, and
  • development of abnormal neurological signs.

Excluding:

  • static intellectual loss e.g. after encephalitis, head injury or near drowning.

Including:

  • children who meet the case definition even if specific neurological diagnoses have been made
  • metabolic disorders leading to neurological deterioration
  • seizure disorders if associated with progressive deterioration
  • children that have been diagnosed as having neurodegenerative conditions but who have not yet developed symptoms.

Reporting restricted to:

Cases seen in the last month but including those whose conditions began earlier (i.e. including 'old cases' of children in follow-up if seen in that month).

Reporting instructions

Please report any child seen in the last month who meets the case definition, including those who have already been given a specific diagnosis.

Duration

May 1997-May 2021.

Funding

Department of Health.

Approval

Approved by Addenbrooke’s NHS Trust and the PHE.

Support groups